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of the small muscles of the left hand since impaired pain and temperature sensations in LETTERS August 1998. This deficit stabilised after a both upper limbs and in the shoulder girdle period of one year. At the onset of illness, he region. Magnetic resonance imaging of the had also developed mild difficulty in using spine and brain revealed a septate intrinsic cord ‘‘Dropped head syndrome’’ in his right hand for performing fine work but hypointensity in T1 weighted images, becom- this symptom remained stable. He noticed ing hyperintense in T2 weighted images syringomyelia: report of two occasional fasciculations over the arms for extending from the C2 to C7 (fig 1) levels, cases one year. After two years and nine months he associated with low lying cerebellar tonsils and ‘‘Dropped head syndrome’’ is characterised developed rapidly progressive head drop and brainstem reaching the lower border of C1. by weakness of the extensor muscles of the required assistance of the hand to maintain Brain images revealed evidence of hydrocepha- neck, with or without involvement of the the head in an erect posture, and he also lus. There was no evidence of myelomeningo- neck flexors, and is commonly caused by a had mild difficulty in lifting his head off the cele. Electromyography of the distal muscles in variety of neuromuscular disorders, including bed while rising from the supine position. the upper limbs showed fibrillation and fasci- myasthenia gravis, polymyositis, amyotrophic There was no history of impaired sensation culation potentials. Motor and sensory conduc- lateral sclerosis, facio–scapulo–humeral dys- to touch, pain, or temperature, or inadvertent tion studies were normal. Investigations trophy, nemaline myopathy, carnitine defi- burns over the hands or shoulders. There including serum chemistry and haemogram ciency, spinal muscular atrophy, and isolated were no features to suggest Horner’s syn- were normal. neck extensor myopathy.12There are isolated drome or symptoms referring to the cranial reports of dropped head syndrome occurring nerves, lower limbs, cerebellar system, or Case report 2 sphincters. There was no nuchal pain, res- in cervical spondylitis and ankylosing spon- A 30 year old man presented in December dylitis.3 In this report, we describe the clinical tricted neck movements, or symptoms of raised intracranial pressure. 2003 with progressive weakness and atrophy and imaging findings of two patients who of the right shoulder girdle muscles with a Examination revealed mild cerebellar dys- had dropped head syndrome as a rare duration of one year, followed by similar arthria and fasciculations over the tongue. neurological sign secondary to syringomyelia. symptoms on the left side for 10 months; at There were no features of Horner’s syndrome presentation he was unable to raise his arms. and the eye movements, facial sensations, In the last six months he had developed head palatal reflexes, and pharyngeal reflexes were Case report 1 drop, with difficulty in maintaining the head normal. Occasional fasciculations were pre- A 46 year old right handed man presented in the erect posture, and since this time he sent over the arms. There was Medical during May 2002 with insidious onset, had noticed weakness and atrophy of the Research Council grade 2 weakness of the gradually progressive weakness and wasting hand muscles. There were no symptoms of neck extensor muscles with head drop and pain or restricted neck movements, and there grade 4 weakness of the neck flexors. There was no sensory impairment, sphincter dis-

was mild wasting of the erector spinae copyright. turbance, or cerebellar ataxia. Neurological muscles in the neck and asymmetric wasting deficits were present in the form of thoracic of the small muscles of the hand, including kyphoscoliosis and wasting of the extensor the thenar, hypothenar, and interossei mus- muscles of the neck, resulting in severe cles bilaterally, with the left side being weakness and dropped head (fig 2). Power affected more severely. Power in the lower in the neck flexors was grade 4. Severe limbs was normal. Deep tendon reflexes were asymmetric wasting of the shoulder girdle sluggish in the upper limbs and exaggerated muscles, in addition to moderate wasting of in the lower limbs, with a bilateral flexor the arm, forearm, and hand muscles was plantar response. The sensory system noted, with bilateral clawing. There was revealed bilateral C2 hypoesthesia, with grade 2 power in the proximal muscles, including the arm, and grade 3 in the small muscles. Deep tendon reflexes were absent in the upper limbs and exaggerated in the lower limbs, with a bilateral extensor plantar res- http://jnnp.bmj.com/ ponse. Temperature sensation was impaired in the entire body except for the face. Posterior column and spinothalamic sensa- tions were normal. Contrast magnetic reso- nance imaging of the spine, including T1 weighted and T2 weighted images, revealed a cerebrospinal fluid signal intensity lesion involving the entire cord, with expansion of

the cord, and no evidence of abnormal on September 26, 2021 by guest. Protected contrast enhancement, suggestive of holo- cord syringomyelia.

Discussion Dropped head syndrome is a well known feature in a variety of neuromuscular disorders, and is also described in diseases of the bone and joints, such as ankylosing spondylitis and cervical spondylitis.3 Neck flexion weakness is typical in most conditions, but prominent neck extensor weakness has been described in several reports. In the recent review article on dropped head syndrome in amyotrophic lateral sclerosis, 19 different conditions are listed as Figure 1 Sagittal T2 weighted image showing Figure 2 Clinical feature of dropped head causes for dropped head syndrome, but syr- the hyperintense lesion in the cervicodorsal with severe wasting and weakness of neck ingomyelia is not mentioned as a cause.4 Our cord with multiple haustrations and associated extensor muscles. This photograph is patients presented with classic features of tonsillar herniation. reproduced with the full consent of the patient. cervical intrinsic cord lesion suggestive of

www.jnnp.com PostScript 291 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.047357 on 14 January 2005. Downloaded from syringomyelia and had the typical features of score was calculated. As previously, we correlation with IQ scores on the same test dropped head syndrome. included only participants with mini-mental given 68 years previously. This is a similar state examination scores greater than 23 and situation to that with blood pressure.2 This A Nalini, S Ravishankar no history of dementia, to exclude patholo- further emphasises the importance of inter- National Institute of Mental Health and Neurosciences, gical cognitive decline as far as possible.4 preting associations between cognition and Bangalore, India None of the participants had a history of other variables in older people in the context head trauma or ongoing CNS affecting of ‘‘pre-morbid’’ mental ability. The effect Correspondence to: Dr A Nalini, National Institute of disease. The resultant sample comprised 470 size of vitamin B-12 is smaller than those Mental Health and Neurosciences (NIMHANS), Hosur participants (194 men, 276 women). found with more domain specific cognitive Road, Bangalore 560 029, India; nalini@nimhans. tests,3 contributing less than 1% of total kar.nic.in; [email protected] Mean (SD) serum vitamin B-12 was 390 (161) ng/l (n = 422) and mean serum folate variance in age 79 IQ. It is unlikely to be doi: 10.1136/jnnp.2004.040428 was 337 (155) mg/l (n = 391). Pearson corre- clinically apparent. However, the effect was lation coefficients with age 11 IQ were: more significant in the small subsample with Received 4 March 2004 laboratory defined deficiency. Revised form received 2 June 2004 r = 0.04 (p = 0.42) for B-12 and r = 0.13 Accepted 8 June 2004 (p = 0.010) for folate; and with age 79 IQ, Inspection of the relation between vitamin r = 0.12 (p = 0.018) for B-12 and r = 0.12 B-12 and the standardised residual score of Competing interests: none declared (p = 0.016) for folate. Linear regression of age age 79 IQ regressed on age 11 IQ (fig 1) 79 IQ controlling for age 11 IQ confirmed a suggests that the overall correlation is The patient gave consent for reproduction of the accounted for by a subgroup that is cogni- photograph. significant effect on age 79 IQ for B-12 (b = 0.092, p = 0.016, R2 improvement = tively vulnerable to vitamin B-12 deficiency. 0.008) but not for folate (b = 0.038, Moreover, this vulnerability occurs at levels References p = 0.33). Only two participants had folate within the normal laboratory range. An IQ levels below the normal range (,5 mg/l) and decline of .1 SD occurred in seven of 18 1 Suarez GA, Kelly JJ. The dropped head omitting these did not affect correlation participants (39%) with a serum vitamin B- syndrome. Neurology 1992;42:1625–7. coefficients with age 11 and age 79 IQ scores. 12 of ,200 ng/l, 19 of 84 (23%) with levels of 2 Lomen-Hoerth C, Simmons ML, DeArmond SJ, 200–299 ng/l, 21 of 103 (20%) with levels of et al. Adult-onset nemaline myopathy: another Twenty five participants had vitamin B-12 cause of dropped head. Muscle Nerve levels below the normal range (,200 ng/l) 300–399 ng/l, and 21 of 149 (14%) with levels 1999;22:1146–9. and there was a stronger correlation with age of .399 ng/l. Further work is required to 3 Swash M. Dropped-head and bent-spine 79 IQ in this group (r = 0.57, p,0.001) than confirm this and ascertain what makes these syndromes; axial myopathies? Lancet in those well within the normal range individuals vulnerable. One explanation is 1998;352:758. >250 ng/l (r = 0.10, p = 0.031). The differ- that this is a group having ‘‘metabolically 4 Gourie-Devi M, Nalini A, Sandhya S. Early or ence between these two correlation coeffi- significant’’ vitamin B-12 deficiency within late appearance of ‘‘dropped head syndrome’’ in cients was significant (p = 0.016). After the normal range with raised homocysteine amyotrophic lateral sclerosis. J Neurol Neurosurg or methylmalonic acid levels.5 This may help Psychiatry 2003;74:683–6. adjusting for all variables known to be associated with lifetime change in IQ (sex, target B-12 therapy, but at present identify- APOE e4 status, cigarette smoking, statin use, ing this group remains challenging. Our data Vitamin B-12, serum folate, and and number of drugs prescribed),4 vitamin B- suggest that in a non-demented, relatively cognitive change between 11 and 12 remained a significant contributor healthy population, serum folate concentra- (b = 0.095, p = 0.011). Together, these vari- tions were not related to IQ in old age after copyright. 79 years ables explained 4.5% of total variance in age controlling for childhood mental ability. A recent Cochrane review reported that 79 IQ scores after adjusting for IQ at age 11. However, only two participants in our sample although vitamin B-12 deficiency is known The number of units of alcohol consumed per had folate values below the normal range, so to be associated with cognitive impairment in week was also positively correlated with age other studies are needed to assess the old age, benefits of supplementation on 79 IQ score (Spearman r = 0.10, p = 0.026), cognitive effects of folate deficiency and mental ability are unclear.1 The situation is but was no longer significantly associated treatment with folic acid. similar to that for blood pressure, in which (b = 20.01, p = 0.73) once age 11 IQ and hypertension is associated with neuropsycho- vitamin B-12 were adjusted for. ACKNOWLEDGEMENTS logical deficits in adulthood but cognitive The research was supported by the UK outcomes of lowering blood pressure in Biotechnology and Biological Sciences randomised controlled trials are equivocal. COMMENT Research Council. JMS is the recipient of a We found that for blood pressure, the limited Both vitamin B-12 and folate correlate with ‘‘Leading Practice Through Research’’ award effect of intervention is partly explained by IQ in old age in a non-demented population. from the Health Foundation. IJD is the the relation between childhood IQ and adult Lower serum B-12 at age 79 is associated recipient of a Royal Society–Wolfson http://jnnp.bmj.com/ blood pressure—children with higher IQs had with cognitive decline between age 11 and 2 Research Merit Award. LJW holds a lower mid-life blood pressures. As approxi- age 79. By contrast, serum folate at age 79 Wellcome Trust Career Development Award. mately 50% of the variance in adult IQ is correlates with age 11 IQ, and controlling for explained by childhood IQ, studies failing to this reduces the correlation with IQ in old age J M Starr account for ‘‘pre-morbid’’ mental ability are to almost zero. Hence, in this sample the Geriatric Medicine unit, Royal Victoria Hospital, likely to overestimate the association between relation between serum folate and old age Craigleith Road, Edinburgh, EH4 2DN, UK adult IQ and blood pressure. Previously we mental ability can be fully explained by its reported significant relations between various A Pattie, M C Whiteman, I J Deary Psychology, School of Philosophy, Psychology and tests of mental ability in old age and vitamin B- on September 26, 2021 by guest. Protected 12 and folic acid concentrations in the blood.3 Language Sciences, University of Edinburgh We now describe the association between 2.0 vitamin B-12 and serum folate and lifetime L J Whalley change in mental ability using the same Department of Mental Health, University of Aberdeen, Clinical Research Centre, Cornhill Royal Hospital, cognitive test at age 11 and age 79. 0.0 4 Aberdeen, UK As reported more fully elsewhere, the 1932 Dr John M Starr; [email protected] Scottish Mental Survey (SMS1932) measured –2.0 the mental ability of almost all (n = 87 498) doi: 10.1136/jnnp.2004.046219 children born in 1921 and attending Scottish Competing interests: none declared schools on 1 June 1932 with a validated test –4.0 of IQ, the Moray House test (MHT). With Age 80 IQ on age 11 local ethics approval, 550 survivors of the 0 200 400 600 800 1000 REFERENCES SMS1932 were recruited in Lothian, Scotland by a variety of means. They underwent a Vitamin B-12 (ng/l) 1 Malouf R, Areosa Sastre A. Vitamin B12 for cognition. Cochrane Database of Systematic health assessment, including blood sampling Reviews. CD004326, 2003. for vitamin B-12 and folate, and the MHT Figure 1 Vitamin B-12 levels and 4 2 Starr JM, Taylor MD, Hart CL, et al. Childhood was re-administered. In all, 483 participants standardised residuals of age 79 IQ regressed mental ability and blood pressure at midlife: were matched to age 11 MHT scores from the on age 11 IQ in a surviving cohort of the linking the Scottish Mental Survey 1932 and the SMS1932, and an age-in-days-adjusted IQ Scottish Mental Survey 1932 (n = 422). Midspan studies. J Hypertens 2004;22:893–8.

