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Head drop Table 2 Causes of ...... camptocormia Head drop and camptocormia Disorders Neuromuscular Motor Amyotrophic lateral T Umapathi, V Chaudhry, D Cornblath, D Drachman, sclerosis17 18 J Griffin, R Kuncl Muscle IBM19 Nemaline myopathy20 ...... Facioscapulohumeral dystrophy The spectrum of bent spine disorders Idiopathic21 Postencephalitic22 23 Villiusk encephalomyelitis24 ead ptosis (drop) results from Sodium valproate CASE A 25 weakness of the neck extensor, or toxicity An 80 year old man developed head pto- Idiopathic increased tone of the flexor mus- H sis insidiously over a period of few cles. It is characterised by marked anterior curvature or angulation of the weeks. A week before this he had an cervical spine and is associated with upper respiratory tract infection and also various neuromuscular (table 1) and experienced transient sharp pain over occasional nuclear sacs, target and tar- extrapyramidal disorders.12–15 Campto- the left and then the right shoulder. He getoid fibres as well as myopathic fea- cormia or the bent spine syndrome was had no , , dysphagia, tures such as the presence of hyper- first described in hysterical soldiers in limb weakness, or fatiguability. Exam- trophic and split muscle fibres; a few 1915 by the French neurologist ination showed severe neck extensor necrotic, degenerating and regenerating Souques.16 Typically there is marked weakness, Medical Research Council fibres; increased internalised nuclei, and anterior curvature of the thoracolumbar (MRC) grade 2. Muscle strength was mild endomysial fibrosis. No type group- spine. In some patients the spine is normal in all other cranial, proximal, and ing was noted, but there was massive angulated forward, the arms propped distal limb muscles. Serum creatine type I predominance. No specific treat- against the thigh for support. More kinase (CK) was 362 IU/l (normal ment was offered. A few months later, he cases, all among soldiers, were reported 24–195) at presentation. Anti- started to improve. At follow up 2 years during the first and second world wars. acetylcholine receptor antibody was not later, he was able to keep his head up for These patients responded well to psycho- detected. Repetitive nerve stimulation of prolonged periods, especially in the therapy. Recently camptocormia arising right biceps, right nasalis and right sitting position. However, he was more symptomatic while standing. He re- as a result of weakness or abnormality in trapezius was normal. A therapeutic trial ported an overall 60% subjective im- the tone of the paraspinal muscles has of pyridostigmine failed. Cervical spine been described (table 2). In contrast with provement in neck strength and weak- MRI did not show significant compres- other skeletal disorders of the spine such ness had not progressed to other sion of the cord, roots, or any specific as kyphosis, the deformity in head ptosis muscles. changes in the paraspinal muscles. An and camptocormia is not fixed and is corrected by passive extension or lying in EMG disclosed positive sharp waves CASE B restricted to lower cervical paraspinal the supine position. It is not possible to A 74 year old man with diabetes mellitus http://jnnp.bmj.com/ straighten the neck or back voluntarily. muscles. To reduce confounding from and hyperlipidaemia (for which he had The evaluation of these disorders can neuropathic changes secondary to coex- been taking a statin for a few years) indeed be challenging and often no defi- istent age related spondylotic changes, complained of progressive anterior cur- nite diagnosis is made, as illustrated by the thoracic paraspinal muscle was cho- vature of the spine associated with four cases of head ptosis and campto- sen for biopsy. The pathological findings proximal limb weakness for few months. cormia seen by us at the Johns Hopkins were mixed. There were neuropathic On examination, he had moderately Hospital. changes such as angular atrophic fibres; severe anterior thoracolumbar curvature

that could be extended passively. Diffuse on October 1, 2021 by guest. Protected copyright. weakness was present; proximal limb strength was MRC grade 4 and distal, Table 1 Neuromuscular causes for head ptosis 4+. Electrodiagnostic evaluation only Site of pathology Disorders showed evidence of an axonal polyneu- ropathy, consistent with diabetes. Serum Motor neuron Amyotrophic lateral sclerosis12 CK was persistently raised (367 IU/l). 3 Postpolio syndrome Spine MRI only showed mild degenera- Peripheral nerve Chronic inflammatory polyneuropathy4 Neuromuscular junction Myasthenia gravis12 tive changes. Anti-acetylcholine receptor Muscle antibody was not detected. Biopsy of the (a) Inflammatory Polymyositis12 rectus femoris showed increased fibre (b) Congenital/hereditary Inclusion body myositis2 size variability, necrosis, mild fibrosis, 56 Nemaline occasional red rimmed vacuoles, and one Mitochondrial myopathy7 Carnithine deficiency8 fibre which stained positive for ubiqui- Unspecified congenital myopathy9 tin, suggestive of inclusion body myosi- (c) Endocrine/metabolic Facioscapulohumeral dystrophy1 tis. However, inflammation or Cushing syndrome7 Hypothyroid myopathy10 Myotonic dystrophy with severe hypothyroidism2 ...... Severe hypokalaemic myopathy11 Abbreviations: CK, ; MSA, multisystem atrophy; ALS, amyotrophic lateral sclerosis

