532 J Neurol Neurosurg Psychiatry 2000;68:532–541 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from

previously described stratifications of disabil- Centre for Cell and Molecular Medicine, Postgraduate ity: firstly EDSS 0–5.5 v 6–10. An EDSS Medical School, Keele University, North StaVordshire LETTERS TO score of 6 (need for unilateral support) is Hospitals, Stoke-on-Trent ST4 7LN, UK clinically important and a clearly defined V L STEVENSON THE EDITOR division. It may therefore be subject to less S M LEARY interrater variability than other points on the A J THOMPSON EDSS. As long term prognosis may be more University Department of Clinical Neurology, The National Hospital for Neurology and Neurosurgery, No association between the APOE å4 clearly established in patients with a greater Queen Square, London WC1N 3BG, UK disease duration,5 patients with a disease allele and outcome and susceptibility in Correspondence to: Dr S J M Weatherby, Depart- duration of either >5yearsor>9 years primary progressive multiple sclerosis ment of Neurology and Neurophysiology, School of disease duration were included. For the pur- Postgraduate Medicine, Keele University, North Recent reports, primarily in patients with poses of comparison with previous data we StaVordshire Hospitals, Princes Road, Stoke-on- also used a second method of stratification Trent ST4 7LN, UK secondary progressive and relapsing- [email protected] remitting multiple sclerosis have suggested (EDSS score divided by disease duration to that the apolipoprotein (APOE) å4 allele may give a “severity score”). Patients were divided 1 Sylantiev C, Chapman J, Chilkevich O. The be associated with a worse prognosis.1–3 The å into those with “mild” and those with “aggressive” disease using the median “sever- APOE 4 allele and progression of disability in class of APOE is involved in lipid transport multiple sclerosis. Neurology 1998;50:A150, and plays a part in brain development, ity score” as the threshold.3 P03,060. synapse repair, and response to neuronal The Pearson ÷2 test was used to compare 2 Dousset V, Gayou A, Brochet B, et al. Apo E the 4 allele frequency between cases and polymorphism in multiple sclerosis. Multiple injury. It has been postulated that patients å Sclerosis 1998;4:357. possessing the å4 allele might have a reduced controls and between outcome groups. The 3 Evangelou N, Jackson M, Beeson D, et al. capacity for neuronal remodelling after mul- influence of the å4 allele on outcome was also Association of the APOE e4 allele with disease 3 studied by logistic regression analysis (Stata activity in multiple sclerosis. J Neurol Neurosurg tiple sclerosis relapses. Psychiatry 1999;67:203–5. There is general agreement that patients statistical package version 5.0, Stata Corp, 4 Thompson AJ, Polman CH, Miller DH, et al. with primary progressive multiple sclerosis Texas,USA). This allowed correction for sex, Primary progressive multiple sclerosis. Brain have a worse outcome both in relation to 1997;120:1085–96. disease duration, and age of onset, as each has 5 Weinshenker BG. The natural history of multi- development of disability over time and in previously been shown to influence ple sclerosis. In: Raine CS, et al,eds.Multiple mortality. Such patients do not have a relaps- outcome.5 sclerosis. Clinical and pathogenetic basis. London: ing course. Moreover, there are pathological Genotype distribution did not significantly Chapman and Hall, 1997:81–9. 6 Weatherby SJ, Carthy D, Mann CLA, et al. and imaging diVerences between primary diVer between the sexes (p=0.21). There was Severity and susceptibility of multiple sclerosis progressive and secondary progressive multi- no significant diVerence in å4 allele fre- related to polymorphisms of apolipoprotein E. ple sclerosis suggesting that primary progres- quency between cases and controls (p=0.95). J Neurol 1999;246:I–21. sive disease may be a less inflammatory form Furthermore, using t test analysis, possession 4 of the disease. The relation between APOE of the å4 allele did not significantly influence and outcome has not been specifically Transverse myelopathy, a rare the age of onset (p=0.78). complication of mixed connective tissue considered in patients with primary progres- Outcome was not significantly influenced disease: comparison with SLE related sive multiple sclerosis, although 11 patients by å4 allele possession (for EDSS >6, with primary progressive disease were in- transverse myelopathy p=0.71, n=34 at >5 year disease duration, cluded in one previous study.3 and p=0.59, n=23 at >9 year disease A possible role for the å4 allele in primary Transverse myelopathy is a rare complication duration, and for the “severity score” p=0.76, of mixed connective tissue disease (MCTD). progressive multiple sclerosis was therefore n=50). The table illustrates the frequency of studied in a well characterised cohort of 50 To date there have been relatively few reports, allele å4 possession in patients stratified 1–6 patients with primary progressive disease (32 all in females. We describe a case of according to outcome. Using logistic men, 18 women), all of whom were under the transverse myelopathy in a man with MCTD regression to correct for potential confound- care of the National Hospital for Neurology and review the previous cases, comparing ing factors (sex, age of onset, and disease and Neurosurgery and had been seen from them with transverse myelopathy related to duration), no significant eVect of the 4 allele their initial presentation. The mean age of å systemic lupus erythematosus (SLE). onset was 40.3 (range 18–63) years, mean was found on the likelihood of having an A 46 year old man with a history of a disease duration 8.7 (range 2–26) years, and EDSS of >6at>5or>9 years (OR=1.09, squamous cell carcinoma of his right upper http://jnnp.bmj.com/ the mean Kurtzke expanded disability status p=0.92, n=34; OR= 0.67, p=0.69, n=23 lobe bronchus anda2yearhistory of MCTD scale (EDSS) was 5.5 (range 2–8.5). All were respectively) or having a “severity score” was admitted to hospital with a 3 month his- white northern Europeans, recruited after greater than the median value (OR=1.24, tory of subacute progressive lower limb local ethics committee approval. No patient p=0.74, n=50). weakness, sensory loss, and sphincter distur- had received disease modifying therapy at the The results of this investigation in patients bance. He had been diagnosed with MCTD time of recruitment. Disability was measured with primary progressive disease suggest that on the basis of Raynaud’s phenomenon, pol- using the EDSS. In addition, age of onset, the å4 allele does not influence prognosis in yarthralgia, synovitis, acrosclerosis, raised sex, and disease duration were noted. One this group. When considered together with muscle enzymes, biopsy established myositis, hundred and fifty nine randomly selected our findings in a large cohort of 370 patients dysphagia for solids, positive test for antinu- on October 2, 2021 by guest. Protected copyright. healthy white subjects served as controls with relapsing-remitting and secondary pro- clear factor (ANF) (speck1ed 1/2560), nega- (60% women, 40% men, mean age 45.6 (SD gressive disease which also showed no tive DNA binding, and high titre antiribonu- 12.8 years). evidence of an association between allelic clear protein (anti-RNP). His bronchial DNA was isolated from leucocytes using a variation of APOE and disability,6 we suggest carcinoma had been treated surgically with a standard phenol/chloroform extraction it is unlikely that the å4 allele when right pneumonectomy and radiotherapy to method. APOE genotypes were determined considered alone significantly influences the bronchial stump at a low dose of 3000 after polymerase chain reaction (PCR) ampli- prognosis in multiple sclerosis. cgrad over 10 days. No lymph node involve- fication and Hha I restriction digestion. Frag- ment was identified either at operation or on S J M WEATHERBY CT. He had recently been prescribed a ments from PCR were separated on an 8% CLAMANN polyacrylamide gel. AAFRYER reducing course of prednisolone for active Associations between the å4 allele and dis- R C STRANGE myositis. There was no history of recent ease outcome were investigated using two C P HAWKINS infections. On examination he was systemically well. There was no clinical evidence of tumour The eVect of å4 allele possession on outcome (EDSS 0–5.5 v 6–10 at >5 years, and “severity score” recurrence. He had an asymmetric spastic median value) (percentages) paraparesis (power of 4–4+ MRC grading) with a sensory level at D10. The remainder of EDSS 0–5.5 EDSS 6–10 “Severity score” >5 years >5 years Total “Severity score” >median value Total the neurological examination was normal. disease disease number of spinal cord MRI with gadolinium duration duration patients “Mild” disease disease patients enhancement showed mild atrophy of the cord at D9/D10 but was otherwise normal. å4 Allele positive 6 (54.6) 5 (45.4) 11 (100) 7 (46.7) 8 (53.3) 15 (100) å4 Allele negative 11 (47.8) 12 (52.2) 23 (100) 18 (51.4) 17 (48.6) 35 (100) Routine haematology (full blood count, vita- min B12, and folate), inflammatory markers, J Neurol Neurosurg Psychiatry 2000;68:532–541 533 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from

Features of MCTD associated transverse myelopathy

Report Duration of 1st author MCTD (y) Age Sex Onset Site Treatment Neurological outcome Imaging Current 2 46 M Progressive over 3 Thoracic Pred/Aza No change Spinal MRI cord months atrophy Mok1 2 46 F Progressive Thoracic Pred/Aza/ Improved. Power increased Spinal MRI normal Anticoagulation from 3–4 over 4 months Flechtner2 5 19 F Progressive with a Thoracic and Pred/Aza/ Each “relapse” resolved. Spinal MRI high subsequent relapsing Cervical Plasmapheresis Sequlae: mild sensory signal intramedullary course disturbances lesion *Obara3 n/k 42 F Progressive Thoracic Pred/Aza Resolved. Sequelae: painful Spinal MRI abnormal tonic spasm *Yamaguchi4 n/k 53 F Progressive Thoracic Pred Improved n/k

Pedersen5 2 16 F Progressive over less Thoracic Pred/Aza Resolved. Sequelae: urge CT brain normal. than 1 week incontinence No spinal CT Weiss6 1 24 F Progressive over 4 Thoracic Pred/Aza No change n/k weeks