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3 Duthie SJ, Whalley LJ, Collins AR, et al. chance of revascularisation with intravenous known carotid artery dissection revealed by Homocysteine, B vitamin status and cognitive thrombolytic therapy, emergent endovascular local signs or a transient ischaemic attack, as function in older adults. Am J Clin Nutr revascularisation followed by intra-arterial in our patient, no recommendation exists 2002;75:908–13. thrombolysis was planned. Angiography about emergent therapy. Some reports sug- 4 Deary IJ, Whiteman MC, Starr JM, et al. The impact of childhood intelligence in later life: (fig 1A) performed within three hours and gest that intravenous thrombolysis might be following up the Scottish Mental Surveys of 1932 30 minutes of onset of the new symptoms safe and effective when given within three and 1947. J Pers Soc Psychol 2004;86:130–47. showed the typical narrowed aspect of the hours of onset of stroke without worsening 5 Clarke R, Grimley Evans J, Schneede J, et al. ICA with no opacification of the intracranial the arterial wall tearing.6 However, in case of Vitamin B12 and folate deficiency in later life. arteries. Right hemispheric circulation was severe stenosis, near occlusion, or even Age Ageing 2004;33:34–41. analysed on the left carotid angiogram: the occlusion of the ICA, regardless of the cause, right anterior cerebral artery (ACA) filled via intravenous or intra-arterial thrombolysis has 7 Stent assisted endovascular anterior communicating artery, and the distal had a poor recanalisation rate, less than 15%. branches of the right MCA was filled via pial Endovascular stenting in patients with thrombolysis of internal carotid collaterals from the right ACA. The right M1 carotid artery dissection has been successfully artery dissection and A1 segments were completely occluded. used in either selected cases with haemody- namically significant stenosis or when anti- Spontaneous dissection of the extracranial Proximal ICA recanalisation was achieved by implantation of two tandem self-expandable coagulation failed to prevent embolic internal carotid artery (ICA) is a major cause 23 of stroke with severe residual handicap in stents (Carotid Wall stent, Boston Scientific stroke. It permitted resolution of the young adults.1 Recently, stent supported Inc, Natick, MA), covering the suprabulbar stenosis with immediate recanalisation of the artery. After the procedure, there was angioplasty has been used to treat intimal cervical portion of the ICA (fig 1B). Sub- no need for anticoagulation and the patient dissection in case of neurological symptoms sequent intracranial angiography showed a was treated with antiplatelet agents. Our while on anticoagulation or as an alternative fresh thrombus in the right carotid siphon to the traditionally accepted use of anti- and confirmed the occlusion of the MCA. patient had acute hemiplegia with MCA coagulation.23 We report a case of internal Intra-arterial tissue plasminogen activator occlusion despite adequate medical therapy. carotid artery dissection causing hemiplegia (tPA) was infused directly into the thrombus Intravenous thrombolysis given within three successfully treated with emergent endovas- four hours after the onset of the new sym- hours of onset of symptoms might be an alternative treatment,8 but it would have cular stenting followed by intra-arterial ptoms (40 mg total). There was good prox- likely not been effective since ICA near thrombolysis. imal recanalisation with residual filling defects in some branches of the MCA occlusion would have been left in place; (fig 1C) and immediate clinical improvement. systemic thrombolysis was contraindicated Case report The patient received a 5000 U heparin bolus because of the anticoagulation therapy. A 44 year old right handed man was admitted during the procedure and then continuous We chose a new approach combining endo- to the emergency room after an acute episode 500 U/h infusion for 24 hours. Heparin ther- vascular stenting and intra-arterial thrombo- of left side weakness, which resolved within apy was then replaced by clopidogrel and lysis. Mechanical reopening of occluded large three hours. The patient had been well until aspirin, both 75 mg daily. On day 2, MRI vessels is currently being explored only in the onset of symptoms. There was no history showed brain infarct in the deep MCA terri- carefully selected cases of acute stroke mana- of trauma, strenuous exercise, hypertension, tories with asymptomatic haemorrhagic tran- gement to minimise the risk of haemorrha- or other medical problems. In the week prior sformation. The carotid artery and the MCA gic complications. By taking advantage of to admission, he reported intermittent head- were fully patent at ultrasound examina- the immediate recanalisation of completely copyright. aches and right sided neck pain after four tion with no evidence of restenosis. There occluded vessels it permits delivery of throm- days of diving. A cranial computed tomogra- were no periprocedural complications and the bolytic agents directly in the clot, maximising phy (CT) scan was normal. Diffusion patient’s symptoms improved gradually after the chance of total distal recanalisation. weighted imaging (DWI) of the brain per- the procedure. On day 7, he was discharged Although so far no clinical trial has formed six hours after the onset of symptoms on aspirin and clopidogrel with no residual documented the efficacy of emergent revas- while the patient was asymptomatic showed symptoms. cularisation in the setting of acute stroke, no evidence of infarction. T2-weighted mag- accumulated anecdotal data show that netic resonance images and fat suppressed endovascular mechanical revascularisation is images showed a semilunar hypersignal of a Discussion likely to become an important alternative mural haematoma in the infrapetrous seg- Ischaemic stroke in patients with ICA dissec- therapeutic approach in properly selected ment of the right ICA suggestive of a tion mainly results from thromboembolic, or, stroke patients. A potential disadvantage of less frequently, haemodynamic mechanisms.4 mechanical reopening is the production of dissection. Low molecular weight heparin 9 treatment (enoxaparin sodium 1 mg/kg sub- Formation of a false channel in the vessel embolic debris. With stent deploying in cutaneously every 12 hours) was started to wall or endothelial damage may favour for- dissected carotid artery, there is a theoretical http://jnnp.bmj.com/ obtain true anticoagulation. mation of a local thrombus, which becomes risk of the intramural clot contained within Three hours later, the patient had sudden less adherent and prone to embolise distally.1 the dissected segment breaking into the left sided hemiplegia, hypaesthesia, hemia- Although no general agreement exists on the cerebral circulation leading to embolisation nopia, and hemineglect. The National best management of extracranial carotid distally. In our case, the MCA emboli were Institutes of Health Stroke Scale (NIHSS) artery dissection, and because of the threat demonstrated prior to stenting using trans- score was 12. Transcranial Doppler ultra- of an embolic complication, anticoagulation cranial Doppler ultrasound. Another alterna- sound did not show any flow signal from the with heparin followed by oral warfarin is tive to intra-arterial thrombolysis could be middle cerebral artery (MCA) pointing to an used in most institutions.5 In case of an the use of mechanical thrombectomy devices to remove the clot from the carotid siphon acute occlusion. Because of the likely poor embolic complication in a patient with on September 26, 2021 by guest. Protected and MCA. This case report is to our knowledge the first example of the potential use of stenting followed by intra-arterial thrombolysis to treat and cure symptomatic carotid artery dissection. However, no conclusions can be drawn about the safety of endovascular app- roach in this clinical setting. Further evalua- tion is needed to address its risk–benefit ratio.