www.jnnp.com 2 EDITORIAL J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.73.1.1 on 1 July 2002. Downloaded from deposition were not seen. Despite stop- isolated antecollis causing forced anter- patients but other sensory abnormalities ping statin, his weakness continues to oflexion of the neck occurs in parkinso- are not prominent. The weakness is pro- progress and CK has remained raised nian syndromes. Rivest et al have docu- found in the neck extensors, and involve- (239 IU/L). mented antecollis in four of their ment of contiguous proximal muscles is necropsy proved cases of multisystem at most modest. The CK was normal in CASE C atrophy (MSA).12 13 The abnormality de- all but two patients. Katz at al2 reported A 59 year old woman presented with a 4 velops, sometimes in a subacute manner oedema-like changes and atrophy of the year history of progressive, painless over a period of weeks, in the late or neck extensor muscles on MRI. An EMG anterior curvature of the spine. She had middle stages of the disease. The neck of the paraspinal muscles was remark- a family history of facioscapulohumeral can only be passively and forcibly ex- able for the increased reports of sponta- but not of bent tended to its normal position with diffi- neous activity. Fibrillations were present spine. Examination showed campto- culty. None of the patients had convinc- in nine and positive sharp waves in one cormia along with scapular winging and ing dystonic spasms of the anterior neck out of the 15 patients for whom EMG mild facial and proximal weakness. Her muscles, although deeper muscles may data are available. Interestingly, such CK was at the upper limit of normal, 186 be involved. Speech, swallowing, and abnormalities were pronounced in the IU/l. An EMG showed short duration, upgaze deficits are often associated. lower cervical segments; and in one case small amplitude motor units with early Trials of botulinum toxin into both ster- reported by Katz et al,2 extended to the recruitment in the proximal muscles. nocleidomastoid muscles are ineffective midthoracic region. The motor unit Abnormal spontaneous activity was seen and response to levodopa generally action potentials in most cases are in the thoracic paraspinal muscles. No disappointing.12 13 Earlier case reports described as having short duration and decremental response to repetitive nerve have also documented the association small amplitude with early recruitment. stimulation was present. Spine MRI was between MSA and head drop.27–29 However, this has to be interpreted with unremarkable. 4q35 gene deletion, con- Yoshiyama et al14 published a series of caution as paraspinal motor unit action sistent with facioscapulohumeral mus- seven patients with parkinsonism exhib- potentials are generally small and may cular dystrophy, was detected. iting head drop, four of whom carried a have multiple turns even in normal indi- diagnosis of probable MSA. Although at viduals. EMG of the limb muscles is CASE D rest there was no anterior neck spasm, mostly unremarkable. The histology of A 63 year old woman developed slowly attempts to extend the head voluntarily limb muscles is normal or reveals mild progressive, painless thoracolumbar or passively was accompanied by con- non-specific myopathic changes such as stoop of about 30 degrees over 8 to 9 traction of the sternocleidomastoid mus- increased fibre size variability. In two of years. The deformity was not fixed and cles on surface EMG in all of these our cases there is some evidence of den- was worse in the standing position, patients. There was no correlation be- ervation. The paraspinal muscle biopsies better sitting, and minimal while lying tween the severity of the head ptosis and show less subtle but again non-specific down. There was no family history of parkinsonism. Three patients had im- changes such as fibrosis, increased fibre similar weakness. She had been taking provement of neck symptoms with treat- size variability, “moth eaten” fibres, and lipid lowering drugs for 10 years and ment of parkinsonism, whereas one myofibrillar disarray. Degeneration, re- symptoms did not improve on their worsened. A recent report described generation, and necrosis were reported withdrawal. Weakness was demonstra- head ptosis in seven out of 459 patients by Jaster et al32 and also seen in one of our ble in the thoracic paraspinal and proxi- evaluated for parkinsonism.15 All seven patients. Interestingly, as mentoned mal limb muscles. Her CK was persist- had features of MSA. Interestingly these above, similar biopsy findings have been ently raised at 606 IU/l (after withdrawal patients were also documented to have reported in the neck extensor muscles of of atorvastatin for 6 months). Anti- weakness as well as electrophysiolgical patients with parkinsonism who also http://jnnp.bmj.com/ acetylcholine receptor antibody was not and pathological features of myopathy in have head drop,15 30 suggesting that these detected. Spinal MRI showed mild de- the neck extensor muscles. Okamiya et al changes may not be specific for a neuro- generative changes in the cervical spine reported a patient with vascular parkin- muscular disorder. and normal thoracic spine. No specific sonism and neck extension weakness The weakness remains localised and changes were seen in the paraspinal who had myopathic changes in the in most cases stabilises after a short muscles. Spontaneous activity consisting extensor muscles.30 One of our patients period of progression. In some patients of positive sharp waves, fibrillation po- (listed JHH case 2 in table 3), who has (including our case A) there is some tentials, and complex repetitive dis- some clinical evidence of MSA, showed documented recovery. Secondary skel- on October 1, 2021 by guest. Protected copyright. charges were only seen in the right lower mixed myopathic and neurogenic fea- etal deformity can result in disability cervical paraspinal muscles. Biopsy of tures in the paraspinal muscles. even after good recovery of neck the rectus femoris muscle disclosed rare strength,32 highlighting the importance necrotic and regenerating fibres, mild IDIOPATHIC HEAD DROP of maintaining good neck posture during increase in fibre size variability, internal In some cases, no obvious aetiology for the period of weakness. Immunosup- nuclei and minimal fibre type grouping. the head drop is apparent even after pressive agents have been tried for vary- No specific diagnosis on the type of extensive evaluation and on prolonged ing periods with disappointing results myopathy could be made. Although she follow up.2 31–33 Combining the data from overall. Rose et al7 reported a positive subjectively felt slightly subjectively various reports of idiopathic head ptosis effect with prolonged corticosteroids, but worse at a review 9 months later there in the literature and patients seen at our their patient had generalised weakness was little change to her condition. institution (table 3), the following profile and inflammatory infiltrates (albeit of idiopathic head ptosis emerges. The scanty) in one biopsy. There may be a HEAD DROP female to male ratio is 3:2. Mean and role for cervical spine fusion in allowing Table 1 shows the various neuromuscular median ages at presentation are 74.5 and patients with disabling and non- disorders reported to cause head ptosis. 73 respectively. The onset is often sub- recovering head drop to maintain a Myasthenia gravis, especially in elderly acute, over days and weeks rather than functionally useful head position. people26 and amyotrophic lateral sclerosis months. Two of our patients and one that are the commonest causes. The predilec- was reported by Lerman33 had a history Camptocormia tion of these diseases for focal or of significant weight loss. Discomfort at The causes of camptocormia are summa- segmental onset may explain this. Severe the back of the neck is reported in some rised in table 2. As in head drop, several