(Pred=Prednisolone; Aza=azathioprine; n/k=not known); *Japanese journal, English abstract only. and biochemistry were normal. ANF re- as progressive in all cases with MCTD, 1 Mok CC, Lau CS. Transverse myelopathy com- mained at high titre with anti-RNP antibody although the duration of symptom onset was plicating mixed connective tissue disease. Clin Neurol Neurosurg 1995;97:259–60. positivity. All other autoimmune markers and not precisely identified in every report. By 2 Flechtner KM, Baum K. Mixed connective serum angiotensin converting enzyme were contrast, however, a review of 20 cases of tissue disease: recurrent episodes of optic normal. A thrombophilia screen (protein C, transverse myelopathy related to SLE,9 myelopathy and transverse myelopathy. Suc- protein S, and antithrombin III, lupus cessful treatment with plasmapheresis. J Neurol showed that 50% evolved over less than a day. Sci 1994;126:146–8. anticoagulant, and anticardiolipin) was unre- Neurological improvement or resolution with 3 Obara K, Tanaka K. A case of mixed connective markable. Complement, creatinine phos- only mild sequelae followed treatment with tissue disease associated with transverse myeli- phokinase, and immunoglobulins were nor- tis responding to pulse steroid therapy. Rinsho high dose prednisolone and azathioprine in Shinkeigaku 1991;31:1197–201. mal, and a venereal disease research five of the seven cases of transverse myelopa- 4 Yamaguchi Y, Yoshimura T, Hosokawa S, et al. laboratory test was negative. Examination of thy related to MCTD. In this case and one A case of mixed connective tissue disease with CSF excluded infection and standard indices other in which there was a delay in presenta- subacute transverse myelopathy. Rinsho Shinkeigaku 1991;31:1099–102. were normal. Oligoclonal bands were nega- tion of over 4 weeks, neurological features 5 Pedersen C, Bonen H, Boesen F. Transverse tive and visual evoked potentials were nor- were stabilised but did not improve. The myelitis in mixed connective tissue disease. Clin mal. clinical course of transverse myelopathy Rheumatol 1987;6:290–2. He received high dose steroid therapy (1g/ 6 Weiss TD, Nelson JS, Woolsey RM, et al. Trans- related to SLE is variable, although histori- verse myelitis in mixed connective tissue day intravenous prednisolone for 3 days cally prognosis is relatively poor. In one series disease. Arthritis Rheum 1978;21:982–5. followed by an oral reducing dose) and was in which 70% of patients received steroid 7 Mok CC, Lau CS, Chan EY, et al. Acute trans- verse myelopathy in systemic lupus started on 125 mg azathioprine. There was treatment, 50% died in less than 6 weeks, no further deterioration in his condition and erythematosus: clinical presentation, treat- 35% had permanent neurological deficits, ment, and outcome. J Rheumatol 1998;25:467– at 6 year follow up his neurological condition and 15% had a near normal recovery.9 73. remains stable. No recurrence of carcinoma Another study8 described seven patients with 8 Harisdangkul V, Doorenbos D, Subramony SH. was detected over the same time period and Lupus transverse myelopathy: better outcome transverse myelopathy and SLE. Four died with early recognition and aggressive high-dose we think that a paraneoplastic cause can be and one remained wheelchair bound. In SLE, intravenous corticosteroid pulse treatment. J excluded. Similarly, radiation myelopathy is early aggressive therapy with high dose Neurol 1995;242:326–31. unlikely, as no vertebral abnormalities were 9 Andrianakos AA, DuVy J, Suzuki M, et al. steroids and cytotoxic agents has been seen on MRI and the patient had only a small Transverse myelopathy in systemic lupus associated with a satisfactory outcome.7810 erythematosus: a report of three cases and a radiation dose to a localised field. review of the literature. Ann Intern Med

Considering this patient together with pre- http://jnnp.bmj.com/ Transverse myelopathy is a rare complica- 1975;83:616–24. vious reports suggests that, by contrast with tion of MCTD and has not been previously 10 Chan KF, Boey ML. Transverse myelopathy in SLE, transverse myelopathy is unlikely to be a SLE: clinical features and functional outcomes. reported in a male. Postulated mechanisms presenting feature of MCTD. Onset is Lupus 1996;5:294–9. include vasculitis of small arachnoid arteries progressive rather than acute, and cervical of the cord and arterial thrombosis causing areas are less often involved.Transverse my- microinfarction of the spinal cord because of Plasmapheresis in multifocal motor elopathy associated with MCTD would seem necrotising vasculitis or the presence of a cir- neuropathy: a case report culating antiphospholipid antibody. The pa- intrinsically to have a better prognosis, as tient presented after an interval of several improvement or resolution occurred in 70% Plasmapheresis is a well established therapy of cases treated with prednisolone and 1 months from onset of symptoms. It is possible in immunogenic peripheral neuropathies. on October 2, 2021 by guest. Protected copyright. that the initial pathology was related to a vas- azathioprine. In this and one other patient However, patients with multifocal motor culitis that had resolved by the time of there was a delay to presentation of over 4 neuropathy do not respond very well to pred- presentation. Magnetic resonance findings in weeks and no neurological improvement was nisone or plasmapheresis.2 This case report transverse myelopathy depend on the timing noted. We therefore suggest that whereas high illustrates that plasmapheresis may even be of the examination and the stage of disease. In dose prednisolone and azathioprine generally disadvantageous in multifocal motor neu- this case the delay to presentation may seems suYcient to treat MCTD associated ropathy. explain the fact that MRI demonstrated atro- transverse myelopathy, if there is a delay to Muscle strength was assessed (MRC scale) phy of the cord only. presentation more aggressive immunosup- and a sum score was calculated from 10 sites The table shows features of this patient, pression may be appropriate. (bilateral shoulder abduction, extension of and of other reports of transverse myelopathy S J M WEATHERBY hands and fingers, hip flexion, dorsal flexion related to MCTD. Transverse myelopathy M B DAVIES of feet); full muscle strength meaning a sum was noted to occur relatively early in the evo- C P HAWKINS score of 50. lution of MCTD, although in no patient was Department of Neurology, Royal Infirmary, Nerve conduction studies were carried out it a presenting feature. By contrast, retrospec- Stoke-on-Trent, UK according to the method described tive studies of patients with SLE and N HAQ elsewhere.3 Compound muscle action poten- transverse myelopathy found that transverse Department of Neuroradiology tials (CMAPs) were recorded from abductor myelopathy was a presenting feature in P DAWES digiti minimi and tibialis anterior bilaterally 78 50%. All cases of MCTD related transverse Department of Rheumatology, Hayward Hospital, by surface electrodes. Ulnar nerves were myelopathy involved the thoracic region. One Stoke-on-Trent, UK stimulated supramaximally with surface elec- patient also had a cervical myelopathy. In Correspondence to: Dr S J M Weatherby, Research trodes at the wrist, distal elbow, proximal 7 SLE, a retrospective analysis found the OYce, Department of Neurology, Royal Infirmary, elbow, axilla, Erb’s point, C7 vertebra; cervical cord to be the most commonly Princes Road, Stoke-on-Trent. ST4 7LN, UK peroneal and sciatic nerve at head of fibula, aVected site (50%). The onset was reported [email protected] sciatic notch, and L1 vertebra by a Digitimer 534 J Neurol Neurosurg Psychiatry 2000;68:532–541 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from high voltage D180 stimulator (Digitimer Ltd, and in both proximal sciatic nerves between (figure). The patient received 120 g IVIg Welwyn Garden City, England). L1 and the sciatic notch. His CSF was immediately (17–19 November) and 60 g Plasmapheresis was carried out by a stand- normal again. Multifocal motor neuropathy IVIg on 24–25 November. Muscle strength ard polypropylene filter P2S (Fresenius, Ger- was diagnosed, and the patient was put on improved gradually. On 30 November he was many) with a maximum pore diameter of 0.5 intravenous immunoglobulin (IVIg) with able to stand. On 11 December—after µm. This filter separates immunoglobulins. 180 g SandoglobinR initially—followed by another three courses of 50 mg IVIg—he Three litres were exchanged against human 100 g IVIg every fortnight. Within two weeks could walk with a stick, but he was still unable albumin (5% on each of 3 consecutive days muscle strength improved. The patient was to use his right hand and to climb stairs. (12, 13, 14 November 1998)). able to use his right arm, strength in his left Treatment was continued with 50 g IVIg a The patient had given fully informed con- leg improved, and position sense became week and 50 mg cyclophosphamide a day. By sent for both nerve conduction studies and normal (muscle sum score 42 in 1996). the end of January 1999 the patient had treatment. Intermittent deterioration was treated with regained his former muscle strength. He was The male patient was born in 1931. In oral methylprednisolone (1994–6 (4 mg a able to walk without a walking aid, to climb 1979 distal atrophic weakness developed in day); 1996–8 (2 mg a day)) which had a sub- stairs, and he could use his right hand (sum his right shoulder and hand muscles. There jective eVect on muscle strength. Additional score 36). Increasing CMAPs after proximal were no sensory symptoms. His CSF and cyclophosphamide had to be withdrawn nerve stimulation (figure) paralleled clinical neuroradiology were normal; electromyogra- because it was not tolerated very well. In improvement. phy showed a chronic neurogenic lesion. Vas- 1998 muscle strength gradually deteriorated This follow up study illustrates that dete- culitis had been excluded by sural nerve under 100 g IVIg every 2 weeks. Muscle rioration can be seen under continuing treat- biopsy. The diagnosis of spinal muscular weakness became more pronounced in the ment with IVIg. Additionally, plasmapheresis atrophy was made. In 1993, when the patient proximal right arm (MRC 2–3) and left foot may stimulate antibody rebound, and cause was first seen here, he had complete paresis of dorsiflexion (MRC 1); sum score was 36 in substantial deterioration in multifocal motor his right arm, paretic left hip flexion (MRC August 1998. Therefore, it was decided to neuropathy. It is assumed that it is not only 4), and peroneal weakness in his left leg with give three courses of plasmapheresis. Before antibodies which are responsible for conduc- slightly impaired position sense in the toes. plasmapheresis muscle sum score was 36 and tion block and muscle weakness that are No weakness was found in the left arm and the patient was able to walk. Plasmapheresis washed out by plasmapheresis but also those right leg. Muscle stretch reflexes were absent was performed on 12-14 November 1998. which act at the lesion site by blocking the in the right arm and both legs. There were no On 14 November muscle strength started to pathogenic agent. In this patient, before plas- central signs. In 1993 GM1 antibodies were deteriorate. On 17 November the patient mapheresis, serum gammaglobulin was in- positive (serum titre 1/200) and remained became tetraplegic with a disability sum score creased at 2.32 g/dl (12 November); thereaf- positive until 1998 (December 1998, GM1- of 19. His left arm and right leg, which had ter it was 0.7g/dl (18 November), and antibodies 29 U/ml, limit of normal 25 not been weak before, were paretic with recovered to 1.79 g/dl (24 November). U/ml). In 1993 muscle sum score was 34 and shoulder abduction MRC 4, hip flexion It has been shown that serum of patients conduction block was seen in the proximal MRC 2. There was proximal conduction with multifocal motor neuropathy can block right ulnar nerve between Erb’s and axilla block in both ulnar and sciatic nerves stimulus conduction in distal motor nerves.4