H Abboud Department of Neurology and Stroke Center, Bichat Figure 1 (A) Baseline right common carotid artery (CCA) angiogram shows near occlusion distal University Hospital and Medical School, Denis Diderot University-Paris VII, France to the origin of the infrapetrous segment of the right internal carotid artery (ICA). (B) Post stenting right ICA angiogram demonstrates complete resolution of the ICA occlusion and shows the E Houdart thrombus in the carotid siphon and in the MCA. (C) Right ICA angiogram after 40 mg of intra- The Department of Interventional Neuroradiology, arterial tissue plasminogen activator, recanalisation of the carotid siphon and of the M1 and M2 Lariboisiere Hospital, Denis Diderot University-Paris segment of the MCA and partial recanalisation of some branches of the MCA. VII, France

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E Meseguer, P Amarenco Disorders and Stroke rt-PA Stroke Study Group. 81%, respectively. In all, there had been 69 Department of Neurology and Stroke Center, Bichat N Engl J Med 1995;333:1581–7. requests received for treatment advice in the University Hospital and Medical School, Denis Diderot 9 Qureshi AI. Endovascular treatment of preceding three months. Referral for gui- University-Paris VII, France cerebrovascular diseases and intracranial dance from neurologists amounted to 26% of neoplasms. Lancet 2004;363:804–13. the total number of cases predicted by Correspondence to: Halim Abboud, Department of incidence studies.1 Neurology and Stroke Center, Bichat Hospital, 46 rue Bell’s palsy: a study of the Only 5% of neurologists said they would Henri Huchard, F-75018 Paris, France; halim. always see the patient, with further 29% if [email protected] treatment advice given by atypical features were present. The use of doi: 10.1136/jnnp.2004.041863 Neurologists steroids depended strongly on the stage of presentation, 76% giving steroids within 24 Competing interests: none declared Bell’s palsy is defined as an isolated unilateral lower motor neurone facial weakness of no hours of onset, 62% within three days, and References obvious cause. The incidence has been only 28% up to seven days. Fewer gave estimated at around 23 to 25 cases per steroids in certain subcategories (12% in 1 Schievink WI. Spontaneous dissection of the 100 000 population annually.1 Although the pregnancy, 19% in Ramsay Hunt syndrome, carotid and vertebral arteries. N Engl J Med prognosis is generally good, around 16% are 62% in a complete syndrome, and 45% in a 2001;344:898–906. left with varying degrees of permanent partial syndrome). 2 Malek AM, Higashida RT, Phatouros CC, et al. 2 Endovascular management of extracranial carotid disability. The steroid regimen advised was variable, artery dissection achieved using stent angioplasty. The use of steroids and acyclovir in the with most advocating 40 to 60 mg of AJNR Am J Neuroradiol 2000;21:1280–92. treatment of Bell’s palsy has been addressed prednisolone, with or without a tapering 3 Cohen JE, Leker RR, Gotkine M, et al. Emergent in two recent Cochrane reviews.34 These dose. Only 20% of neurologists gave acyclovir stenting to treat patients with carotid artery found no benefit from either but concluded in every instance; a further 20% gave it if dissection: clinically and radiologically directed that available studies were insufficiently there was evidence of Ramsay Hunt syn- therapeutic decision making. Stroke powered to detect a treatment effect. drome. 2003;34:e254–7. Neurologists are often asked by primary On the whole the responses from consul- 4 Lucas C, Moulin T, Deplanque D, et al. Stroke patterns of internal carotid artery dissection in 40 care physicians for treatment advice and in tants and registrars were similar. However, patients. Stroke 1998;29:2646–8. view of this uncertainty we were interested in while both advised steroids early on, con- 5 Lyrer P, Engelter S. Antithrombotic drugs for carotid studying the recommendations given. A sultants still recommended steroids up to artery dissection. Stroke 2004;35:613–14. questionnaire (appendix) was emailed to all seven days (42%) compared with only 6% of 6 Derex L, Nighoghossian N, Turjman F, et al. consultant neurologists (n = 35) and specia- specialist registrars (p = 0.009). Geographical Intravenous tPA in acute ischemic stroke related to list registrars (n = 21) in Scotland. Responses variability was evident; Glasgow neurologists internal carotid artery dissection. Neurology were collated at six weeks following an advised steroids more readily, with 95%, 74%, 2000;54:2159–61. interim reminder. Fisher’s exact test was and 21% giving them at 24 hours, three days, 7 Arnold M, Schroth G, Nedeltchev K, et al. Intra- used to compare groups; odds ratios with and seven days, respectively. This compared arterial thrombolysis in 100 patients with acute stroke due to middle cerebral artery occlusion. 95% confidence intervals and significance with 42% (p = 0.002), 42% (p = 0.065), and Stroke 2002;33:1828–33. were calculated (table 1). 17% (p = 0.34) at Edinburgh. There was also 8 Tissue plasminogen activator for acute ischemic Replies were received from 27 consultants a trend for Glasgow physicians to prescribe stroke. The National Institute of Neurological and 17 registrars, response rates of 77% and more acyclovir (21% v 11% (p = 0.37)). copyright.

Table 1 Treatment of Bell’s palsy by subcategory

‘‘Yes’’ responses by ‘‘Yes’’ responses by ‘‘No’’ responses by ‘‘No’’ responses by consultants registrars consultants registrars OR (95% CI) p Value

Do you see the patient? 1 1 14 9 0.642 (0.355 to 11.63) 0.5 Steroids within 24 h? 20 12 6 5 1.389 (0.347 to 5.55) 0.25 Steroids within 3 d? 17 9 9 8 1.68 (0.482 to 5.85) 0.18 Steroids within 7 d? 11 1 15 16 11.7 (1.35 to 102) 0.009 Steroids in pregnancy? 2 3 19 13 0.456 (0.067 to 3.12) 0.27 Steroids in Ramsay Hunt? 5 5 20 11 0.55 (0.13 to 2.32) 0.21 http://jnnp.bmj.com/ Steroids in complete syndrome? 18 10 8 7 1.58 (0.44 to 5.64) 0.20 Steroids in partial syndrome? 11 7 14 8 0.9 (0.25 to 3.25) 0.25 Do you give acyclovir? 6 3 14 9 1.29 (0.255 to 6.49) 0.30

‘‘Yes’’ responses by ‘‘Yes’’ responses by ‘‘No’’ responses by ‘‘No’’ responses by Edinburgh Glasgow Edinburgh Glasgow on September 26, 2021 by guest. Protected neurologists neurologists neurologists neurologists OR (95%CI) p Value

Do you see the patient? 20514NA Steroids within 24 h? 5 18 7 1 0.4 (0.004 to 0.403) 0.002 Steroids within 3 d? 5 14 7 5 0.255 (0.05 to 1.19) 0.07 Steroids within 7 d? 2 4 10 15 0.75 (0.11 to 4.9) 0.35 Steroids in pregnancy? 139140.52 (0.05 to 5.8) 0.39 Steroids in Ramsay Hunt? 2 5 10 12 0.48 (0.08 to 3.03) 0.26 Steroids in complete syndrome? 3 14 9 5 0.12 (0.02 to 0.63) 0.01 Steroids in partial syndrome? 39890.38 (0.08 to 1.89) 0.15 Do you give acyclovir? 138110.46 (0.04 to 5.3) 0.37

CI, confidence interval; OR, odds ratio.