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Table 3 Reports on idiopathic head drop

Weakness Radiological Onset CK examination Cx Report Sex age At onset Pattern IU/l Paraspinal mus EMG Limb EMG Cx (cervical) paraspinal bx Limb mus bx (cervical spine) Outcome

Lange1 (3 – – – – – – – *Non-diagnostic *Non-diagnostic – Unchanged 3–5 y patients) Suarez31 M 71 2 months Mild shoulder weakness N Fibs, small amp, short – N Biceps: ↑ fibre size Degen Unchanged 5 months case 1 duration MUAP; early rec. at variability lower Cx 2 F 81 3 months Mild deltoid, neck flexor N Fibs, small amp, short – – Deltoid: type II N weakness duration polyphasic MUAP at atrophy Cx 3 F 68 3 months Mild weakness N Fibs, small MUAP; early rec at Early rec, Fibre size variability; NADH: –N lower Cx sternomastoid moth-eaten; GT: 2 fibres with mus vacuoles 4 F 63 several Mild deltoid weakness 192 Fibs, short duration MUAP; – – Biceps: type II atrophy N months early rec at Cx and Th Katz2 case F 85 6 weeks Mild neck flexion and N Fibs, short duration MUAP; N Fibre size variability, ↑ Deltoid: N Mild spondyosis, no Unchanged 6 months 1 deltoid weakness early rec at lower Cx to internal nuclei and fibrosis neural impingement mid-Th 2 M 64 1 week Nil N Fibs, short duration MUAP; N Fibre size variability, split Biceps: N ” Unchanged 20 months early rec. at lower Cx to Th 1 fibres; ↑ internal nuclei and 3 F 84 3 months Nil N Nfibrosis, myofibrillar disarray Biceps: N ” Unchanged 54 months in hypertrophic fibres 4 M 65 2 months Nil N N As above and ↓ central Deltoid: N ” Unchanged 48 months NADH staining. Khella34 F 84 – Nil N Short duration, polyphasic – – – – Stable after 5 y MUAP, early rec Jaster32 M 69 4 h Nil N Small amp, short duration N Degen, regen, and necrotic – N Improves after 6 months MUAP at lower Cx fibres JHH, case 1 M 80 3 weeks Nil 362 PSW in Cx 5 and Cx 8 N Neurogenic and some – N Improves after 12 months myopathic changes 2 M 79 1–2 days Mild weakness of neck N Small amp, short duration Small amp, short Neurogenic changes Biceps: neurogenic Multilevel degen Improves after 12 months; flexion and shoulder MUAP, early rec at Cx 7–Cx duration MUAP changes has some features of MSA abduction 8 3 F 81 “Days” Mild ptosis N 1+ fibs, PSW N – Deltoid: N Mild degen Remains the same 4 y later 4 F 70 “Days”; pain Mild proximal weakness N N Few small amp, Non-specific myopathic Quadriceps: mild N Remains the same 4 y at onset short duration changes neurogenic changes, later MUAP type II atrophy 5 F 73 “Weeks” Nil N Small amp, short duration N – Trapezius: type II – Remains the same 4 y MUAP; early rec atrophy later

N, normal; ↑, increased; ↓, decreased; CK, creatine kinase; mus, muscle; bx, biopsy; EMG, electromyogram; PSW, positive sharp waves; Fibs, fibrillation; amp, amplitude; MUAP, motor unit action potential; rec, recruitment; regen, regenerative changes; degen, degenerative changes; Cx, cervical segment; Th, thoracic segment; NADH, nicotinamide adenosine dehydrogenase; GT, gomori-trichome; *site not indicated; “–”, not done/information not available; JHH, Johns Hopkins Hospital. www.jnnp.com