27 August 1998 2 November 1998 17 November 1998 30 November 1998 11 December 1998 http://jnnp.bmj.com/ on October 2, 2021 by guest. Protected copyright.

10 ms 5 mV

IVIg 50g/week Plasmapheresis After 5 × 30g IVIg After 3 × 50g IVIg 36 12–14 November 34 32 30 28 26 24 22 MRC sum score 20 18 Recording of CMAPs from right and left hypothenar after ulnar nerve stimulation at wrist, distal and proximal elbow, axilla, Erb’s point, and C7 vertebra. Before plasmapheresis no muscle weakness had been seen in the left arm and no conduction block in the left ulnar nerve. After plasmapheresis a pronunced conduction block was seen in the right ulnar nerve, and proximal conduction block developed in the left ulnar nerve. After IVIg treatment muscle strength recovered and stimulus conduction improved (MRC sum score indicated in lower trace). J Neurol Neurosurg Psychiatry 2000;68:532–541 535 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from

This could be due to an eVect on sodium associated with severe headaches, but nausea Postural headaches that are worse in the channel function in the distal motor nerve. and vomiting were absent. supine position can be a diagnostic challenge. Furthermore, the rapid clinical improvement In the previous weeks he had received sev- They may be present in Chiari-type I malfor- after IVIg could be due to an interference of eral unsuccessful treatments including high mation, as a symptom of intracranial hyper- IVIg with the blocking eVect of antibodies on doses of intravenous acetylsalicylic acid tension with or without papilloedema and the sodium channels at the motor nerve (1000-3000 mg daily), other non-steroidal with papilloedema but without increased endings.5 It is unlikely that plasmapheresis anti-inflammatory drugs such as ibuprofen intracranial pressure. 1−3 caused acute demyelination in our patient. In and naproxen, metamizol, and tramadol, and However, to our knowledge this is the first plasmapheresis, antibodies, complement, and short term treatment with prednisolone over case report of a secondary headache syn- cytokines are removed; therefore, this case 1 week. Concomitant medication related to drome without papilledoema but with strong supports the hypothesis4 of a complement HIV infection consisted of antiviral triple dependence on intracranial pressure with independent eVect of antibodies on sodium therapy with lamivudin, stavudin, and nelfi- relief after lumbar puncture although ntracra- channel function in the distal motor nerve. navir for 2 months. Other medication in- nial pressure was within normal limits. Under Even with good clinical results the patho- cluded foscavir and ganciclovir for previous normal circumstances the trigeminal intrac- genic process of multifocal motor neuropathy cytomegalovirus retinitis, pentamidine for ranial sensory system is insensitive to small may remain active under the treatment with sustained pneumocystic pneumonia preven- changes in intracranial pressure and even IVIg for many years. Therefore, muscle tion, and pyrimethamine/sulfadoxine for pre- high pressures may not result in headache. weakness develops if immunoglobulins are vious cerebral toxoplasmosis for 12 months. The nociceptive sensory threshold for pres- washed out by plasmapheresis. Six months previously he also had been sure diVerences can be lowered by inflamma- D CLAUS started on ethambutol when atypical myco- tory processes as seen in meningitis, after his- S SPECHT bacteria were detected during bronchial tamine administration, and during acute 4 Department of Neurology, Hospital Darmstadt, lavage although without clinical manifesta- migraine attacks. In our patient none of these Teaching Hospital of the University of Frankfurt, tion. conditions were met. Germany On neurological examination there was It has been demonstrated in experimental M ZIESCHANG mild meningism but no papilloedema. Vision animals that trigeminal sensory fibres can Department of Nephrology was permanently impaired bilaterally after change their transduction and transmission Correspondence to: Professor D Claus, Depart- cytomegalovirus retinitis 3 years previously properties due to their chemosensitivity and ment of Neurology, Klinikum Darmstadt, Teaching and fundoscopy showed only old lesions and lead to sensitisation inducing increased me- 5 Hospital of the University of Frankfurt, Heidel- no florid inflammatory signs. Proximal re- chanical intracranial hypersensitivity. This berger Landstraâe 379, D-64297 Darmstadt, Ger- flexes were normal but were reduced periph- lowered threshold for the activation of nocic- many erally due to HIV and medication related eptive trigeminal fibres may have been the [email protected] sensorimotor neuropathy. case in our patient. From the clinical presen- On admission cranial CT and MRI (in- tation during the detailed lumbar puncture it 1 Toyka KV, Augspach R, Wiethölter H, et al. cluding contrast enhancement) showed no seemed evident that a drop in pressure was Plasma exchange in chronic inflammatory signs of local or generalised oedema. Ventri- beneficial, whereas an increase was barely polyneuropathy: evidence suggestive of a tolerable; pressures below 6 cm H O pro- pathogenic humoral factor. Muscle Nerve cle size and configuration was normal and 2 1982;5:479–84. identical with previous scans. Neuroimaging duced no headache, whereas pressures above 10 cm H O did. 2 Chaudhry V, Corse AM, Cornblath DR, et al. showed no evidence of intracerebral tumours 2 Multifocal motor neuropathy: response to nor opportunistic CNS infection. White On ethical grounds the patient was not human immune globulin. Ann Neurol 1993;33: re-exposed to ethambutol. Therefore, despite 237–42. blood cell count was 2100/ml; routine blood 3 Claus D. Conduction studies in proximal chemistry including antinuclear antibody the temporal link between the ethambutol nerves. Schweiz Arch Neurol Psychiatr 1991;142: screening was normal. treatment and the new onset headache no 531. further evidence for a causative relation could Diagnostic lumbar puncture showed nor- 4 Roberts M, Willison HJ, Vincent A, et al. Multi- be established. Because of the many other focal motor neuropathy human sera block dis- mal CSF opening pressure (16 cm H O), cell 2 drugs coadministered with ethambutol a tal motor nerve conduction in mice. Ann Neu- count, and protein. Extended CSF analysis rol 1995;38:111–18. noxious direct drug synergism or accumula- was normal including microbiology, antigen 5 Lagueny A, Le Masson G, Burbeaud P, et al. tion of metabolites cannot be excluded. It Single fibre electromyography in multifocal screening for HIV, cytomegalovirus, and also remains unclear to what extent an HIV motor neuropathy with persistent conduction polymerase chain reaction for mycobacteria. blocks. 1998; : or cytomegalovirus related change of the cra- J Neurol Neurosurg Psychiatry 65 However, within minutes after the lumbar http://jnnp.bmj.com/ 357–61. nial sensory nociceptive system may have puncture the patient reported substantial contributed to the described headache. relief from his headache and meningism dis- Ethambutol, apart from its known neurotox- Ethambutol related headache in HIV appeared. Two days after the lumbar punc- icity particularly involving the optic nerve, infection: sensitisation to normal CSF ture the headache worsened again and the can cause headaches as an adverse event.6 pressure? A case report procedure was repeated on day 5. Because of How ethambutol may cause headache is not the lack of response to any analgesic medi- known but induction of trigeminal sensitisa- It is well recognised that headache can be a cation the procedure had to be repeated every tion seems a plausible explanation. A postural key symptom in intracranial hypertension, 4 to 5 days for 3 weeks. The CSF opening headache after ethambutol has not been often accompanied by lesions of the optic and pressure was always in the range 10-18 cm described previously. on October 2, 2021 by guest. Protected copyright. other cranial nerves. Typical characteristics of H O. Altogether, six lumbar punctures were 2 In conclusion a clearly postural headache this headache are postural dependence, wors- performed and the headache resolved each clinically suggestive of intracranial hyper- ening during exercise, Valsalva’s manoeuvre, time. tension can occur with normal intracranial and relief of headache after lumbar puncture. To exclude a placebo eVect of the lumbar pressure. Here, we report on a patient with a puncture the patient gave consent to a headache that bears all of the above symp- blinded protocol under continuous monitor- HOLGER KAUBE toms including relief after lumbar puncture ing of pressure via a three way connector. Department of Clinical Neurology, Institute of but with normal CSF opening pressures. Lumbar puncture without withdrawal of CSF Neurology, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, A 48 year old white man with a long history had no beneficial eVect on the headache. Two UK of HIV infection (first diagnosis 1991, actual withdrawals of 10 cm3 CSF resulted in a sig- staging CDC/WHO C3, CD4 79/ml, CD8 nificant decrease of the headache when the PETRA MUMMEL 179/ml, CD4/CD8 0.4) was referred to the pressure changed from 12 (10) to 4 (4) cm SUSANNE KOEPPEN Department of Neurology, University of Essen, neurological department because of new H O. By contrast, a slow intrathecal injection 2 Hufelandstr. 55, 45122 Essen, Germany onset progressive intractable headache that of sterile saline increasing the pressure to 16 had started 3 months previously. The head- cm H O produced an immediate exacerba- Correspondence to: Dr Holger Kaube 2 [email protected] ache was described as generalised but with tion of the headache. The treatment option of highest intensity retro-orbitally and of a con- lumboperitoneal shunting was denied by the stricting and pulsating character. Further local neurosurgeons because of lack of objec- 1 Wang SJ, Silberstein SD, Patterson S, et al. Idio- characteristics were a pronounced aggrava- tive signs of increased intracranial pressure. pathic intracranial hypertension without tion when resting supine that forced the Eventually, ethambutol was stopped be- papilledema: a case- control study in a patient to sit upright at night and extreme cause of its general neurotoxicity and the headache center. Neurology 1998;51:245-9. 2 Green JP, Newman NJ, Stowe ZN, et al. exacerbations during coughing or sneezing. headaches stopped 5 days afterwards without “Normal pressure” pseudotumor cerebri. J The patient complained of photophobia further need for lumbar puncture. Neuroophthalmol 1996;16:241-6. 536 J Neurol Neurosurg Psychiatry 2000;68:532–541 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from