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COMMENT 2 Peitersen E. The natural history of Bell’s palsy. carried out, and showed a Chiari malforma- Am J Otol 1982;4:107–11. tion with tonsillar herniation at C2 level and Although many neurologists advise steroids 3 Cochrane review. Acyclovir for Bell’s palsy and some would recommend acyclovir, the a cervical syrinx. (idiopathic facial paralysis). 2003;3. After posterior cranial fossa decompression uncertainty regarding the treatment of Bell’s 4 Cochrane review. Corticosteroids for Bell’s palsy palsy is reflected in our questionnaire (idiopathic facial paralysis). 2003;3. the patient recovered spontaneous breathing responses. and was off any ventilatory support within six hours. She and her child have been The majority of responders indicated that followed for two years following this event. their advice was not based on local guidelines Ondine’s curse during pregnancy She remains in good health with normal and many commented on the lack of evidence. We report a case of a 34 year old right handed polysomnographic testing. Some felt it was imperative to discuss the woman seen at 29 weeks’ gestation who uncertainty with the patient; others that better suffered from apnoea of unknown aetiology. randomised controlled trials are needed. This pregnancy, as well as her first gestation, COMMENT was complicated by generalised oedema and A new Scotland based randomised con- Ondine’s curse is a rare form of central high blood pressure. Starting at week 25, her trolled trial will start later this year (Morrison respiratory failure which includes severe husband noticed she had developed inter- J, personal communication). This study, sleep apnoea.1 We describe the case of mittent brief periods of apnoea only while coordinated by primary care physicians and recurrent sleep apnoea associated with preg- sleeping, which lasted as long as one minute ear, nose and throat surgeons, will compare nancy and delivery and related to a Chiari but of variable duration. Her husband awa- four treatment arms (comprising steroids, malformation that became symptomatic only kened her each time she had a protracted acyclovir, placebo) within 48 to 72 hours of during pregnancy. We hypothesise that the episode of apnoea. She was asymptomatic onset. Assessment of treatment effect will generalised oedema that occurred during while awake. In the 29th week she suffered a include photographs and questionnaires pregnancy, with a potentially mild increase more severe apnoea. She was intubated in about objective and subjective outcomes. in intracranial pressure, produced dysfunc- the field and taken to the hospital for an The aim is to recruit up to 720 patients, of tion of central chemoreceptors or the respira- emergency caesarean section. There was no whom 480 will have begun treatment within tory integrative system of the brain stem. spontaneous labour. Inability to breathe 48 hours of onset. Given the annual incidence Additionally there may have been compres- spontaneously persisted for two weeks post- of Bell’s palsy in Scotland, the researchers sion of the lower cranial nerves, impairing partum and a neurological consultation was input from peripheral chemoreceptors.2 estimate that this will take up to 18 months. requested. Related to this, our patient suffered severe Uncertainty in managing this condition can On initial evaluation blood pressure was sequelae from a central malformation that only be resolved by well conducted rando- 140/90 and the heart rate was 90 beats/min. was previously clinically inapparent. mised controlled trials. The neurological examination revealed Although Ondine’s curse has rarely occasion- upbeat nystagmus of small amplitude in the M Shaw ally described in patients with Chiari mal- primary position which did not change with formation,3 the expression of secondary sleep Glasgow Royal Infirmary, 10 Alexandra Parade, upward or downward gaze. She had lack of Glasgow G31 2ER, UK apnoea uniquely during pregnancy has not spontaneous breathing. She was fully awake been reported before. Finally we wish to and cooperative, sitting up in bed with no F Nazir, I Bone highlight the complete recovery of this assistance. While intubated she had an Southern General Hospital, Govan Road, Glasgow patient after posterior fossa decompression, obvious cough reflex but the gag reflex was as we found in another published case.4 Correspondence to: Dr Monica Shaw; not formally tested. Tongue examination copyright. [email protected] showed normal movement and power with J J Ochoa-Sepulveda, J J Ochoa-Amor no evidence of atrophy or fasciculation. Neurology Department, ‘‘Hospital Universitario Reina doi: 10.1136/jnnp.2004.048439 Otherwise, cranial nerve and sensorimotor Sofia’’, 14012 Cordoba, Spain examinations were entirely normal. There Competing interests: none declared. was normal tone, with downgoing plantar Correspondence to: Dr Juan Ochoa-Sepulveda; [email protected] APPENDIX reflexes and no evidence of other pyramidal findings. There was no record of arrhythmia. No yawning, vomiting, or hiccups were doi: 10.1136/jnnp.2004.048025 Bell’s palsy questionnaire present during the examination and they Competing interests: none declared 1. How frequently in the last 3 months have were not seen by nursing staff. you received a query from a GP about It was of interest that this patient had REFERENCES treatment of a Bell’s palsy? ______suffered from apnoea presenting immediately 2. Do you arrange to see the patient before after her first vaginal delivery two years 1 Severinghaus JW, Mitchell RA. Ondine’s curse: giving advice? Yes/No previously. This was treated with intubation failure of respiratory center automaticity while http://jnnp.bmj.com/ If you are satisfied with the clinical diagnosis: and resolved spontaneously with successful awake. Clin Res 1962;10:122. 3. Would you advise steroids extubation approximately four hours later. 2 Botelho RV, Bittencourt LR, Rotta JM, et al. Magnetic resonance imaging of the brain, Polysomnographic respiratory findings in patients with Arnold-Chiari type I malformation and N within 24 hours? Yes/No brain stem, and cervical spinal cord was basilar invagination with or without N within 3 days? Yes/No syringomyelia: preliminary report of a series of N within 7 days? Yes/No cases. Neurosurg Rev 2000;23:151–5. 3 Levitt P, Cohn MA. Sleep apnea and the Chiari I N in pregnancy? Yes/No malformation: case report. Neurosurgery

N with Ramsay Hunt syndrome? Yes/No 1988;23:508. on September 26, 2021 by guest. Protected N with complete facial palsy (loss of taste/ 4 Gupta R, Oh U, Spessot A, et al. Resolution of hyperacusis)? Yes/No Ondine’s curse after suboccipital decompression in a 72-year-old woman. Neurology N with partial facial palsy? Yes/No 2003;61:275–6. 4. If steroids are advised, what regime would you suggest? ______Gender influence on the 5. Is the advice you give on steroids based on progression of HTLV-I associated local guidelines? Yes/No 6. Would you advise acyclovir? Yes/No myelopathy/tropical spastic If yes: paraparesis 7. Is the advice you give on acyclovir based on HTLV-I associated myelopathy/tropical spas- local guidelines? Yes/No tic paraparesis (HAM/TSP) is a chronic and 8. Any additional comments: disabling disease caused by the human T- lymphotropic virus type I. Onset of the References Figure 1 Magnetic resonance imaging of the disease is insidious and the disease usually 1 1 Martyn CN, Hughes RAC. Epidemiology of brain (sagittal section) showing a Chiari progresses slowly over years. However, there peripheral neuropathy. J Neurol Neurosurg malformation with tonsillar herniation at C2 have been reports of the rapid evolution of Psychiatry 1997;62:310–18. level and a cervical syrinx. HAM/TSP over months or even weeks. The

www.jnnp.com PostScript 295 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.047357 on 14 January 2005. Downloaded from basis for these different progression patterns developed exclusively for the prospective The mean age at onset was 40.7 years and the is poorly understood and only a few studies assessment of HAM/TSP.3 We evaluated mean duration of disease was 12.5 years. have dealt with this matter.2 The present clinical progression using a disease progres- Comparison between the fast (n = 22) and study aimed at evaluating a Brazilian HAM/ sion index (DPI) defined as the IPEC the slow (n = 22) progression groups showed TSP population for possible factors implicated disability final score divided by the duration a significantly higher prevalence of women in in the progression of the disease. of the disease, from onset of symptoms, in the former group (p = 0.02). Statistical ana- years. We used this value to divide our lysis of other variables failed to show sample into quartiles. Patients whose values Methods significant differences. were under the 25th percentile were called To evaluate the possible role of sex We reviewed the files of 338 HTLV-I infected slow progressors and patients whose values patients evaluated at the outpatient clinic of hormones in this difference, because the were above the 75th percentile were called 4 the Reference Centers for Neurological mean age of menopause is around 50 years, fast progressors. Both groups were compared we compared men and women according to Infections and HTLV-I, Evandro Chagas for their demographic and clinical character- Clinical Research Institute (IPEC), age at onset of the disease (early onset: istics using Fisher’s test. DPI values were ,50 years; late onset: >50 years). The mean FIOCRUZ, Rio de Janeiro, Brazil. Patients compared using the Mann-Whitney U test. were included in the study if they fulfilled the DPI of women and men were, respectively, All p values were two sided and an a = 0.05 1.79 and 1.17 (p = 0.009) in the early onset World Heath Organization criteria for HAM/ was employed. TSP,1 but were excluded if they had con- group (n = 66; 66% women) and 2.59 and current infections or other disabling diseases 1.78 (p = 0.731) in the late onset group that could interfere with clinical progression. Results (n = 22; 68% women), suggesting that The eligible patients were submitted to a A total of 250 individuals were excluded due women have a faster progression than men clinical questionnaire and physical examina- to lack of neurological disease or the presence if the disease starts before the menopause. tion between June 2002 and February 2003. of concurrent infections. The mean age of the Clinical severity was evaluated using the remaining 88 individuals was 53.1 years, and IPEC disability scale (table 1), which was there were more women (68.2%) than men. Discussion This is the first study to suggest that HAM/ TSP progresses faster in women than in men. Table 1 The IPEC disability scale This difference seems to be particularly important in women whose disease started Motor score: Gait before the menopause. Although gender 0. Normal differences regarding the clinical evolution 1. Abnormal but can walk independently of HAM/TSP have not been reported before, 2. Abnormal and dependent on eventual unilateral support there has been evidence of a disproportional 3. Abnormal and dependent on permanent unilateral support number of women with this disease. Firstly, 4. Abnormal and dependent on eventual bilateral support there is a worldwide female to male pre- 5. Abnormal and dependent on permanent bilateral support ponderance of HAM/TSP patients ranging 6. Abnormal, dependent on permanent bilateral support, and occasional use of a wheelchair (WC) 5 7. Permanent use of a WC, stands up, and remains upright without support from 1.5:1 to 3.5:1. Secondly, Nagai et al 8. Permanent use of a WC, uses arms to stand up, and remains upright without support analysed the proviral load of 202 HAM/TSP patients and 243 asymptomatic HTLV-I car-

9. Permanent use of a WC, needs assistance from others to stand up and remain upright with copyright. support riers and found a significantly higher proviral 10. Permanent use of a WC, unable to stand up, exhibits voluntary movements of the lower limbs load in female patients when compared to when seated males.6 It is well known that a higher proviral 11. Permanent use of WC, unable to stand up, and does not have any voluntary movements of the load is associated with the development of lower limbs clinical disease. Motor score: Running The reason for the gender difference in our 0. Runs study is unknown, but it is possible that sex 1. Unable to run hormones play a role in the evolution of the Motor score: Climbing stairs disease. To test this hypothesis, we compared 0. Climbs 1. Climbs only when holding the handrail the DPI of patients whose disease had started 2. Unable to climb before and after the age of 50, the mean age Motor score: Jumping of the menopause. The finding of a signifi- 0. Jumps on one or two feet cantly worse evolution in the female group