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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.73.1.1 on 1 July 2002. Downloaded from from Downloaded 2002. July 1 on 10.1136/jnnp.73.1.1 as published first Psychiatry: Neurosurg Neurol J http://jnnp.bmj.com/ on October 1, 2021 by guest. Protected by copyright. by Protected guest. by 2021 1, October on 4 EDITORIAL J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.73.1.1 on 1 July 2002. Downloaded from neuromuscular diseases can present deltoid muscle biopsy) and fingerprint occurs among the Lakut people of with segmental involvement of the pos- morphology on ultrastructure, sugges- Siberia, termed Viliuisk encephalo- tural muscles, resulting in campto- tive of a congenital myopathy. ,24 is also characterised by a cormia. forward bent posture. Other features Extrapyramidal disorders include rigidity, bradykinesia, , Amyotrophic lateral sclerosis (ALS) A mild stooped posture is a hallmark of and dysarthria. Pathological findings The kyphoscoliotic posture along with parkinsonism, but severe anterior curva- 21 include multiple areas of inflammation, head drop is typical in patients with ture is not common. Djaldetti et al necrosis, perivascular leucocytic cuffing, moderately advanced ALS. Involvement described eight patients with presumed spongiform changes, and fibrosis as well of paraspinal muscles was described by idiopathic Parkinson’s disease, at Hoehn 24 ± as marked atrophy of the . Gower in his account of patients with and Yahr stage 2 to 4, mean age of 66 5, Camptocormia was reported as a side 17 ± ALS, “In most cases the wasting in- and symptom-duration of 13.1 5.1 years effect of sodium valproate toxicity in a 23 volves the muscles of the back, and it who had severe forward flexion of the year old epileptic patient.25 Interestingly sometimes begins in them”. Our group thoracolumbar spine. This was apparent the deformity resolved when the plasma had previously reported a patient with on standing, less obvious while sitting, valproate level dropped to about 300 ALS who developed severe campto- but disappeared in the supine position. µmol/l, only to return 4 months later There was no mention of the nature of cormia, requiring him to support himself when the plasma concentration rose to onset. The EMGs of the paraspinal mus- with hands on his thighs. Limb muscles about 330–530 µmol/l. This patient had 18 cles done in five patients were normal. were less involved in this patient. no other extrapyramidal features. Re- Three patients apparently had immedi- versible parkinsonism has been well Myopathy ate and reversible aggravation of the documented as a rare side effect of Most generalised , because of bent spine with levodopa therapy valproate42 43 and an extrapyramidal pelvic girdle weakness, cause lumbar whereas the others improved. The au- mechanism might have been responsible lordosis rather than kyphosis. The excep- thors postulated that camptocormia in for the effect of valproate on spinal pos- tion may be inclusion body myositis parkinson’s disease may represent a type ture. (IBM), This was reported as a cause of of . 19 erector spinae weakness by Hund et al. Postencephalitic parkinsonism has IDIOPATHIC CAMPTOCORMIA A 70 year old man developed back and also been reported to cause curvature of later leg weakness over a period of the spine. Onuaguluchi22 reported five In some cases of camptocormia, such as months. The CK was slightly raised. An cases in 1964, followed a year later by a head ptosis, the clinical abnormality EMG showed spontaneous activity and series consisting of 14 cases from remains confined to the back and no polyphasic motor units with reduced 23 37 apparent cause is found even on pro- Martin. The latter included seven 16 36 44–47 recruitment in the paraspinal muscles. A photo illustrated case histories. The longed follow up. Is idiopathic biopsy of a relatively non-atrophic part spinal curvature in these patients was in camptocormia a distinct entity? Table 4 of the paraspinal muscles was reported both anteroposterior as well as lateral summarises the various reports on this to show morphology typical of IBM. The planes and diminished in the supine condition. The data from these studies predisposition for focal muscle weakness position. In many patients it was noted were combined with our cases B and D to may explain the occurrence of campto- to extend “from sacrum to neck”. In one develop a profile of idiopathic campto- cormia in IBM. Alternatively it may woman the forward flexion of the neck cormia. The incidence of camptocormia, occur as a secondary phenomenon. The was only seen when she was blindfolded. like head drop, increases with advancing preferential weakness of the quadriceps There was no consistent relation of the age. All but eight patients developed it at over the iliopsoas often forces the patient spinal curvature to the side of greater an age older than 60. The earliest age of http://jnnp.bmj.com/ to lock the knee in extension and predis- muscular rigidity. Three patients under- onset is 49 years. The female to male poses to a bent over posture for better went stereotactic surgery. One patient ratio is about 3:1. The weakness is balance. This may stretch the back mus- received lesions to the ventrolateral tha- mainly confined to the extensor muscles cles excessively, putting them in a lamus and pallidum and another to the of the spine. Onset and progression are position of mechanical disadvantage and pallidum alone, ipsilateral to the concav- more chronic compared with head drop, in turn lead to further bending. Katz et al ity of the spine. A third patient received a often extending over a period of months has proposed an important role for lesion to the pallidum contralateral to to years. Mild back discomfort was mechanical factors in the evolution of the side of spinal curvature. In all the present in a few. Sensory symptoms were 2 on October 1, 2021 by guest. Protected copyright. head drop. A similar pathophysiology cases rigidity and on the opposite absent. The presence of a family history 36 46 may cause camptocormia in IBM. Nema- side was abolished; but the spinal curva- in some patients suggests that inves- line myopathy involves the paravertebral ture was corrected only in the cases tigations for genetic and congenital muscles and can cause kyphoscoliosis or where the stereotactic lesion was placed neuromuscular conditions such as facio- lordosis.35 This congenital myopathy can ipsilateral to the concavity of the spinal scapulohumeral muscular dystrophy and rarely present in adult life. A case of a 62 curvature. Animal work by Ferrier,38 nemaline myopathy may result in better year old woman with adult onset nema- Demas-Marsalet39 and Hassler40 had pre- characterisation of the idiopathic cases. line myopathy who presented with se- viously shown that unilateral caudate The CK is raised in cases B and D and in vere camptocormia has been reported.20 lesions could cause a similar deformity some of the cases described by Hilliquin Furthermore, nearly half the cases of of the spine in animals, which can be et al48 and Serratice et al. 36 The paraspinal adult onset nemaline myopathy in the corrected by destruction of the contra- muscles have been reported to show literature have been associated with lateral head of the caudate nucleus. atrophy and a heterogenous appearance head drop.56We think that facioscapulo- Severe pathological changes have been on radiological examination (CT or humeral muscular dystrophy has not described in the caudate nucleus in MRI). The EMG findings are not uni- been previously reported as a cause of postencephalitic parkinsonism.41 Martin form, with reports of both myogenic and camptocormia (case C). Interestingly in hypothesised that tonic activity in the neurogenic features. In case D, spontane- the series of Lange et al on head ptosis caudate nuclei and its efferent pathways ous activity consisting of positive sharp there was one patient with facioscapulo- to the pallidum might play an important waves, fibrillation potentials, and com- humeral muscular dystrophy.1 In the part in maintaining spinal posture.41 plex repetitive discharges is seen only in series of Serratrice et al36 one patient had The slowly progressive and chronic the right lower cervical paraspinal mus- type I predominance (in quadriceps/ forms of a rare that cles, probably an incidental finding.