3 Johnston I, Hawke S, Halmagyi M, et al. The decision making. Dramatic clinical recovery example, patients with middle cerebral artery pseudotumor syndrome. Disorders of cerebro- can be achieved during TPA infusion if early branch occlusions, which are not detectable spinal fluid circulation causing intracranial hypertension without ventriculomegaly. complete recanalisation of the middle cer- by TCD, may also benefit. In addition, it is Arch 7 Neurol 1991;48:740-7. ebral artery occurs. Microembolic signals unclear from the thrombolysis trials whether 4 Schumacher GA, WolV HG. Experimental have also been described during monitoring patients with lacunar stroke may also benefit. studies on headache. Arch Neurol Psychiatry of thrombolysis or spontaneous recanalisa- Stroke subtyping was not suYciently rigorous 1941;45:199-214. 7–8 5 Strassman AM, Raymond SA, Burstein R. Sen- tion and may signify clot dissolution. On to answer this question. Despite these provi- sitization of meningeal sensory neurons and the the other hand, persisting arterial occlusion sos I would agree with Barber and colleagues origin of headaches. Nature 1996;384:560-4. may identify patients at high risk of worsening that this hypothesis needs testing. I would 6 Mandell GL, Petry WA Jr. Antimicrobial agents: or recurrent stroke who may require aggres- drugs used in the chemotherapy of TB and strongly encourage any future thrombolysis leprosy. In: Hardman JG, Gillman AG, Lim- sive measures of secondary stroke prevention. trials to include assessment of middle cer- bird LE, editors. Goodman and Gillman’s the Although TCD can oVer a wealth of inter- ebral artery patency by transcranial Doppler pharmacological basis of therapeutics. 9th ed: esting information, its use is still limited due McGraw-Hill, 1996;1161-2. at the time of patient recruitment. The advent to many factors, including operator depend- of new small easily portable TCD machines ency and the need for in depth knowledge of which are considerably cheaper than the pre- cerebrovascular pathophysiology. Standard- vious PC based models, will greatly facilitate isation and prospective validation of TCD such a study. methodology is necessary to broaden its CORRESPONDENCE application for this exciting new setting. If TCD can be shown to be both valid and reli- Giant cell arteritis of the cervical able for diagnosing occlusion among a group radicular vessels presenting with of clinicians we expect broader acceptance of diaphragmatic weakness Transcranial Doppler sonography in this modality. We view TCD as a neurological “stetho- acute stroke We were interested to read the paper by Bur- scope” of the brain (as Camilo Gomez ton reporting the association between described it) in the acute setting and utilise et al We read with great interest Markus’ overview giant cell arteritis and cervical radiculopathy. this technique to compliment our clinical of transcranial Doppler (TCD) ultra- The evidence for giant cell arteritis seems 1 findings in the emergency room. We encour- songraphy. Markus highlighted the new incontrovertible, but the possibility remains aspects of such clinical applications of this age others to consider TCD for this applica- tion. that this patient might have had motor technology in cerebral haemodynamics and neuron disease coexisting with giant cell detection of emboli. Although the application PHILIP A BARBER arteritis. Details of the clinical examination, of TCD in acute stroke was briefly discussed, ANDREW M DEMCHUK investigations, and pathology point to this we point out some of the recent and exciting Department of Clinical Neurosciences, University of possibility. Furthermore, the neuropathologi- Calgary, Seaman Family Magnetic Resonance findings in the acute stroke setting. cal report does not include immunocyto- There is increasing evidence that intra- Research Centre, Foothills Hospital, Calgary, T2N 2T9, Canada chemical studies that would be necessary to venous tissue plasminogen activator (TPA) is exclude the diagnosis of motor neuron an eVective therapy for ischaemic stroke.2 ANDREI V ALEXANDROV Stroke Programme, University of Houston, Texas, disease. However, the impact of this therapy has been The clinical picture is one of a lower motor limited by several factors. Clearly TPA is not United States Correspondence to: Dr P A Barber neuron syndrome aVecting all four limbs eYcacious for all eligible patients, with about without objective sensory involvement. Den- 50% of treated patients left dependent or ervation was confirmed in all four limbs, 1 Markus HS. Transcranial Doppler ultrasongra- dead. Explanations for this incomplete eVect whereas the vascular inflammatory changes include poor recanalisation rate, completed phy. J Neurol Neurosurg Psychiatry 1999;67:135–7. concentrated around the radicular arteries in infarction before reperfusion, no reflow 2 Razumovsky AY, Gillard JH, Bryan RN, et al. the lower cervical roots. Pathological exam- phenomenon, and haemorrhagic complica- TCD, MRA, and MRI in acute cerebral ination of the spinal cord demonstrated “dif- tions. New strategies must be adopted to both ischemia. Acta Neurol Scand 1999;99:65–76. 3 Alexandrov AV, Demchuk AM,Wein TH, . fuse pallor of myelin” with minor loss of improve our understanding of how and when et al Yield of transcranial Doppler in acute cerebral anterior horn cells “and chromatolysis”. In thrombolysis treatment works to improve our ischaemia. Stroke 1999;30:1604–9. the diVerentiation of the syndrome from approach to achieving benefit from these 4 Kaps M, Link A. Transcranial sonographic http://jnnp.bmj.com/ motor neuron disease, it must be confirmed therapies. Vascular neuroimaging in the acute monitoring during thrombolytic therapy. Am J Neuroradiol 1998;19:758–60. that the classic pathological features are setting of ischaemic stroke is an important 5 Ringelstein EB, Biniek R, Weiller C, et al. Type absent. In addition to myelin pallor in the step in this direction. The technique of TCD and extent of hemispheric brain infarctions and corticospinal tracts, staining for the micro- is inexpensive, portable, and non-invasive, clinical outcome in early and delayed middle cerebral artery recanalization. Neurology 1992; and is well suited to the acute stroke setting glial marker CD68 and astrocyte marker 42:289. GFAP should be performed. Furthermore, and readily accessible at many centres. 6 Toni D, Fiorelli M, Zanette EM, et al. Early Several recent studies have shown that TCD spontaneous improvement and deterioration of staining of the motor cortex, brain stem, and can define arterial obstruction in acute ischemic stroke patients. A serial study with spinal cord sections for ubiquitin and neuro- transcranial Doppler ultrasonography. Stroke filament should be performed to exclude a cerebral ischaemia with a high degree of sen- 1998;29:1144. on October 2, 2021 by guest. Protected copyright. sitivity and specificity compared with mag- 7 Demchuk AM, Felberg RA, Alexandrov AV. primary motor neuron degeneration. Early brain reperfusion with intravenous Lymphocyte cuYng and microglial infiltra- netic resonance (MRA) or conventional thrombolysis leads to dramatic clinical recov- 2–4 tion are common in motor neuron disease, angiography. ery. N Engl J Med 1999;340:894–895. It is able to confirm the presence of arterial 8 Alexandrov AV, Demchuk AM, Felberg RA, et even in those cases attributable to mutant 1 occlusion, determine site and extent of al. Intracranial clot dissolution is associated SOD1. Ubiquitin staining is a very sensitive with embolic signals on transcranial Doppler. and specific marker for motor neuron disease, obstruction, and uniquely monitor for signs Journal Neuroimaging 2000 (in press). of recanalisation.2–7 Previous studies have and should be searched for extensively before shown that early TCD studies can predict the diagnosis can be excluded with outcome. Arterial patency or early recanalisa- Markus replies: confidence.2 It is well known that motor neu- tion predicts neurological improvement with I agree with Barber and colleagues that tran- ron disease may present with symptoms due TPA whereas persistent occlusion is associ- scranial Doppler (TCD) may well have to respiratory muscle weakness,3 and other- ated with poorer outcome.5–6 important applications in selection of patients wise the clinical phenotype in this case is very Hence,TCD could become the ideal mo- with acute stroke for thrombolysis. It is an suggestive of the flail arm variant of the dality to assist the clinician in the emergency appealing hypothesis that it may allow the disease,4 and we would hope that a compre- room with patient selection for intra-arterial identification of patients in whom recanalis- hensive neuropathological search for this therapy. If the middle cerebral artery stem tion has already occurred, and in whom diagnosis was performed in a case such as this occlusion can be confirmed or ruled out in a thrombolysis is not indicated, therefore spar- before it was ascribed to a vasculitis and matter of minutes by bedside TCD, the need ing them treatment with thrombolysis and recorded as such in the literature. for angiography could be reduced to a possible complications. This could increase 3 P N LEIGH minimum in this setting. Obtaining continu- the risk-benefit equation when giving throm- C E SHAW ous information about the status of an arterial bolysis. It is possible, however, that patients in Department of Clinical Neurology, Institute of occlusion during intravenous thrombolysis whom the middle cerebral artery is patent Psychiatry and Guy’s, King’s and St Thomas’ School has the potential to be very helpful in further may also benefit from thrombolysis. For of Medicine, De Crespigny Park, London SE5 8AF UK J Neurol Neurosurg Psychiatry 2000;68:532–541 537 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from