1. Jumps on two feet, but not with only one with early onset of disease, coupled with no http://jnnp.bmj.com/ 2. Jumps on two feet only with hand support significant gender difference being observed 3. Unable to jump in the late onset group, suggests that female Spasticity score: Clonus hormones may be implicated in HAM/TSP 0. Absent pathogenesis and that their presence at 1. Only induced by the examiner higher levels may be associated with a faster 2. Spontaneous clinical progression. Further support for this Spasticity score: Flexor/extensor spasms idea is provided by the beneficial effects of 0. Absent danazol, an androgenic drug, in the treat- 1. Present 7 Sensory score: Paresthesias ment of some HAM/TSP cases. on September 26, 2021 by guest. Protected 0. Absent In summary, we found evidence of worse 1. Present, eventually clinical progression in women with HAM/TSP 2. Present, permanently compared to men. We hypothesise that sex Sensory score: Lumbar and/or lower limb pain hormones may account for this difference. If 0. Absent confirmed by further studies, this informa- 1. Present, eventually tion may lead to a better understanding of 2. Present during most of the day the mechanisms involved in HAM/TSP patho- Sphincter score: Bladder control genesis and suggest different treatment 0. Total options. 1. Urgency 2. Eventual incontinence or retention M A S D Lima, R B S Bica 3. Use of permanent catheter or regular use of relieve catheter Federal University of Rio de Janeiro (UFRJ), Sphincter score: Bowel continence Clementino Fraga Filho University Hospital (HUCFF), 0. Normal Rio de Janeiro, Brazil 1. Constipation 2. Incontinence or total retention, needs manual extraction or enemas A Q C Arau´jo Total: 0–29 Reference Centers on Neuroinfections and HTLV, Evandro Chagas Institute of Clinical Research (IPEC), FIOCRUZ, Rio de Janeiro, Brazil

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Correspondence to: Marco Antonio S D Lima, 109 cardinal manifestation, and included three marked decrease in plasma VEGF after Queensberry Street #12, Boston, MA 02215, USA; patients with polyarteritis nodosa, one with treatment (from 461 to 91.3 pg/ml and from [email protected] vasculitic neuropathy associated with Sjo¨gren 496 to 77.8 pg/ml, respectively). In the other syndrome, and one with non-systemic vascu- patient with polyarteritis nodosa, the plasma doi: 10.1136/jnnp.2004.035709 litic neuropathy. None of the patients were VEGF levels decreased mildly, from 313 to Competing interests: none declared on drug treatment at the time of sampling. 281 pg/ml. After disease remission was achieved by References treatment with corticosteroids or other immunosuppressants, we analysed plasma COMMENT 1 Osame M. Review of WHO Kagoshima meeting VEGF again in three of the patients, includ- Our results indicated that increased plasma and diagnostic guidelines for HAM/TSP, 1st ed. ing two with polyarteritis nodosa and one VEGF could be a useful marker for the New York: Raven Press, 1990. with Sjo¨gren syndrome. As a control group, diagnosis of vasculitic neuropathy and for 2 Kuroda Y, Fujiyama F, Nagumo F. Analysis of monitoring a therapeutic effect. factors of relevance to rapid clinical progression we used plasma from 18 age matched healthy in HTLV-I-associated myelopathy. J Neurol Sci volunteers, eight patients with Guillain-Barre´ This is the first report to show a significant 1991;105(1):61–6. syndrome, five with chronic inflammatory increase in plasma VEGF levels in patients 3 Oliveira AL, Bastos FI, Afonso CR. Measurement demyelinating polyradiculoneuropathy with vasculitic neuropathy compared with of neurologic disability in HTLV-I associated (CIDP), and seven with amyotrophic lateral other neuropathies. As our patients with myelopathy: validation of a new clinical scale. sclerosis, after obtaining informed consent. vasculitic neuropathy did not have cancer or AIDS Res Hum Retroviruses 2001;17(suppl):S65. Patients with diabetes mellitus or cancer diabetes mellitus, and as the plasma VEGF 4 Greenblatt RB. Fertility in the middle-aged were not included in the study. concentrations were significantly decreased woman. IPPF Med Bull 1980;14(4):2–4. Venous blood was sampled into an EDTA after treatment, we consider that VEGF 5 Gessain A, Gout O. Chronic myelopathy would be secreted into blood by the vasculitic associated with human T-lymphotropic virus type I tube with minimal stasis. The sample was (HTLV-I). Ann Intern Med 1992;117(11):933–46. centrifuged and the plasma VEGF concentra- lesions in this conditions. We could find no 6 Nagai M, Usuku K, Matsumoto W, et al. Analysis tion was determined by a quantitative sand- significant increase in plasma VEGF levels in of HTLV-I proviral load in 202 HAM/TSP patients wich-enzyme immunoassay technique using CIDP, Guillain-Barre´ syndrome, or amyo- and 243 asymptomatic HTLV-I carriers: high a Quantikine kit (R&D Systems, Minneapolis, trophic lateral sclerosis. Vasculitic neuropa- proviral load strongly predisposes to HAM/TSP. Minnesota, USA). As VEGF is secreted by thy may present with clinical manifestations J Neurovirol 1998;4(6):586–93. platelets in the clotting process,5 we mea- similar to CIDP or other peripheral neuropa- 7 Harrington WJ Jr, Sheremata WA, Snodgrass SR, sured plasma samples, not sera, to evaluate thies.1 The increase in plasma VEGF could be et al. Tropical spastic paraparesis/HTLV-1- the circulating VEGF level precisely. a helpful marker to distinguish vasculitic associated myelopathy (TSP/HAM): treatment neuropathy from CIDP and other peripheral with an anabolic steroid danazol. AIDS Res Hum Differences between the groups were Retroviruses 1991;7(12):1031–4. tested by the Kruskal–Wallis test and the neuropathies in such patients. Mann–Whitney U test. Differences were Although our results indicate the potential considered significant when the probability value of plasma VEGF as a marker in the Plasma VEGF as a marker for the (p) value was ,0.05. Significance tests for diagnosis and treatment of vasculitic neuro- diagnosis and treatment of group differences were computed with pathy, the significance of the results is StatView v5.0 (SAS Institute, Cary, North limited by the relatively small number of vasculitic neuropathy Carolina, USA). patients. Further studies with a larger study

Vasculitic neuropathy is treatable with The mean (SD) plasma VEGF concentra- population are necessary to confirm our copyright. immunotherapy. However, histological evi- tions in patients with vasculitic neuropathy results. dence of vasculitis is not always obtained (303 (182) pg/ml) were significantly higher from nerve and muscle biopsies.1 In particu- than in the healthy controls (30.9 (31.7) pg/ K Sakai, K Komai, D Yanase, M Yamada lar, in cases of non-systemic vasculitic neuro- ml) (p,0.01) as well as in patients with Department of Neurology and Neurobiology of pathy showing no or minimum abnormal Aging, Kanazawa University Graduate School of Guillain-Barre´syndrome (85.7 (57.3) pg/ml) Medical Science, Kanazawa 920-8640, Japan findings in serological tests, negative biopsy (p,0.05), CIDP (49.9 (48.3) pg/ml) (p,0.05), results cause considerable difficulty in the and amyotrophic lateral sclerosis (88.1 (55.7) Correspondence to: Dr K Sakai; ksakai@med. 1 diagnosis. pg/ml) (p,0.05) (fig 1). There was no kanazawa-u.ac.jp Vascular endothelial growth factor (VEGF) statistical difference in plasma VEGF con- is a potent, multifactorial cytokine.2 VEGF is centrations between healthy controls and doi: 10.1136/jnnp.2004.047571 derived from endothelial cells and pericytes patients with CIDP, Guillain-Barre´syndrome, Competing interests: none declared in response to hypoxia, and induces angio- or amyotrophic lateral sclerosis. The plasma genesis and microvascular hyperpermeability VEGF concentrations in patients with vascu- 2 References through its binding to VEGF receptors. litic neuropathy before treatment (423 (97.1) http://jnnp.bmj.com/ Vascular involvement by vasculitic neuropa- pg/ml) decreased significantly after success- 1 Collins MP, Periquet MI, Mendell JR, et al. thy results in hypoxia. It was reported that ful treatment with corticosteroids or other Nonsystemic vasculitic neuropathy insights from a VEGF was overexpressed in vasculitic lesions immunosuppressants, to 150 (114) pg/ml clinical cohort. Neurology 2003;61:623–30. in biopsied sural nerves, and that plasma (p,0.05). One case with polyarteritis nodosa 2 Nomura M, Yamaguchi S, Harada S, et al. VEGF levels were found to be raised in and the patients with vasculitic neuropathy Possible participation of autocrine and paracrine dermatomyositis with peripheral neuropathy.3 associated with Sjo¨gren syndrome had a vascular endothelial growth factors in hypoxia- These findings suggest that VEGF levels may be induced proliferation of endothelial cells and pericytes. J Biol Chem 1995;270:28316–24. increased in patients with vasculitic neuropa- 3 Matsui N, Mitsui T, Endo I, et al. Dermatomyositis thy. Although an increase in plasma or serum 600 with peripheral nervous system involvement: on September 26, 2021 by guest. Protected VEGF concentrations has been reported in activation of vascular endothelial growth factor some patients with systemic vasculitis,3 there 500 (VEGF) and VEGF receptor (VEGFR) in vasculitic have been no studies to evaluate plasma VEGF lesions. Intern Med 2003;42:1233–9. in a series of patients with vasculitic neuro- 400 4 Watanabe O, Arimura K, Kitajima I, et al. Greatly raised vascular endothelial growth factor pathy. With respect to VEGF levels in neuro- 300 pathies, a marked increase in serum levels was in POEMS syndrome. Lancet 1996;347:702. 5 Maloney JP, Silliman CC, Ambruso DR, et al. In 200 reported in the Crow-Fukase (POEMS) syn- vitro release of vascular endothelial growth factor drome.4 In addition, alterations in VEGF are VEGF (pg/ml) 100 during platelet aggregation. Am J Physiol associated with cancer and diabetes mellitus, 1998;275:1054–61. and VEGF is involved in the angiogenesis of 0 these diseases. VN Healthy GBS CIDP ALS In this study, we investigated the plasma control Acute aspiration pneumonia due VEGF concentrations in patients with vascu- to bulbar palsy: an initial litic neuropathy in comparison with other Figure 1 Plasma levels of vascular endothelial manifestation of posterior fossa neuropathies. After obtaining informed con- growth factor (VEGF) in patients with vasculitic sent, samples were obtained from five neuropathy (VN), healthy controls, Guillain- convexity meningioma patients with vasculitic neuropathy con- Barre´syndrome (GBS), chronic inflammatory False localising signs of intracranial lesions firmed by muscle or sural nerve biopsies. demyelinating polyradiculoneuropathy (CIDP), are defined as signs not generally associated They all presented with neuropathy as a and amyotrophic lateral sclerosis (ALS). with disturbances of function at the site of