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Ehrenstein reported complex repetitive discharges in the lumbar paraspinal and limb muscles in his single case report.47 The paraspinal muscle biopsy findings are generally myopathic with increased fibrosis, variation in fibre size, and atrophy. Limb muscle histology is vari- Extrapyramidal features in 5 No extrapyramidal features similar deformity in 2 Family history of similar deformity in 20 able and no definite pattern was discern- ible. Cases B and D had necrotic muscle fibres, and the former rimmed vacuoles, but no specific diagnosis could be made. Information on therapeutic options is sparse and difficult to interpret.16 47 48 – Radiological examination. lumbar spine Miscellaneous –– – Facet joint arthritis, spinal stenosis at lumbar 4 and 5 segments – N Family history of Lumbar disc arthrosis, spondylolisthesis in 5. Some authors have reported dramatic recovery with intravenous methylprednisolone.48 The early use of corsets and other forms of spinal support may be useful to prevent the formation of fixed secondary deformities. Radiological examination paraspinal mus Areas of heterogeneous appearance Atrophy and hypodensity hypodensity Atrophy and heterogeneity Atrophy hypodensity heterogeneous appearance Idiopathic head ptosis and camptocormia: possible pathophysiological factors Head ptosis and camptocormia are age related disorders – Quadriceps: type II atrophy – Atrophy; fibrosis; mod inflam infiltrate in 6 myopathic in 4 Areas of Deltoid:N in 5 Low density Idiopathic head ptosis and campto-

/information not available. cormia occur mainly in those beyond the age of 60 and are almost never seen at ages less than 50 years. It seems that the age at presentation of head ptosis and camptocormia is advanced even in pa- Neurogenic Non-specific changes changes including atrophy Lumbar paraspinal mus bx Other mus bx Atrophy, fibrosis; mod inflam infiltrate in 6, necrotic fibres in 3 necrosis and regenin1, “fingerprint” in 1 Fibrosis, mod inflam infiltrate in 1 tients with an underlying disorder. In a recent study of 10 elderly patients with myasthenia gravis, age range 80–88 years, head drop was the commonest presentation, occurring in six patients.26 By contrast, ptosis was the presenting – – – – Fibrosis in 2, Small, short MUAP with CRD Deltoid, gluteal: myopathic in 6, distal leg: neurogenic in 5 complaint in only three patients.26 Nema- line myopathy in childhood is associated with spinal abnormalities such as rigid- ity, scoliosis, and lumbar lordosis. How- 20 ever, in adult onset cases campocormia http://jnnp.bmj.com/ and a high incidence of head ptosis56 Small, short MUAP – – Neuropathic in 4, myopathic in 1 myopathic and neuropathic in 1, Nin2 with CRD at lumbar segment myopathic in 11, “uninterpretable” in 16 have been reported. Katz et al2 have proposed that in elderly people, age related loss of tissue elasticity and mild kyphosis50 predis- in 4 (value in 2 Myopathic in 5, – N – ↑ not indicated) ↑ CK IU/l Paraspinal EMG: Limb EMG N Neurogenic in 5, poses to chronic stretch injury of par- aspinal muscles and may contribute to

the development of head ptosis. Similar on October 1, 2021 by guest. Protected copyright. factors may be involved in the pathogen- esis of idiopathic camptocormia. As No other weakness –of other mus in 4 – – – Fibrosis –discussed Low density above, some of the reported histological changes in the paraspinal muscles of patients with idiopathic head 3 y Mild weakness

± ptosis were also seen in those with an 15 30 weeks–9 y 5.2 – Weakness Onset Pattern obvious extrapyramidal cause, sug- gesting that these changes may be non-specific changes, resulting from chronic mechanical strain rather than a 81 75 Age, mean/ range specific myopathic disorder. Further- ↓ , decreased; mod, moderate; CK, creatine kinase; mus, muscle; bx, biopsy; EMG, electromyogram; PSW, positive sharp waves; fibs, fibrillation; amp, amplitude; MUAP, motor unit action potential; rec, more, in a study comparing the cervical 1:13 57–755:22 Few 63.5/51– 4:12 61–86 – 0:1 752:0 63–64 6 months 0:1 – 725:3 16 60.6/49– months Sex M:F paraspinal muscle morphology in pa-