Correspondence to: Professor P N Leigh tion of word strings occurs during these 2 Miller NR, Newman NJ. Topical diagnosis of [email protected] pauses between the saccades. At the end of a lesions of the optic radiation. In: Miller NR, Newman NJ, eds. Walsh and Hoyt’s clinical line, a large saccade in the opposite direction, neuro-ophthalmology. Baltimore: Williams and to the beginning of the next line occurs and Wilkins 1998:371–5. 1 Shaw CE, Enayat ZE, Powell J, et al. Familial 3 amyotrophic lateral sclerosis: molecular pathol- the pattern of small saccades is repeated. 3 Sharpe JA. Neural control of ocular motor ogy of a patient with a SOD 1 mutation. Horizontal saccadic eye movements for systems. In: Miller NR, Newman NJ, eds. Walsh and Hoyt’s clinical neuro-ophthalmology. Neurology 1997;49:1612–16. reading are generated to peripheral visual 2 Ray Chaudhuri K, Leigh PN. Genetics of motor Baltimore: Williams and Wilkins 1998:1107– 19. neuron disorders. Advances in Clinical Neuro- stimuli (letters following those already inter- 1994; :267–6. preted). If the stimulus is in the hemianopic 4 Feldon SE, Burde RM. The ocular motor sciences 4 system. In: Hart WM, ed. 3 Chen R, Grand’Maison F, Strong MJ, et al. Adler’s physiology of field, there is a primary inability to initiate the the eye. St Louis: Mosby, 1992:176. Motor neuron disease presenting as acute saccade (saccades can be generated without a respiratory failure: a clinical and pathological 5 Lessell S, Lessell IM, Glaser JS. Topical 4 diagnosis-retrochiasmal visual pathways and study. J Neurol Neurosurg Psychiatry 1996;60: target, but tend to be inaccurate ). Also, the 455–8. parietal eye fields are involved in horizontal higher cortical function. In: Duane’s clinical ophthalmology. Philadelphia: Harper and Row, 4 Hu MTM, Ellis CM, Al-Chalabi A, et al. Flail saccadic eye movements, and may be impli- arm syndrome: a distinctive variant of amyo- 7:17–19. trophic lateral sclerosis. J Neurol Neurosurg Psy- cated in the reported case given the anatomi- chiatry 1998;65:950–1. cal location of the haematoma. Parietal eye field neurons discharge while executing visu- The authors reply: ally guided saccades, and during learned sac- We thank Mohamed, Elsherbiny, and Gould- Winer et al reply: cadic tasks, which may be important during ing for their insightful comments regarding We are pleased that Leigh and Shaw found reading,4 and parietal lesions are known to our paper.1 We also think that the essence of our report of interest and that the evidence cause saccadic defects. Vertical saccades are this case is the distinction between a periph- that the patient had from giant cell arteritis is mediated via a separate system. eral and a central processing defect causing incontrovertible. In true alexia, reading error results from an the lexical phenomenon encountered by our We also agree that the clinical features were inability to interpret written material. The patient. Mohamed et al suggest saccadic dys- very suggestive of motor neuron disease, anatomical basis of alexia without agraphia is function as a mechanism for explaining the which was in fact the antemortem clinical well described. This usually occurs in associ- unidirectional dyslexia in our patient. How- diagnosis. The only disagreement is whether ation with a right hemianopia, colour anomia, ever, whereas such a mechanism can explain the patient had two diseases or just giant cell cerebral dyschromatopsia, and prosopagno- right to left dyslexia when reading sentences, arteritis. sia, and is seen in left occipital lesions with our patient had the same language abnor- Histological studies of the postmortem damage to the contralateral visual association malities when reading isolated words when material, however, showed evidence of arteri- fibres which cross in the splenium of the cor- such saccadic mechanisms are not as impor- tis in the exact distribution of the clinical pus callosum.5 Reading pathways are well tant. We agree with Mohamed et al that visual deficit—that is, the cervical roots associated defined from visual cortex to auditory cortex hallucinations in the hemianopic field repre- with diaphragmatic function which we found via the dominant left angular gyrus, and such sent a favourable sign as indeed exemplified convincing as the sole explanation for the lesions have essentially isolated the angular by the favourable outcome in our patient. clinical picture. gyrus from visual input. We think that the Furthermore, we concede that the fluttering We agree that it is not possible to exclude reported case describes “pseudoalexia”5 or motion probably contributed to our patient’s the rare possiblity of both motor neuron dis- “hemianopic dyslexia”,1 where a fixation lexical diYculties. As pointed out by Mo- ease and arteritis and will undertake the sug- splitting hemianopia causes diYculty reading hamed et al such motion results from gested staining of sections of the brain stem, by obscuring parts of words. alterations in interhemispheric (occipital) spinal cord and motor cortex with ubiquitin. Visual hallucinations are common in pa- connections.2 This favours a more central JOHN WINER tients with hemianopias despite being rarely processing abnormality. Moreover, the per- ED BURTON mentioned,5 and in this case represent a sistence of the unidirectional dyslexia while PETER BARBER release phenomenon, as ictal activity and the visual fields returned to normal, as well as University Department of Clinical Neurology, pharmacological causes were excluded. They the dissociation between reading Yiddish Queen Elizabeth Hospital, Edgbaston, Birmingham are a prognostically favourable sign.2 Palinop- (written in Hebrew letters from right to left), B15 2TH, UK sia is also seen in hemianopic fields. It would which was preserved, to reading Hebrew Correspondence to: Dr J Winer be interesting to know what palinoptic which was abnormal both support a higher imagery was seen in this case, and under what hierarchical disorder. http://jnnp.bmj.com/ circumstances. Was it perseveration of the Last but not least, the role of the right Unidirectional dyslexia in a polyglot hallucinations, or “blindsight” imagery of hemisphere in language functions as sug- objects in motion being detected in the blind gested by our case is supported by recent We read with interest the short report by Leker observations on aphasia recovery after 1 hemifield (the Riddoch phenomenon). and Biran, describing unidirectional dyslexia The “fluttering” described in the intact stroke.3 in a polyglot, and would like to oVer alternative visual field of the reported case may reflect hypotheses for the symptomatology. R R LEKER the “bilateral eVect” seen from unilateral I BIRAN The case describes alexia while reading occipital lesions, which aVect the primary Hebrew, but not when reading English. This Department of Neurology, Hadassah University visual and extrastriate cortex. Such damage is Hospital, Ein Kerem, P.O. Box 12000, Jerusalem is a recognised neuro-ophthalmic phenom- thought to alter interhemispheric connec- 91120, Israel on October 2, 2021 by guest. Protected copyright. enon, which we have encountered in our tions along their presplenial course and cause Correspondence to: Dr R R Leker practice in patients reading the Urdu lan- disturbance of visual attention.2 The eVects leker@ cc.huji.ac.il guage, another script written right to left. are usually subtle relative to the hemianopia The extent of reading diYculties in pa- but often functionally significant. tients with hemianopia is well described.2 1 Leker RR, Biran I. Unidirectional dyslexia in a We think that all the symptoms in the polyglot. J Neural Neurosurg Psychiatry Patients with a right hemianopia cannot see reported case can be accounted for within the 1999;66:517–19. letters following those already deciphered. realms of established knowledge, without 2 Miller NR, Newman NJ. Topical diagnosis of Patients with a left hemianopia can read lesions of the optic radiation. In: Miller NR, postulating the existence of novel language Newman NJ, eds. Walsh and Hoyt’s clinical without diYculty, until they have to refixate associated neuronal networks in the right neuro-ophthalmology. Baltimore: Williams and the beginning of the next line which is in the hemisphere. Wilkins 1998:371–5. hemianopic field, and their place in the text is 3 Ola AS. Recovery from aphasia: activating the MOIN D MOHAMED “right’’ hemisphere. Ann Neurol 1999;45:419– then lost. It would be interesting to know if 20. this was the case with this patient while read- SAMER M ELSHERBINY ing English. Turning the page through 90° Department of Ophthalmology, Bradford Royal allows reading in the intact hemifield, and is Infirmary, Duckworth Lane, Bradford BD9 6RJ, UK Dyslexia in Hebrew standard advice to all patients with hemiano- PETER J GOULDING pia.2 Also by turning a page through 180° the Department of Neurology, St James’s University This letter concerns the very interesting short Hebrew text would have been readable and Hospital, Beckett Street, Leeds, LS9 7TF,UK report about unidirectional dyslexia in a comprehensible, as this would allow horizon- Correspondence to: Dr Moin D Mohamed polyglot by Leker and Biran.1 We wonder if tal reading in a left to right direction. the authors would oVer an explanation for the The process of reading consists of repeti- fact that at a time the patient still had “minor 1 Leker RR, Biran I. Unidirectional dyslexia in a tive small saccadic eye movements alternating polyglot. J Neurol Neurosurg Psychiatry deficits when reading Hebrew” (page 518) he with periods of fixation. Semantic identifica- 1999;66:517–19. made no mistakes when reading Yiddish? 538 J Neurol Neurosurg Psychiatry 2000;68:532–541 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from