www.jnnp.com PostScript 297 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.047357 on 14 January 2005. Downloaded from the lesion.12 An intracranial tumour which left side on tandem walking suggested invol- shifting to the opposite side causing tentorial has not metastasised may give rise to focal vement of the cerebellar system. In view of notching, pressure at the rim of the foramen signs of disordered nervous function at a these findings, a left posterior fossa mass lesion magnum, pressure at points of emergence of distance from itself in a number of ways. involving the lower cranial nerves such as a cranial nerves, and involvement of the Even though these neurological signs are schwannoma was suspected. However, mag- corticospinal tract.6 Gassel pointed out that labelled as false localising signs, it is impor- netic resonance imaging (MRI) of the brain false localising signs are commonly asso- tant to be aware that such signs are in no way revealed a large isointense homogenously ciated with intracranial meningiomas as they ‘‘false’’.3 Various cranial nerve palsies have enhancing mass lesion attached to the con- are discrete tumours that tend to compress been reported as false localising signs, with vexity dura (fig 1). It also revealed evidence and displace the brain rather than infiltrate the sixth cranial nerve being the most of herniation of the cerebellar tonsils below cerebral tissues.1 He also commented upon common.1 According to Gassel, ninth to the margin of the foramen of magnum and the rigidity of the bony skull and its dural 12th cranial nerve palsies never provide false anterior displacement of the cerebellum caus- compartments, as well as the outcome of localisation.1 Since Dodge reported the first ing stretching of the lower cranial nerves on the pressure within the skull causing movement case of false localising sign involving the left side. The patient underwent midline sub- of parts of the brain towards the tentorial lower cranial nerve, only two cases have been occipital craniectomy and total excision of the opening and foramen magnum resulting in 1 reported in the literature.4 We report a third lesion. The cerebellum was found compressed herniation pressure on various blood vessels. case of false localising sign involving the left and deeply indented by the tumour. Posto- O’Connell suggested that displacement of the ninth and 10th cranial nerves. peratively he improved neurologically. His gag brainstem by the tumour results in slacken- A 29 year old man presented to the medical reflex and palatal movements progressively ing of the horizontally directed nerves such department of our hospital with history of improved and he was asymptomatic at the seventh to 11th cranial nerves and hoarseness of voice of 15 days duration, 2 month follow up. stretching of the anteriorly directed nerves such as the fifth and sixth around the lateral dysphagia of 1 week duration, and cough False localising signs are unexpected neu- 7 with expectoration and respiratory distress of rological deficits and reflect pathology distant margin of the dural foramen. Matsuura and 2 days duration together with history of from the expected anatomical locus. Promi- Kondo proposed that displacement rather than rotation of the brainstem, causing com- fever. On examination, he was febrile, with nent false localising signs are less common pression and/or angulation of the affected a pulse of 100 bpm and blood pressure of 120/ today, as diagnosis is usually made at an early nerve rather than stretching or traction, is the 80 mm Hg. Respiratory examination revealed stage.4 Cranial nerve involvement as a false most significant factor for inducing contral- bilateral coarse crepitations. Neurological localising sign is found in 12.5% of cerebral ateral trigeminal neuralgia and hemifacial examination revealed absent gag reflex on tumours.2 According to Gassel, false localising spasm.5 However, according to Haddad and the left side with deviation of the palate to signs are more common in patients with signs Taha, rotation of the brainstem shifts the the right side without any other neurological of raised intracranial pressure. Due to the long basilar artery or loop of its branches close to deficit. Indirect laryngoscopic examination intracranial course, sixth cranial nerve palsy is the trigeminal nerve at its root entry zone and revealed paralysis of left vocal cord. commonly associated with supratentorial mass 8 1 causes trigeminal neuralgia. Haematological examination revealed lesions as a false localising sign. Most reports Most cranial nerve dysfunctions presenting haemoglobin (Hb) 13.6%, a WBC count of described single cranial nerve disturbance as a as false localising signs appear as hypoactive 16 800/mm, and an erythrocyte sedimenta- false localising sign. Rarely have multiple dysfunctions.16 Rarely has hyperactive dys- tion rate (ESR) of 120 mm/h. Chest x ray of cranial nerve palsies been reported as false function syndrome involving the cranial the patient revealed bilateral pneumonitis. He localising signs.45

nerve, such as trigeminal neuralgia or hemi- copyright. was treated with antibiotics according to Ehni proposed various mechanisms facial spasm, been reported in the litera- culture sensitivity. He progressively improved responsible for false localising signs. These ture.58 False localising sign involving lower and was discharged. At discharge, he had include: (i) general compression of a nerve cranial nerves is extremely rare with just two persistent hoarseness of voice and vocal cord having a long course; (ii) meningitis; (iii) cases reported in the literature.4 Maurice- palsy on the left side. About 4 weeks later he oedema and gliosis; (iv) metastatic deposits; Williams proposed two mechanisms causing presented with a history of bifrontal head- (v) infarctions at a distance from the primary lower cranial nerve palsy: firstly, cerebellar ache and was referred to our department. lesion due to occlusion of a vessel by a hemisphere impacting the foramen magnum, Neurological examination revealed bilateral neoplasm or by cerebral herniation through a causing reaction and oedema and thereby papilledema and left palatal palsy with dural aperture; (vi) gross brain displacement compression of the lower cranial nerve and absent gag reflex. Other cranial nerves were involving the brainstem and causing traction secondly, displacement of the cerebellum to normal. Motor and sensory system examina- of cranial nerves and kinking of cranial the contralateral side, forcing the brainstem tion was normal. Occasional swaying to the nerves over vessels; and (vii) brain stem to the ipsilateral side and thus exerting traction on the contralateral lower cranial 4

nerve. The case reported here involved a http://jnnp.bmj.com/ large cerebellar convexity meningioma caus- ing cerebellar herniation downwards into the foramen magnum and anteriorly into the lateral cerebello-medullary cistern which resulted in stretching of the lower cranial nerves. The cranial nerve function improved following excision of the tumour. Awareness of the possibility of false localising signs and the conditions in which they are most likely to occur is very important as they may be on September 26, 2021 by guest. Protected indicative of serious life threatening pathol- ogy within the neural pathway.3

S N Shenoy, A Raja Department of Neurosurgery, Kasturba Medical College and Hospital, Manipal, India

Correspondence to: Satyanarayana N Shenoy, Department of Neurosurgery, Kasturba Medical College and Hospital, MANIPAL–576 119, UDUPI, Karnataka, India; [email protected]

doi: 10.1136/jnnp.2004.040444 Competing interests: none declared Figure 1 MRI of the brain. (A) Sagittal section shows herniation of the tonsils below the foramen magnum due to an isointense mass lesion (arrow). (B) Axial T1 weighted image showing isointense mass lesion, enhanced homogenously with gadolinium, causing compression of the cerebellum and References anterior displacement of the cerebellum into the lateral cerebello-medullary cistern resulting in 1 Gassel MM. False localizing signs. A review of the stretching of the lower cranial nerves (arrow). concept and analysis of the occurrence in 250