45 tients with cervical and , increased;

Case reports and series of idiopathic camptocormia normal necropsy controls, necrosis, core- ↑ 47 36 targetoid changes, type II atrophy, and 48 46 44 49 type I predominance were seen in both groups with similar prevalence and Report (first author) Laroche Pennison-Besnier Poullin Delisle Ehrenstein Serratrice N, normal; recruitment; CRD, complex repetitive discharges; degen, degenerative changes; regen, regenerative changes; inflam, inflammatory; “–”, not done Hilliquin 51

Table 4 severity. The pathological abnormalities were more common and severe with

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Table 5 Similarities and differences between head ptosis and camptocormia

Similarities Differences

Clinical features Advanced age Head ptosis often subacute in onset, while In some extrapyramidal disorders both can occur in the same patient23 camptocormia tends to be insidious Underlying disorders Similar neuromuscular and extrapyramidal causes Camptocormia is more often associated with myopathic rather than neurogenic disorders Paraspinal muscle pathology Mixed myopathic and neuropathic features Prognosis/Outcome Improvement and worsening with levodopa therapy has been reported in Camptocormia tends to be progressive with parkinsonian patients with head drop as well as camptocormia14 21 no reports of spontaneous improvement

increasing age of patients in disease and (Parsonage-Turner syndrome). immunosuppressive or other pharmaco- control biopsies.51 Hence, it may be diffi- Indeed the clinical diversity of this therapy. The case report of valproate cult to make a specific aetiological diag- condition has been well documented,54 55 induced reversible camptocormia intro- nosis by studying the paraspinal muscles including involvement of structures as duces the intriguing possibility of using alone. proximal as the roots. Kidron et al had a GABA agonist in cases with an reported C6 root involvement with par- apparent extrapyramidal aetiology. Pa- Probable neuropathic contribution to the aspinal denervation.56 Whether a similar tients whose head ptosis does not im- pathophysiology of idiopathic head process can selectively involve the cervi- prove should be considered for cervical drop and camptocormia cal plexus and the dorsal spinal nerves fusion, which allows the patient to 45 Pennison–Besnier et al described a 72 can only be confirmed by studying the maintain a functionally useful head year old woman with a 16 month history histology of paraspinal muscles and dor- position. of camptocormia, whose lumbar para- sal spinal nerves early in the course of vertebral muscle showed fibrosis, fatty the illness. infiltration of interfascicular septa, ACKNOWLEDGEMENTS We thank Dr R Kurtzke, Fairfax, Virginia for groups of angular atrophic fibres, nuclear HEAD PTOSIS AND bags, fibre type grouping on ATPase providing us with information on his patient CAMPTOCORMIA: PARTS OF A and valuable comments, and DrMPTLunn, stain, core, and target fibres. They think SPECTRUM? Guy’s, St Thomas’, and King’s School of Medi- that denervation of the paraspinal mus- cine, London, for contributing important cles from impingement of the dorsal In most instances head drop and camp- tocormia are not related. It is rare to find ideas. rami by osteoarthritic facet joints causes Competing interests: none declared camptocormia. They asserted that the a patient with both disorders except in extrapyramidal disorders such as MSA J Neurol Neurosurg Psychiatry 2002;73:1–7 paraspinal muscle biopsies of other 23 reports and two of their patients might and postencephalitic parkinsonism. not have shown typical neurogenic However, the similarities between them ...... changes because they were performed (table 5) suggest that both head ptosis Authors’ affiliations late in the course of the illness. However, and camptocormia may occur as a result T Umapathi, Department of , National Neuroscience Institute, Singapore as already mentioned, neurogenic of similar pathophysiological processes affecting paraspinal muscles of different V Chaudhry, D Cornblath, D Drachman, changes on muscle biopsy are common J Griffin, R Kuncl, Department of Neurology, in elderly people and may not be parts of the spine. In any individual Johns Hopkins University School of Medicine, http://jnnp.bmj.com/ clinically significant.51 52 This is especially patient local factors such as chronic den- Baltimore, USA true in the cervical and lumbar paraspi- ervation and loss of tissue elasticity may D Drachman, J Griffin, Department of determine the spinal segment involved. Neuroscience, Johns Hopkins University School nal muscles, where there is a high of Medicine, Baltimore, USA incidence of degenerative vertebral dis- In summary, head ptosis and campto- J Griffin, R Kuncl, Department of Pathology, ease in elderly people. Furthermore, to cormia include a diverse group of disor- Johns Hopkins University School of Medicine, cause significant weakness of spinal ders that share in common involvement Baltimore, USA of different parts of the spine resulting in extension large segments of the paraspi- The second to sixth authors contributed equally nal muscles need to be denervated as disabling anterior curvature of the spine. to this work. sectioning of C1-C6 dorsal branches in In addition to the known causes of flex- on October 1, 2021 by guest. Protected copyright. 53 ion deformity of the spine, such as Correspondence to: Dr T Umapathi, Department patients with does of Neurology, National Neuroscience Institute, not cause any functional impairment. In myasthenia gravis, amyotrophic lateral Singapore 308433; [email protected] addition, although spondylosis could sclerosis, and Parkinson’s disease, multi- ple cumulative age related factors such conceivably damage neural elements by REFERENCES vascular compromise, either from arte- as loss of muscle tone, tissue elasticity, 1 Lange DJ, Fetell MR, Lovelace RE, et al.The rial ischaemia at the watershed region of chronic stretch and denervation of the floppy head syndrome. Ann Neurol the spinal arteries of Adamkiewicz or paraspinal muscles from degenerative 1986;20:133. venous congestion, direct impingement spinal disease, may be involved in the 2 Katz JS, Wolfe GI, Burns DK, et al. Isolated neck extensor myopathy: a common cause of of spinal roots have not been reported in pathogenesis. In practice, neuromusc- dropped head syndrome [comments]. patients with idiopathic head ptosis and ular and extrapyramidal disorders, be- Neurology 1996;46:917–21. camptocormia (tables 3 and 4). There- cause of therapeutic and prognostic 3 Brumback RA, Borge AF, Leech RW. implications, should be ruled out in all Postpoliomyelitis amyotrophy with rod fore it is more likely that chronic dener- (nemaline) bodies in skeletal muscle. vation of the paraspinal muscles is a patients. Measures to prevent fixed Transactions of the American Neurological contributing factor rather than the basis deformities—for example, passive mobi- Association 1980;105:430–1. for the development of idiopathic head lisation of the spine and the use of 4 Hoffman D, Gutmann L. The dropped head syndrome with chronic inflammatory ptosis and camptocormia. supports such as corsets and stiff demyelinating polyneuropathy. Muscle Nerve The presence of pain, relatively acute collars—should be instituted early. At 1994;17:808–10. onset, and recovery of head drop over a least some patients with idiopathic head 5 Lomen-Hoerth C, Simmons ML, Dearmond SJ, et al. Adult-onset nemaline myopathy: period of weeks in some patients is remi- drop recover partial function spontane- another cause of dropped head. Muscle niscent of the clinical course of brachial ously. There is no clearly defined role for Nerve 1999;22:1146–50.