The languages are diVerent to be sure, but age of relevant areas to disease processes. The the first edition of this book was published in the script and the right to left reading are book reflects the hard work and dedication of 1993. This second edition has grown in nearly the same. Does this support the the authors in pursuing a good radiological- volume by 180 pages and now comprises 24 hypothesis that “the flaw lies within the cen- anatomical correlation, something which al- chapters organised into sections covering basic tral multimodal, word processing neuronal though being crucial for neuroradiology, is techniques and physics of Doppler ultrasound, network”? The ability to read Hebrew sometimes forgotten or taken for granted! cerebrovascular physiology, evaluation of cer- vertically would still be unexplained. The book is divided in 19 chapters, 18 of ebrovascular disease, monitoring for microem- Did the authors examine mirror reading in them covering diVerent anatomical regions of boli, intraoperative and intensive care monitor- English and Hebrew? If so, what were the the brain. The last chapter presents some ing, transcranial duplex imaging, paediatric results? anatomical variants, as well as pitfalls on applications, and the role of contrast media. To MONROE COLE MRI, which should not be confounded with some extent the first edition of this book ALAN J LERNER real lesions. In general, the book will be use- seemed to play second fiddle to the volume of Department of Neurology, University Hospitals of ful for the training neuroradiologist, and also Newell and Aaslid— Transcranial Doppler. Cleveland, 11100 Euclid Avenue, Cleveland, OHIO for all of us dealing with neuroradiology and Now however, with the introduction of several 44106, USA MRI and having to exercise our anatomical new chapter authors, this book is likely to knowledge on a daily basis! Although the occupy the prime place on the shelf of any cli- authors base the book’s structure on classic nician or technologist with an interest in tran- 1 RR Leker, I Biran. Unidirectional dyslexia in a polyglot. J Neurol Neurosurg Psychiatry anatomy, sometimes putting too much em- scranial ultrasound. 1999;66:517–19. phasis on anatomical classifications that may Any book written by 42 authors is bound to not be too useful for neuroradiologists nowa- have some overlap in content but the current days, it results quite enjoyable, easy to read, edition is well edited and the illustrations The authors reply: and a useful teaching tool to have. (many in colour) are of high quality. Each We thank Cole and Lerner for their com- Those chapters dealing with the vascular chapter is comprehensively referenced. Par- ments. We think that a higher hierarchial anatomy on MRA are specially useful, in my ticularly welcome to this edition is the chap- damage is supported by the discrepancy opinion, since MRA is playing an increasingly ter by David Evans, Professor of Medical between the relatively preserved reading abil- more important role in neuroradiology, Physics at Leicester University. The ability, or ity of our patient in Yiddish compared with replacing conventional angiography for many otherwise, of TCD devices to count embolic Hebrew. clinical indications. phenomena accurately and to distinguish This discrepancy favours the existence of BEATRIZ GOMEZ-ANSON particulate from gaseous emboli has been a separate neuronal networks activated when point of some contention amongst workers in reading in diVerent language paradigms. The this field. Thus it is with some relief that here preserved vertical reading is suggestive of Neurosurgical Treatment of Movement we have a physicist of some authority in the either a coexistent lower hierarchial damage Disorders. Edited by ISABELLE M GERMANO. field of Doppler ultrasound trying to ensure or of a diVerent pathway connecting primary (Pp 276). Published by AANS, Illinois, that clinicians understand the limitations of receptive neurons with associative cortices 1998. ISBN 1-879284-58-8. the technique before making potentially inac- when interpreting vertically presented infor- curate assumptions. mation. We agree with Cole and Lerner that This volume is one in the series Statements regarding the value of TCD testing mirror reading might have yielded Neurosurgical published by the American Association monitoring during carotid endarterectomy interesting information, but unfortunately we Topics of Neurological Surgeons. It provides an are now all the more concrete given the work did not examine that at the time. excellent introduction to what is a fast which has been published during the mid- R R LEKER moving and increasingly important area of 1990s regarding this application, but much I BIRAN clinical neuroscience. uncertainty remains over the exact clinical Hadassah University Medical Center, Jerusalem, Israel The book deals with neurosurgery for utility of TCD in other settings—for exam- excessive or insuYcient movement, mainly in ple, monitoring “vasospasm” or the patient the context of Parkinson’s disease but also with head injury. The relevant chapters covering hemifacial spasm, torticollis, and provide a balanced summary of the available dystonia. It is divided into five sections. The evidence. The section on colour coded first is an overview of classification, clinical trancranial imaging has enlarged significantly CORRECTION since the first edition and further technology features, neuropathology, and neuroimaging. http://jnnp.bmj.com/ The second describes in detail indications, such as power Doppler, three dimensional techniques, and results of ablative surgery reconstruction, and echo contrast agents are Khudados E, Cody FWJ, O’Boyle D. Prop- while the third covers restorative procedures comprehensively discussed. These technolo- rioceptive regulation of voluntary ankle by neurostimulation or by neural grafting. gies are rather at the stage at which TCD movements, demonstrated using muscle vi- The last two sections cover ongoing technical found itself a decade ago—that is, promising bration, is impaired by Parkinson’s disease. developments in surgery for Parkinson’s techniques awaiting a role. Quite possibly by J Neurol Neurosurg Psychiatry 1999;67:504- disease, and surgery for hemifacial spasm, the time the third edition of this book arrives 10. In this paper the address of Etedal spasmodic torticollis, and dystonia. Nearly in a few years time their clinical relevance will Khudados should read Department of 40 contributors have contributed a total of 19 have been delineated. on October 2, 2021 by guest. Protected copyright. Neurological Surgery, Manchester Royal chapters, all of which are clearly written and Overall this is a welcome summary of the Infirmary, Manchester M13 9WL, UK. well referenced. Two of the authors appear TCD literature. I would not rush to replace under the misapprehension that Parkinson my first edition of the book with this edition wrote in 1917 rather than 1817. However, as much of what is contained in the first is not this publication provides a comprehensive made redundant by the later edition. How- and very practical guide not only for neuro- ever, for those clinicians and scientists surgeons but also for neurologists and other seeking an overview of TCD and its current BOOK REVIEWS clinical neuroscientists. status this text will suYce very well. DAVID THOMAS PETER MARTIN