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cases in intracranial meningioma. Arch Neurol above paediatric patients reporting an inci- never fully regain consciousness but 1961;4:526–54. dence of syrinx formation of 4%. The devel- remain in a vegetative or minimally con- 2 Collier J. The false localizing signs of intracranial opment of Chiari 1 and syringomyelia scious state.1 Patients in the vegetative state tumours. Brain 1904;27:490–505. formation following lumboureteral shunting may appear at times to be wakeful, with 3 Larner AJ. False localising signs. J Neurol Neurosurg Psychiatry 2003;74(4):415–8. for the treatment of pseudotumour cerebri is cycles of eye closure and eye opening 4 Maurice-Williams RS. Multiple crossed false recognised but has been less commonly resembling those of sleep and waking, 245 localizing signs in a posterior fossa tumour. reported. but show no sign of awareness or of a J Neurol Neurosurg Psychiatry 1975;38:1232–4. There is a small number of papers reporting functioning mind.2 In contrast, patients 5 Matsuura N, Kondo A. Trigeminal neuralgia and chiari development following lumbar shunt- considered to be in a minimally conscious hemifacial spasm as false localizing signs in ing for communicating hydrocephalus in state are said to show inconsistent but patients with a contralateral mass of the posterior children, but only two case reports of definite evidence of awareness despite pro- cranial fossa. Report of three cases. J Neurosurg syringomyelia formation. 3 1996;84:1067–71. found cognitive impairment. 6 Ehni G. False localizing signs in intracranial The association of syrinx formation and At present, the pathophysiology underly- tumour. Report of a patient with left trigeminal cerebellar tonsillar descent through the fora- ing the vegetative and minimally conscious 6 palsy due to right temporal meningioma. Arch men magnum is well described, and is states is unclear, a standard treatment Neurol Psychiatry 1950;64:692–8. postulated to occur as a consequence of a approach is lacking, and very little has been 7 O’Connell JEA. Trigeminal false localizing signs cranial-spinal CSF pressure gradient and diver- discovered to advance rehabilitation techni- and their causation. Brain 1978;101:119–42. sion of CSF down the central canal of the spinal ques. It is widely acknowledged that active 8 Haddad FS, Taha JM. An unusual case of cord rather than over the cerebral convex- rehabilitation should begin early in the trigeminal neuralgia: contralateral meningioma of 346 ities. It would seem remarkable that this intensive care setting, and should be applied the posterior fossa. Neurosurgery complication is not seen more commonly in the 1990;26:1033–8. to all patients (including those who remain treatment of pseudotumour cerebri. mechanically ventilated). However, this is The non-resolution of the syrinx, in our not yet routine practice. Several reports have Acquired Chiari 1 malformation case following lumboperitoneal shunt remo- highlighted the generic benefits of early and syringomyelia following val, is consistent with other workers’ experi- rehabilitation,4 however, the benefits of ences, although resolution has been reported specific interventions remain to be demon- lumboperitoneal shunting for 7 in one instance. strated. Over the last year, our group has pseudotumour cerebri In conclusion, we describe the development investigated the effects of postural change on of Chiari 1 deformity and syrinx formation as An important but not widely recognised levels of arousal and awareness. an important but otherwise poorly recognised complication of lumboperitoneal shunting is A total of 12 patients (eight men, four complication of lumboperitoneal shunting in the development of a Chiari 1 deformity and women; mean age 49 years, range 19–71) patients with pseudotumour cerebri. syringomyelia. We present a case of a patient classified as either vegetative (n = 5) or mini- who developed symptomatic cerebellar ton- R Padmanabhan, D Crompton, D Burn, mally conscious (n = 7) according to interna- sillar descent and syrinx formation following 23 D Birchall tional guidelines were assessed using the treatment of pseudotumour cerebri with Wessex Head Injury Matrix (WHIM), a 62 lumboperitoneal shunting. Regional Neurosciences Centre, Newcastle upon Tyne, UK point score, which records the recovery of behaviours in brain injured patients.5 Pati- Correspondence to: Daniel Birchall, Neuroradiology, ents were assessed lying in bed, during a 20 Case report Newcastle General Hospital, Newcastle upon Tyne minute period of standing using a tilt table at copyright. A 31 year old woman was diagnosed with NE4 6BE, UK; [email protected] 85˚, and again while lying in bed. During the pseudotumour cerebri following development observations blood pressure was measured doi: 10.1136/jnnp.2004.051276 of headaches, loss of vision, and papilloe- using an oscillometric cuff. The observa- dema, in association with a cerebrospinal Competing interests: none declared tions were repeated over a one week period, fluid (CSF) opening pressure of 36 cm H2O. and the median highest ranked behaviour Cranial imaging showed an attenuated ven- References and median total number of behaviours tricular system and no other abnormality. In 1 Chumas PD, Armstrong DC, Drake JM, et al. observed were recorded. The local research particular, the posterior fossa was satisfactory Tonsillar herniation: the rule rather than the ethics committee approved all investigations. in appearance. She was treated with lumbo- exception after lumboperitoneal shunting in the Informed assent was obtained from the next peritoneal shunt insertion, with resolution of pediatric population. J Neurosurg of kin. her symptoms. 1993;78:568–73. Twelve months later, the patient reported a 2 Sullivan LP, Stears JC, Ringel SP. Resolution of 6 month history of left hemisensory loss, left syringomyelia and Chiari 1 malformation by arm weakness, and unsteadiness. Neuro- ventriculoatrial shunting in a patient with http://jnnp.bmj.com/ logical examination revealed wasting and pseudotumour cerebri and a lumboperitoneal reduced power of the intrinsic muscles of shunt. Neurosurgery 1988;22:744–7. the left hand, and left-sided hyperaesthesia 3 Fischer EG, Welch K, Shillito J. Syringomyelia to pin-prick. Magnetic resonance (MR) ima- following lumboureteral shunting for communicating hydrocephalus. J Neurosurg ging showed the development of cerebellar 1977;47:96–100. tonsillar descent and syringomyelia through- 4 Hart A, David K, Powell M. The treatment of out the cervico-thoracic spinal cord. The ‘acquired tonsillar herniation’ in pseudotumour patient underwent insertion of a low pressure cerebri. Br J Neurosurg 2000;14:563–5.

ventriculoperitoneal shunt and removal of 5 Johnston I, Jacobson E, Besser M. The acquired on September 26, 2021 by guest. Protected the lumboperitoneal shunt, with subsequent Chiari malformation and syringomyelia following symptomatic improvement. There was, how- spinal CSF drainage. Acta Neurochir ever, no resolution of the syrinx on follow up 1998;140:417–28. MR imaging. 6 Williams B. The distending force in the production of ‘communicating syringomyelia’. Lancet 1969;4:189–93. Discussion 7 Peerless SG. Commentary. Neurosurgery 1988;22:747. The development of cerebellar tonsillar des- cent is a recognised but rarely reported complication following lumboperitoneal Effect of posture on levels of shunting,1–5 usually in the treatment of Figure 1 The median highest ranked communicating hydrocephalus.135 It has arousal and awareness in behaviour and total number of behaviours been reported to occur in a large proportion vegetative and minimally observed in the lying and standing positions for of paediatric patients undergoing this proce- conscious state patients: a both vegetative and minimally conscious dure, with Chumas et al reporting a 70% patients. The highest rank (p = 0.008) and total incidence in this age group,1 but its incidence preliminary investigation number of behaviours (p = 0.0013) observed in the adult population is undefined. The Moderate to severe brain injury is estimated increased significantly in the standing position. development of secondary syringomyelia to occur in 25 individuals per 100 000 Error bars indicate the interquartile range. appears to be much less common, with the population every year. Of these, 10–20% WHIM, Wessex Head Injury Matrix.

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Table 1 Highest ranked behaviours recorded in the supine and standing BOOK REVIEWS positions for each patient Neurological disorders in pregnancy Supine Standing Patient VS/MCS* score Behaviour observed score Behaviour observed Edited by Jacqueline M Washington. Published 1 VS 43 Smiled 43 Smiled spontaneously by the Parthenon Publishing Group, 2004, 2 VS 4 Eyes held by painful stimulus 4 Eyes held by painful stimulus ,2s £87.00 (hardcover), pp 150. ISBN 1-84214- ,2s 189-9 3 VS 5 Looked at person briefly 26 Frowned/grimaced during physio 4 VS 1 Eyes opened briefly 49 Vocalised in response to pain Many neurological disorders occur in women 5 VS 14 Yawned, sighed 26 Frowned/grimaced during physio of childbearing age. This small book is 6 MCS 13 Looked at person moving 16 Turned eyes to look at person talking designed to bridge the wide gap existing limbs ,3s between the disciplines of neurology and 7 MCS 20 Vocalised during physio 36 Switched gaze from one person to another obstetrics. It provides a concise overview of 8 MCS 26 Frowned/grimaced in response 34 Monosyllabic response to questions the most common neurological disorders that to pain may be seen during pregnancy. Three cate- 9 MCS 14 Yawned, sighed 14 Yawned, sighed gories of problems are encountered by obste- 10 MCS 18 Tracked for 3–5 seconds 28 Looked at object when requested tricians and neurologists: management of 11 MCS 8 Made eye contact 23 Showed selective response to neurological pre-existing disorders during pre- preferred people 12 MCS 42 Could find a card from four 43 Smiled spontaneously gnancy, neurological disorders directly due to pregnancy, and neurological affections that *Patient classification at the time of recruitment is denoted VS (vegetative state) or MCS (minimally conscious state). require special treatment considerations during pregnancy. The book covers these three cate- gories with eight chapters devoted to migraine, cerebrovascular disease, epilepsy, back pain, We found that eight patients (three vege- D Badwan multiple sclerosis, peripheral nerve disorders, tative and five minimally conscious) showed Royal Leamington Spa Rehabilitation Hospital, myasthenia gravis, and central nervous infec- consistent improvements in the highest Warwick, UK tions. Each chapter includes consideration of ranked behaviours (table 1; p = 0.008) and the influence of pregnancy on the disorder, the total number of behaviours (p = 0.013) D Menon effect of the disorder on pregnancy, and observed in the standing position (fig 1). Wolfson Brain Imaging Centre, University of potential effects of proposed therapies on the Three patients (two vegetative and one Cambridge, Cambridge, UK developing foetus—all concerns shared by minimally conscious) showed no change every clinician who care for pregnant women. and one minimally conscious patient showed J Pickard The different chapters provide a useful resource only an increase in the highest ranked Wolfson Brain Imaging Centre, Cambridge, UK with lists of dosages, contraindications, mon- behaviour observed. Although WHIM scores itoring guidelines, and side effects of drugs in Correspondence to: L Elliott, Senior Physiotherapist, in three vegetative patients increased during pregnancy. Physiotherapy Department, Box 185, Addenbrooke’s Two other chapters, covering muscle dis- standing, the behaviours observed did not copyright. Hospital, Cambridge, CB2 2QQ, UK; louise.elliott@ eases (in particular myotonic dystrophy) and reach a level suggesting awareness of self addenbrookes.nhs.uk and/or environment. After standing the brain tumours, could have been useful. One WHIM scores in the supine position were All authors are members of the Cambridge Coma also could regret the nearly complete absence equal to or below those acquired before Study Group of figures or diagrams for a book intended standing. No change in blood pressure was not only for neurologists but also for obste- Authors’ contributions: tricians. In contrast, most chapters contain observed (p = 0.3). L Elliott—Principal investigator (data acquisition and Our preliminary results suggest that posi- many useful tables. patient recruitment). A few remarks are also worth mentioning. tional changes may have a significant impact A Shiel—Data acquisition and patient recruitment. on behaviours in vegetative and minimally M Coleman—Data analysis and manuscript For example, the section covering the course of migraine during and after pregnancy is conscious patients. Although the benefit of preparation sometimes redundant and could have been this phenomenon in rehabilitation remains B Wilson—Project supervision. D Badwan—Patient recruitment and data analysis. summarised. Post partum angiopathy should unproved, these findings have clear implica- D Menon—Project supervision. also be added to the aetiologies of postpartum tions for the assessment and categorisation of J Pickard—Project supervision. headaches. In the chapter on cerebrovascular patients. Neurological assessments used to http://jnnp.bmj.com/ disease, eclampsia and hemorrhage sections classify patients according to international doi: 10.1136/jnnp.2004.047357 could have been better detailed. guidelines relating to the vegetative and On the whole, this book represents a useful minimally conscious states typically take This work was funded by the Smiths Charity, the fund concise text (more than in-depth literature place with the patient lying in bed. Where for Addenbrooke’s and an MRC programme grant summary or detailed analysis of complex physical constraints permit, it may be impor- (number G9439390 ID 56833). issues) written in a balanced, practical, and tant to also observe patients in the standing informative way. It can be used by a wide Competing interests: none declared position. audience and will facilitate understanding and treatment of neurologic problems in pregnant women. on September 26, 2021 by guest. Protected Acknowledgement References The authors are grateful to Dr R Barker for the C Lamy neurological assessment. 1 Royal College of Physicians (UK). Rehabilitation following acquired brain injury—National Clini- cal Guidelines. London: Lavenham Press, 2003:8. The neuropathology of dementia, L Elliott 2 Royal College of Physicians (UK). The vegetative 2nd edition Department of Physiotherapy, Addenbrooke’s NHS state: guidance on diagnosis and management. A Trust, Cambridge, UK report of a working party of the Royal College of Physicians. Clin Med 2003;2:249–54. Edited by M Esiri, M-Y Lee, J Q Trojanowski. Published by Cambridge University Press, M Coleman 3 Giacino JT, Ashwal S, Childs N, et al. The minimally conscious state: definition and Cambridge, 2004, £195.00, pp 563. ISBN 0- Wolfson Brain Imaging Centre, University of diagnostic criteria. Neurology 2002;58:349–53. Cambridge, Cambridge, UK 5218-1915-6 4 Talbot LR, Whitaker HA. Brain-injured persons in an altered state of consciousness: measures and A Shiel Inspecting the hardcover graphics of this new intervention strategies. Brain Injury 1994;8(Pt edition your reviewer was startled to find Faculty of Medicine, National University of Ireland, 8):689–99. blazoned his comments on the previous, and Galway, Ireland Shiel A 5 , Horn SA, Wilson BA, et al. The Wessex first, edition to encourage your purchase. It is Head Injury Matrix (WHIM) main scale: a B A Wilson preliminary report on a scale to assess and therefore clear that I am a supporter of this MRC Cognition and Brain Sciences Unit, Cambridge, monitor patient recovery after severe head injury. enterprise in principle – though modesty will UK Clin Rehabil 2000;14:408–16. prevail and I will not flaunt my prescience in