www.jnnp.com EDITORIAL 7 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.73.1.1 on 1 July 2002. Downloaded from

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not confined to DSA, and the scatter plots interpretation of all tests and some are CORRESPONDENCE from Patel et al (fig 2) would suggest—in specific to certain tests. In our study we were keeping with other studies—that observer endeavouring to quantify the effect on patient variability is greater for MRA and CTA than management if non-invasive tests were used Measuring carotid stenosis for DSA.1 It is surprising that this did not instead of intra-arterial angiography to assess Comparing a new test with a standard translate into more clinically important disa- carotid stenosis. Our study has several limita- involves measuring disagreement. In the case greements when MRA and CTA were com- tions, including a relatively small sample size, of measuring carotid artery stenosis, some of pared with DSA. This is probably accounted and the fact that we were not able to get all the disagreement between different tests is for by the fact that in this study, for MRA and scans read by all observers but rather had to because of inherent differences in how the CTA, consensus views were taken for any get pairs of observers to concentrate on read- stenosis is demonstrated (test characteris- disagreements greater than 10% between ing only CTA, or MRA, or DSA. A better design tics). This is what we are most interested in observers. would have been to keep the same workers when assessing a new technology. However, This highlights the important point that together in pairs but randomly assign the some of the disagreement simply reflects combining multiple observations made on the CTA, MRA, or DSA films to each pair. As it is, variability in how we physically make the same data will reduce observer variability, and it is possible that some of the apparent differ- measurement with the standard technique. ultimately improve agreement with other ence between imaging modalities is specific to Choosing the point of maximum stenosis, methods. Partly for this reason, but also the pair of observers, not to the modality. choosing the point in the common carotid because to some extent the strengths and However, imaging studies are difficult to fund artery for use as a denominator, measuring weaknesses of CTA, MRA, and duplex ultra- and expensive to do, and the result and design from an eyepiece, or measuring from calipers sound are complementary, we would suggest of our study was a compromise involving all that a combination of tests (we use the all introduce variation when measuring ca- these factors. combination of ultrasound and MRA) should rotid stenosis. The resulting observer variabil- We identified that the observer reliability of be used in preference to DSA. ity in reporting contributes to disagreement CT angiography or MR angiography was What is clear from this study is that most of between methods but to some extent is inde- worse than that for digital subtraction angio- the disagreement between the different meth- pendent of the method used to generate the graphy, as highlighted by Drs Young and ods of measuring carotid stenosis can be angiogram in the first place. Humphrey. Also in general there was more In the medical literature, disagreement attributed to observer variability in reporting rather than to the test characteristics of the scatter between the observers for the reading between methods is often attributed entirely of asymptomatic stenoses than for sympto- to test characteristics, with little appreciation individual methods themselves. The 10% of patients injured as a result of DSA in this matic stenoses (emphasising the importance of the role of observer variability in reporting. of considering patient characteristics, not just When one method is compared with another study, and those who continue to be put at risk from catheter angiography in these the imaging technique). In the determination and disagreements emerge, it is not readily of the effect that this disagreement might apparent how much of the disagreement is circumstances, would be quite entitled to ask why they are exposed to a procedure which have on patient management, we used nomo- caused by the method used and how much by grams derived from the European carotid sur- the process of measurement, unless observer appears to offer no great advantage over safer alternatives. We suggest that more studies are gery trial which were based on intra-arterial variability data are also presented. In the angiographic measurement of stenosis. We recent paper from Patel et al, interobserver not required, simply a more thorough under- standing of presently available information. therefore had to use the comparison of variability data are presented but their signifi- non-invasive test reading with DSA rather cance in relation to overall agreement does G Young than being able to use the individual observ- not appear to have been appreciated.1 Middlesbrough General Hospital, Ayresome Green ers readings of non-invasive tests. Thus as Drs Using the data from Patel et al (tables 2 and Lane, Middlesbrough TS5 5AZ, UK Young and Humphrey point out, the actual 4) for symptomatic carotid arteries, it is noted effect of using non-invasive tests maybe worse that when 34 carotid digital subtraction angi- P Humphrey than we have estimated. ograms (DSA) are measured by one radiolo- The Walton Centre, NHS Trust, Liverpool, UK Finally, Drs