MRI of the Brain. Normal Anatomy and Transcranial Doppler Ultrasonography. Russell and Rubinstein’s Pathology of Normal Variants. Edited by PATEL and Edited by VIKEN L BABIKIAN and LAWRENCE R Tumors of the Nervous System. Sixth . Two volume set. Edited by DARELL FRIEDMAN. (Pp 480, £61.00) Published by W WECHSIER. (Pp 409, £100.00). Published By Edition B Saunders Co, London, 1997. ISBN Butterworth Heinemann, Oxford, 1999. D BIGNER, ROGER E MCLENDON, and JANET M 0-7216-6945-X. ISBN0 7506 9969 8. BRUNER. (vol 1 pp 615, vol 2 pp 757, £265.00 the set (HB)). Published by This book presents the normal anatomy of the In the 17 years since Aaslid’s first description Arnold, London 1998. ISBN brain as seen on MRI studies in a very didac- of transcranial Doppler ultrasonography 0-340-58113-1. tic, well presented, and novel manner. Images (TCD) the technique has developed from a are well chosen, and are of high quality. The research tool to a routine clinical investigation, The sixth edition of Russell and Rubinstein’s authors also attempt to relate some of the ana- at least in some circumstances. This transition Pathology of Tumours of The Nervous System tomical structures to their functions and dam- has been especially rapid in the 6 years since has appeared in 1998, 39 years after the first J Neurol Neurosurg Psychiatry 2000;68:532–541 539 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from edition. For the first time it is divided into two Neurosurgical Management of Aneurys- diVerences between them comes in the extent volumes, which together contain nearly 1400 mal Subarachnoid Haemorrhage. Edited to which they dwell on treatment. Thus, pages. It is now edited by three North Ameri- by IVER A LANGMOEN, TRYGGVE LUNDAR, RUNE although acute headaches are logically di- can pathologists who have assembled a team vided into chapters by aetiology, with diagno- AASLID, and HANS-J REULEN (Pp 177, DM140). of 30 authors, most of them also from North Published By Springer-Verlag, Wien, 1999. sis and symptomatic treatment covered in America but with a few from elsewhere. By some detail, treatment of the bacterial contrast with the highly individual stamp ISBN 3 211 83256 4. meningitis or subarachnoid haemorrhage imparted by Lucien Rubinstein on the previ- itself is mentioned either barely ("with antibi- The editors have gathered contributions on ous few editions, we now have a book in otics”) or not at all. Clearly this approach aneurysmal subarachnoid haemorrhage from which most of the 23 chapters are multiau- flows naturally from the book’s concept as a several authors, each chapter dealing with a thored. The result is a book that bears virtu- publication confined to headache, but in such particular component of the subject and its ally no resemblance to its preceding editions a context the chapter divisions seem rather up to date management. Chapters 1 to 3 despite the full quotation in the preface from forced, and it also becomes diYcult to see cover the pathology, aetiology, and patho- the memorial tribute to Lucien Rubinstein how the approach would satisfy any particu- physiology; the next two chapters are con- and the inclusion of some figures from previ- lar target audience. That said, the qualm cerned with the monitoring of neurointensive ous editions. becomes less relevant in the subacute section, care of the condition. They are succeeded by The first volume deals mainly with general where treatment of the conditions is at least sections on the technical aspects of surgical aspects of the biology of nervous system covered (albeit succinctly), and completely treatment and finally the assessment of func- tumours—their epidemiology, the factors irrelevant in the final section where the treat- tional outcome is discussed in the last that are known to influence their growth, ments are entirely symptomatic and dis- chapter. invasion, and metastasis and the numerous cussed in detail. The format of the chapters is consistent relevant experimental studies of neurocar- Throughout, there is an obvious attempt to and they are well written, the coverage of the cinogenesis. These chapters are a very useful keep the text digestible; the chapter subsec- aetiology of cerebral vasospasm being espe- source of information and are, like the rest of tions are clearly headed and keenly trimmed, cially comprehensive. The chapter entitled the book, very thoroughly referenced at least usually to just one or two paragraphs. The ,I up to 1996. A tremendous body of data has Hemodynamics of Cerebrovascular Spasm text is also liberally sprinkled with illustrative found to contain original and stimulating been assembled now on experimental aspects case histories to guide your understanding ideas. Much of the remainder of the book of nervous system tumours and these are well (that you will find either entertaining and consists of summaries of technical issues covered in the early chapters of the book. The helpful or simply distracting), and one or two many of which have been covered in previous first volume also contains chapters on cellular useful tables also summarise treatment re- publications. My main criticism is the omis- immunology and immunohistochemistry and gimes and eYcacies. In keeping with this sty- sion of any mention of the integration of the first chapter on pathological anatomy of listic trend to brevity, the chapters themselves endovascular methods, without which a tumours of central neuroepithelial origin. are kept very much “bite sized”, a strategy modern book on the topic of aneurysmal This chapter alone occupies more than 200 that results, for example, in no fewer than subarachnoid haemorrhage and its neurosur- pages and has well over 1000 references. Vol- four chapters devoted to various aspects of gical management must be considered in- ume 2 contains chapters covering all the migraine and its treatment. Overall though, complete. other tumour types encountered in the the eVect is a book that is both clear and suc- I am happy to recommend this book to central and peripheral nervous system and in cinct, and where there are frustrations they neurovascular specialists and surgeons in the pituitary gland in a similarly comprehen- are almost inevitably a result of the con- training, with the aforementioned warning sive fashion, as well as chapters on the eVects straints of size and scope placed on the book that it is inadvisable to approach intracranial of radiotherapy and chemotherapy, and on by its title and philosophy. Certainly the end aneurysms in isolation without having con- cerebrospinal fluid cytology, flow cytometry, result remains easily digestible, but if you are sidered the endovascular options. That said, and DNA ploidy. someone with a ravenous appetite for knowl- this will prove to be a useful contribution to Thus, the book represents a formidable edge about headache, this minitome remains the literature on the subject. eVort in assembling a vast amount of a mere tasty snack that, although it may take information. For whom is it intended and PETER KIRKPATRICK the edge oV your hunger, certainly will not how useful will it be to its users? The leave you satisfied for long. intended readership is not clearly spelt out by Headache. By EGILIUS LH SPIERINGS. (Pp 236, the editors but at its core must be the clinical PHILIP BUTTERY £15.99). Published by Butterworth Heine- http://jnnp.bmj.com/ neuropathologists charged with reaching mann, Oxford, 1998. ISBN 0750671289. pathological diagnoses in individual cases. Lecture Notes on Neurology. Seventh For them there is undoubtedly a wealth of Edition.ByL GINSBERG. (Pp 199, £14.95). relevant information available but I do This attractively attired palm sized handbook Published by Blackwell Science, Oxford, wonder if some of it may be hard to find is one of several titles in a new series entitled because of the additional detail, not as yet The Most Common Complaints. Others so far 1999. ISBN 0-632-04827-1. translatable into clinical diagnostic relevance. include Confusion and Neck Complaints, and Illustrations are of great importance to this one at least is aimed squarely, the Over the past few years, increasingly well diagnostic neuropathologists. As you might publishers state, at a general market that will designed basic neurology textbooks have expect there is a wealth of macroscopic and include general practioners and physicians come on to the market, their attractive layout on October 2, 2021 by guest. Protected copyright. microscopic images, many of them in colour. and psychiatrists, as well as neurologists. The and quality illustrations serving to liberate There are also a large number of radiological author is a headache specialist at Harvard today’s students from the challenge of reams and MR images, and electron micrographs. and has written a small and engaging treatise of unappetising text interrupted only by an Most of the illustrations are excellent but that consideres the practicalities of managing occasional line drawing. The latest Lecture some have not printed well and have a muddy patients with headaches of various aetiolo- Notes in Neurology is one of this cohort and, as appearance or an intrusive grid-like overlay, gies. Starting with a brief overview of with several of its contemporaries, it now has perhaps reflecting a video image source. The headache, the book then divides itself into several editions behind it; the previous incar- book is generally well put together. Typo- three main sections, splitting headaches into nation (now over 12 years old) had long since graphical errors are few. Both volumes sensi- the acute, the subacute, and the chronic. The fallen out of favour with its customers, show- bly have a full index. It is less easy to under- first two sections thus take on what might be ing clear signs of being rather “long in the stand why they both need the preface. The termed organic causes of headache, with tooth” in the face of fierce competition and a running heads are not always quite as helpful separate chapters on meningitis, subarach- rapidly evolving specialty. On these grounds as they might be—the same one is used for noid haemorrhage, and hypertensive en- alone an update was long overdue, and the chapters 2, 3, and 4. cephalopathy in the first section, and on current guise (the seventh edition) has duly Overall, this book is an essential reference tumour, pseudotumour, opthalmic zoster, been substantially reworked; its new author text for clinical neuropathologists and it will temporal arteritis, and subdural haematoma has, however, not only brought along up to be of considerable use to neurosurgeons and in the second. This leaves the third section to date knowledge, but has also imbued the text tumour biologists interested in nervous cover the episodic and chronic headaches of of this edition with a welcome clarity, coher- system tumours. But for this reader it is not conventional classification—tension type ence, and attention to detail. The overall lay- updated Russell and Rubinstein but some- headache, migraine, and cluster headache out meanwhile has also been modernised, thing else altogether! among others. Each chapter has subsections with intelligent use of tables, figures, and text on aetiology, presentation, and diagnosis and boxes alongside useful illustrations (mostly MARGARET ESIRI is individually and well referenced. The main radiological). 540 J Neurol Neurosurg Psychiatry 2000;68:532–541 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from