www.jnnp.com 300 PostScript J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.047357 on 14 January 2005. Downloaded from remarking on the desirability of this latest, alarming jargon. Continuum-mechanical warp- There are different levels of reading whether and any subsequent, redrafting. ing using calculations based on Cauchy-Navier one searches for general data on a disease, I regret that this review will not take the equations with variable Lame´ elasticity coeffi- differential diagnoses, or more precise elements form of a direct comparison between the cients, and purple brains, add a new and such as a reference or pathophysiological present and previous editions since my distracting element to a neuropathology book. explanations. Illustrations are very helpful; recollection of the former is based on a However the integration of neuropathology in particular, the ones on oculomotor distur- fading and rose-tinted affection, rather than and neuroimaging data is a highly desirable bances and neuroradiological resources are direct consultation with the source material. goal in clinical neuroscience and it would be well chosen. This book can also be an excellent Obsessionality is a professional hazard in luddite to reject it here. This content illustrates source of inspiration for teaching. neuropathology—either acquired or innate— just how widely a ‘‘neuropathology’’ text needs All the doctors (from students to consul- but in my case it does not extend to detailed to cast its net to retain its value in such an tants) who have to walk through an emer- record keeping of book loans. I hope the interdisciplinary world as neurodegeneration gency department should have this book on trainee who chose to keep the book has had research and the clinical neuroscience of their bookshelves. A pocket edition of such much joy of it. No doubt his extended loan dementia. an educational book would be useful! reflects the esteem he/she felt for the educa- In summary, another triumph and an tional value of the first edition. indispensable addition to this field. If I had C Cordonnier There is no doubt that the present book is any hope of getting the book back I would considerably larger, reflecting significant automatically loan it to any new neuro- increased content. However there is no flab, pathology trainee, but its appeal is far and the overall size and scope are, respect- broader and it should be studied by anyone Cerebrovascular disease, cognitive ively, manageable and focussed. The book entering dementia research from a tissue- has acquired distinguished American co- based angle. For a quotable plug to blurb over impairment, and dementia editors in place of Dr James Morris (whose the sleeve of the third edition I offer the career trajectory has taken him deep into publisher: ‘‘buy one, get one free’’. Edited by J O’Brien, D Ames, L Gustafson, M F health service management) but much of Folstein, E Chui. Published by Martin Dunitz, James’s contribution remains, suitably L F Haas London pp406. updated, as a core of practical advice related to the diagnostic process in dementia neuro- The proportion of patients who will be victim pathology, and the particular pathologies of stroke or dementia is terrifying: after the associated with Alzheimer’s disease and age of 80 years, 1 in 5 people is affected by Vascular dementia. The latter section parti- Catastrophic neurological disorders dementia, and 1 in 10 hav‘e had a stroke or cularly reflects his welcome and homespun in the emergency department transient ischaemic attacks. The burden of wisdom in an area fraught with unresolved stroke and dementia will continue to increase during the next 20 years in western countrie, problems of clinicopathological correlation. I Edited by E F M Wijdicks. 2004: Published by am glad they have retained it. Similarly the owing to increasing life expectancy. Oxford University Press, Oxford £70.00 (hard- Therefore, the economic burden of both contribution to the text by Professor Esiri back), pp 306. ISBN 0-19-516880-1 shares this feeling of direct personal tutoring disorders will also become a major public health issue. Stroke is an important cause of from an approachable expert. The editors Neurological conditions in the emergency have also retained the previous structure, cognitive impairment and dementia. Stroke department have to be quickly identified copyright. prevention, the only way to prevent vascular roughly summarisable as: what dementia is, because more and more therapeutic options dementia, may also be an effective way to where in the brain might be affected, how to are available. Most of the academic ‘‘prevent’’ Alzheimer’s disease—or at least to go about a pathological survey of a dementia approaches focus on diseases. Wijdicks offers prevent the anticipation of its clinical onset, brain, and, finally, what you might find an interesting and very practical insight of possibly due to the summation of vascular related to specific diagnostic categories. This neurology in the emergency room. This is the and Alzheimer lesions. Although the term comprehensive approach is now fleshed out second edition of catastrophic neurological ‘‘vascular dementia’’ appears in several chap- by the introduction of more authors to bring disorders in the emergency department, third ters, the editors discuss two other important expertise related to individuals’ conditions, book of a trilogy dedicated to critical care concepts. The first is the wide notion of additional ‘‘introductory’’ material about the neurology. Eight new chapters were added, ‘‘vascular cognitive impairment’’, which clinical genetics of dementia (styled ‘‘mole- seven of which appear in an entirely new first includes a large range of severity of cognitive cular diagnosis’’ for some reason) and neuro- section on the evaluation of presenting impairments associated with vascular lesions; imaging in dementia, and increased content symptoms indicating urgency. There is a final behind this term is the hope of an effective reflecting on pathogenesis and research into new chapter on forensic neurology. relevant disease models. The book is now an prevention. The second is the interaction The first part is original and very practical: http://jnnp.bmj.com/ between Alzheimer lesions and stroke, edited multi-author compilation rather than from the initial symptoms in the emergency explaining that many patients already have a more personal distillation from a small room such as ‘‘confused and febrile’’, the some degree of cognitive impairment before group. Looking at the arithmetic there are 27 evaluation of the patient, diagnosis orienta- stroke, which may be degenerative in origin USA authors, 14 UK, and four others from tion, algorithms for the choice of paraclinical in many cases. Australia and Scandinavia—the latter tools, and therapeutic issues are discussed. The book is divided into 26 chapters, empowered only to pronounce on alcohol One of the chapters I really found original is including classification and diagnosis, epide- and dementia and CADASIL. Bar this small the one entitled ‘‘Shortness of breath’’. This miology and risk factors, pathophysiology, non-cross Atlantic contingent the chapters question is not often taken into account in clinical features, assessment, and manage- work out at 11 USA and 10 UK, with two teaching regarding neurological disorders. If ment. The organization of the book proceeds on September 26, 2021 by guest. Protected mixed, illustrating a previously unrecognised you look for more precise data, you will find logically. All chapters end with the most American propensity for job sharing. ‘‘little boxes’’ that point out to more precise important references. The information is The content is uniformly well presented issues such as the role of the ascending made clear and is accurate. The target and informative. Referencing largely peters reticular activating system. audience consists of all care providers who out in 2002 indicating the long lead time for The second part is about how to evaluate treat patients with dementia or cerebrovas- this type of book but this is not a bad thing. conditions that can deteriorate (coma, acute cular disorders. Its length and its level of The modern tendency to rush into print with obstructive hydrocephalus, brain oedema). details make it appropriate for residents one’s latest minor observation on, for exam- The chapter on coma is exhaustive, with looking for a practical knowledge, and also ple, yet another apoptotic or oxidation illustrations and figures commenting on for trained specialists. This book will be of marker contributes little to the overall pro- different mechanisms. Toxicology is also very major interest for all those who treat patients gress of neuropathological research in neuro- present in this book. with cognitive decline or patients at risk. degeneration. The purpose of a book like this The last part is more conventional and is to record those aspects of the under- deals with most of the urgent neurological D Leys standing of dementia disorders that with- conditions such as ischaemic stroke, haemor- stand time and become part of the accepted rhagic stroke, and spinal cord injury. Unfor- D Leys has been paid or received funds for wisdom rather than twitching with every tunately, the author did not integrate a research during the last 5 years by Sanofi- modish straw in the wind. chapter on myasthenia or Guillain-Barre´syn- Synthelabo, AstraZeneca, Takeda, Lilly, and The imaging chapter is an especially fine drome, which are often under-recognised in Servier for educational programmes, speaking, thing with dazzling illustration and some the emergency department. and consulting.

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