The book is organised into two basic modern examples. However, this book tries to Fifty Neurological Cases from the sections, the first of which takes on the present a balanced account of the various National Hospital. By ADRIAN WILLS and C neurological approach, and includes six options and to my mind gives a clear account DAVID MARSDEN. (Pp 184, £24.95). Published chapters which partition the neurological of the issues and the advantages and disad- by Martin Dunitz, London, 1999. ISBN examination into logical chunks. These are vantages of these various options. 1-85317-677-X. preceded by a short chapter on neurological The book is divided into 16 chapters, of history taking, which emphasises the ration- which half deal with the therapeutic options One of the most challenging parts of neurol- ale of the neurological approach to diagnosis, and the others concentrate on aetiology, ogy is being able to give a coherent account of with a chapter on neurological investigations pathology, diVerential diagnosis, and evalua- a case when presented with just the history rounding oV the section. Within each chapter tion of patients with parkinsonism. Each and examination. This becomes all the more the text is supported by clear illustrations that chapter is typically only 10 pages long and challenging when the exercise is witnessed by illuminate examination techniques and high- learned professors, less learned colleagues, light important anatomical points. The sec- uses an attractive combination of tables and ond and larger section deals systematically bullet points, which actually means that each and a cast of thousands—and thus was born with the so called neurological disorders. chapter can be read in full without losing the the Gowers round at the National Hospital, Among these, common conditions—for ex- plot, point, or interest. Sadly the photographs Queen Square, London. The game as a train- ample, multiple sclerosis, epilepsy, stroke— are a bit disappointing, for example figure 3.2 ing registrar at this centre of excellence was to warrant their own individual chapters, demonstrating 18F-dopa uptake in a grafted try to put the case together while not making whereas other conditions are intuitively patient with Parkinson’s disease loses a lot for a fool of oneself. All well and good until the grouped either by their anatomical basis or by being small and in back and white. This is “very familiar well known syndrome” turns their aetiology—for example, spinal condi- especially a shame given the cost of the book. up and you are the only one in the auditorium apparently not in the know —how those tions, nerve and muscle, and neurological The book, though, is up to date and there knowing head nods from the front row would infections. Three diverse chapters complete are good accounts on the newer dopamine irritate at such times! Nevertheless, as a way this section, on neurology and other medical agonists ropinirole and pramipexole, as well specialties, neurological emergencies, and of learning it is hard to beat, and many will as the relative merits of stimulators over cherish their battles at the Gowers round to neurorehabilitation. lesions. However the account on neural The text layout is clear throughout, the the end of their day—which for many was transplantation for Parkinson’s disease was thought to be the day of battle itself. Adrian diagrams straightforward and useful, and disappointing, especially given all the recent many text boxes ( in current publica- Wills has now taken some of these cases and de rigeur controversy with this approach as a result of tions) make light work of the inevitable lists presented them as short case histories, with the double blind placebo controlled trial of (causes, syndromes, treatments, etc). Each commentaries from leading authorities on the chapter covers its topic(s) logically, with vari- embryonic nigral grafts for Parkinson’s dis- diagnosis. Indeed, many of us who had the ations on a basic format of aetiology, ease in the United States. Indeed a more privilege to train with Dr Wills will recognise epidemiology, clinical features, diagnosis, and critical and extensive discussion on this many of the cases, I certainly came across one management; and each is also tidily rounded subject would have been useful in a book of or two that I personally had presented. How- oV with a summary of important key points this sort, as whereas this approach is ever, the approach adopted by this book is a —with text again neatly boxed. Minor gripes experimental, it is nevertheless a common useful exercise given the quality of cases that exist: one or two of the tables are a little area of misconception and very much in the such a round boasts, although it is very awkward—for example, the categorisation of public domain with patients not uncom- diVerent from the Gowers round; for a start the epilepsies—and one or two of the monly asking about this type of therapy. Fur- the patients have a diagnosis! For another the chapters are perhaps a little light on detail; thermore, it is the only approach that challenge was to formulate an approach to however, in a book aimed at an undergradu- attempts to cure the patient rather than the general problem being presented and ate audience, some such compromises are ameliorate their symptoms and ultimately the then to dissect the diVerential diagnosis. This book does not enter into di erential diag- probably unavoidable. The compromises are eYcacy of stimulators and lesions will have to V noses, which thus undermines its value in my particularly obvious in the chapter on neurol- be compared with transplants. mind. However, for those in training who like ogy and other medical specialties where, with There are occasional omissions in this to solve written case histories then this book so much ground to cover, the text loses its book—for example, the chapter on neuropa- rhythm somewhat and becomes inevitably is useful, and certainly the range of cases is thology fails to discuss á-synuclein. However, fragmented. Overall, however, the feeling is of interesting and varied and overall I must say I other chapters are excellent and such an http://jnnp.bmj.com/ a carefully thought out and well written book really enjoyed reading it. with, in most chapters, the balance between example is that on the genetic epidemiology lucid explanation and more comprehensive by Golbe. This chapter does not just rehash ROGER BARKER detail being well maintained. Overall too, the old reviews on this topic; it actually presents the information for the likely audience for a logic of the assessment/disorder split is satis- Peripheral Neuropathy in Childhood. book of this sort, so that after presenting the fying, as it facilitates access to the essence of Second Edition. Edited by RA OUVRIER, JG what can seem to be a bewildering and unap- rudimentary concepts of epidemiology it goes MCLEOD, and JD POLLARD. (Pp 335, £50.00). proachable volume of knowledge and exper- on to discuss such things as “The utility of Published By Cambridge University Press, tise. In the days of “core curriculae“ the book anecdote"; an approach which is refreshing seems to have succeeded in encompassing and engaging and one which I found Cambridge, 2000. ISBN 1 898 68317 4. on October 2, 2021 by guest. Protected copyright. enough core detail to be a truly useful exceptionally helpful and useful. foundation for neurological assessment, while Obviously there are areas which one may Our understanding of peripheral nerve dis- ease has come on in leaps and bounds. retaining the necessary clarity and approach- disagree with and the value of the schematic Although the anatomical description of nerve ability that is so important in fostering an figures of the basal ganglia explaining the interest in the specialty. fibres flourished in the 17th and 18th centu- pathophysiology and therapeutic options in ries, it was the 19th century that saw the PHILIP BUTTERY Parkinson’s disease are often presented as beginning of our understanding of nerve definitive, even though a large number of function and pathology (Dejerine, Freidre- connections are omitted. ich, Charcot, Marie, and Tooth to name but a Parkinson’s Disease: The Treatment Overall though the book is a useful and few of those responsible). Despite much Options. Edited by PETER LEWITT and manageable addition to the burgeoning progress through this century, it has been the WOLFGANG OERTEL. (Pp 272, £65.00). literature on Parkinson’s disease, although it last decade of the 20th century that has Published by Martin Dunitz, London. ISBN is rather expensive which will limit its appeal. witnessed a huge expansion in our knowledge 1-85317-379-7. I thoroughly enjoyed it and found it helpful of the molecular processes controlling pe- and easy to use and apart from the photo- ripheral neuropathies. This book is one of many that are emerging graphs, well presented. I think it is especially The authors of this book acknowledge the on the market which consider the issues of helpful to those just entering the clinical recent major advances and this second the best approaches to the evaluation and arena of Parkinson’s disease and its manage- edition has been written to consider these treatment of patients with Parkinson’s dis- ment, but even for those who are familiar with issues not contained within the 1990 first ease. This is a complex area that is constantly the literature there is a lot to be gained by edition. Part of the International review of changing—the move away from levodopa to reading this book. child neurology series, this text aspires to be a dopamine agonists and deep brain stimula- reference text for childhood peripheral neu- tion as opposed to pallidotomy being two ROGER BARKER ropathy and in doing so, acknowledges the J Neurol Neurosurg Psychiatry 2000;68:532–541 541 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.4.532 on 1 April 2000. Downloaded from larger text Peripheral Neuropathy by Dyck, structures. The chapters share a common Community Treatment of Drug Misuse: Thomas, and colleagues which has, in turn, format of indications, surgical techniques, More than Methadone. By NICHOLAS been their reference text. Childhood should and case presentations, illustrated with nu- SEIVEWRIGHT. (Pp 243, £22.95). Published by not deter the adult neurologist as most merous line diagrams, scans, and photo- Cambridge University Press, Cambridge, aspects of peripheral neuropathies are men- graphs. Surgical pearls and technical pitfalls 1999. ISBN 0 521 59091 4. tioned, highlighting and lingering longer on are highlighted, and each chapter has a bibli- those that aVect the paediatric group. It is ography for further reading. comprehensive without being overdetailed or After describing the surgical anatomy and exhaustive: this is not an overlarge, weighty appropriate imaging of the orbit, some chap- Neurology in Tropics. Edited by JS CHOPRA textbook. Conditions are clearly and con- ters are devoted to the standard surgical and IMS SAWHENY (Pp 692, US$85). cisely discussed with detailed referencing to approaches. These include anterior, lateral, Published by Churchill Livingstone, New facilitate more in depth study. The authors do and transcranial orbitotomy, supraorbital rim Delhi. ISBN 81-7042-121-7. state their opinions and draw on personal craniotomy and its variations such as orbit- cases to illustrate conditions but nowhere is ozygomatic osteotomy, and transfacial ap- there blurring of the edges between published proaches to the anterior cranial fossa and Progress in Neurology. Edited by BERGEN, data and personal experience. What is midline skull base. The second section deals CHOPRA, SILBERBERG, BARAC, AND LECHNER. (Pp particularly appealing about this book is an with specific orbital conditions such as introductory chapter providing a historical thyroid eye disease, orbital exenteration, cor- 323, US$18). Published by Churchill overview of the field. This emphasis on the rection of post-traumatic enophthalmos, or- Livingstone, New Delhi, 1998. ISBN context in which developments have been bital repositioning, optic nerve sheath and 81-7042-133-0. made is continued throughout, particularly in optic canal decompression, and distraction of the description of the hereditary motor and the bony orbit. The final chapters consider sensory neuropathies. bone and cartilage grafting, rigid fixation Late Onset Schizophrenia. Edited by Aided by careful indexing, information is techniques, and soft tissue procedures. ROBERT HOWARD, PETER RABINS, and DAVID easily accessible and this book provides a reli- This is a beautifully illustrated book which CASTLE. (Pp 276, £43.00). Published by able first step for anyone seeing patients with is very easy to read, and provides a predomi- Wrightson Biomedical Publishing Ltd. peripheral nerve disease. The comprehensive nately plastic surgical and ophthalmological referencing will guide the specialist further. approach to orbitocranial disease. Although Petersfield, 1999. ISBN 1-871816-39-4. GILLIAN HALL its subject matter is not suYciently broad to make it the text of choice for neurosurgeons, particularly when considering transfacial ap- Muscular Dystrophy in Children: A An Atlas of Orbitocranial Surgery. proaches to the skull base, it is none the less Guide for Families. By IRWIN M SIEGEL. (Pp Edited by BRYANT TOTH, ROBERT KEATING, an excellent companion to standard anterior 130, $19.95). Published by Demos Medical and WILLIAM STEWART. (Pp 368, £149.95). skull base texts and a very useful volume to Publishing, New York 1999. ISBN Published by Martin Dunitz Publishers, consult when managing these complex and 1-888799-33-1. London, 1999. ISBN 1 85317 376 2. challenging cases. ROBERT MACFARLANE This multiauthor atlas combines the experi- Epilepsy and Mental Retardation. Edited ence of plastic and ophthalmic surgeons with By M SILLANPAA L GRAM SI JOHANNESSEN maxillofacial and neurosurgeons to create a Drug Action in the Central Nervous , , and multidisciplinary approach to congenital, System. Edited by PAUL M CARVEY. (Pp 416, T TOMSON. (Pp 212, £39.00). Published by acquired, traumatic, and neoplastic processes £22.50). Published by Oxford University Wrightson Biomedical Publishing Ltd, involving the orbit and adjacent craniofacial Press, Oxford, 1998. ISBN 0-19-509334-8. Petersfield, 1999. ISBN 1 871816 41 6. http://jnnp.bmj.com/ on October 2, 2021 by guest. Protected copyright.