Young and Humphrey suggest gist, there was disagreement in seven cases when the same films were reported by a Correspondence to: Dr G Young; that more studies are not required but we are second radiologist. Therefore if only DSA was [email protected] not entirely sure that that is completely true. used, seven patients would have had “inap- References Non-invasive imaging tests are continually undergoing modifications, many of which propriate” surgery according to which radi- Patel SG 1 , Collie DA, Wardlaw JM, et al. may be improvements in accuracy or practi- ologist read the angiogram. This is not Outcome, observer reliability, and patient surprising, and such disagreement is a con- preferences if CTA, MRA, or Doppler cality, but this cannot be assumed to be the sistent finding in observer variability ultrasound were used, individually or together, case. Much of this tinkering with technology studies.23 Observer variability in reporting instead of digital subtraction angiography is driven by the manufacturer’s desire to DSA therefore accounted for approximately before carotid endarterectomy. J Neurol encourage purchase of new machines. Im- 73 20% of disagreement in this particular series Neurosurg Psychiatry 2002; :21–8. provements have also occurred in intra- 2 Rothwell PM, Gibson RJ, Slattery J, et al. of angiograms. This sets a limit on the arterial angiography with smaller and more Prognostic value and reproducibility of manoeuvrable catheters and greater aware- maximum agreement that any alternative measurements of carotid stenosis. A method can demonstrate when compared comparison of three methods on 1001 ness of the risks, which may have helped to with DSA. It is clearly not reasonable to expect angiograms. European Carotid Surgery reduce the risk of angiography. Our “snap better agreement from another method than Trialists’ Collaborative. shot” of CTA, MRA, and ultrasound is already can be obtained by re-reporting the DSAs 1994;25:2440–4. out of date because contrast MRA is now themselves. In Patel’s table 2, when the same 3 Young GR, Sandercock PA, Slattery J, et al. increasingly used. While we would hope that arteries are assessed by computed tomo- Observer variation in the interpretation of non-invasive tests (probably in combination intra-arterial angiograms and the risk of rather than alone) would eventually replace graphic angiography (CTA) there was disa- inappropriate decisions about carotid greement with DSA in seven cases, while with endarterectomy. J Neurol Neurosurg intra-arterial angiography in the majority of magnetic resonance angiography (MRA) and Psychiatry 1996;60:152–7. patients being considered for carotid inter- ultrasound there was disagreement in six and vention, we feel it likely that there will always seven cases, respectively. The three alterna- Author’s reply be a need for some intra-arterial angiography tives thus disagree with DSA to the same in specific cases, or depending on local extent as can be attributed to observer Doctors Young and Humphrey highlight that resources. In any case DSA did not appear less disagreement in reporting DSA. Put simply, differences between tests arise from several popular than MRA among the patients in our the same number of missed or unnecessary factors, some of which are inherent in the test study. There is certainly room for much more operations would have occurred (roughly 20% and some of which arise from aspects in depth examination of existing data but we in this series) whatever method was used, attributable to observer variation. Some of the shouldn’t close the door on the need for including DSA alone. Observer variability is aspects to do with observer variation apply to further studies.

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J M Wardlaw 3 Wronski MD, Maor MH, Davis BJ, et al. rapid advances in our understanding of Department of Clinical Neurosciences, Bramwell External radiation of brain metastases from subcortical in recent years, and in par- Dott Building, Western General Hospital, Crewe renal carcinoma: a retrospective study of 119 ticular the increasingly sophisticated neuro- Road South, Edinburgh EH4 2XU, UK; patients from the M D Anderson Cancer imaging techniques. Given the large number [email protected] Center. Int J Radiat Oncol Biol Phys 1997;37:753–9. of contributors, consistency of style and 4 Culine S, Bekradda M, Kramar A, et al. approach is limited, but this is more than Cerebral metastasis after Prognostic factors for survival in patients with made up for by the breadth of expertise and brain metastases from renal cell carcinoma. opinion. primary renal cell carcinoma Cancer 1998;83:2548–53. There are some particular strengths. These

1 include the editors’ short chapter providing a The article by Roser et al, in which it was summary classification of subcortical infarcts, shown that the treatment of intracranial BOOK REVIEWS which is best appreciated if read both before metastases originating from renal cell carci- and after tackling most of the other chapters. noma can on occasion be successful, was most The excellent chapter on pathology of lacunar interesting. , medical causes and infarction is a welcome addition to this We have followed the clinical course of a management edition, while the chapters discussing risk patient with a renal cell carcinoma with a low factors and prognosis provide very useful mitotic index since 1989. In this patient the Edited by Norman Delanty (Pp 352, commentaries and summary tables of all the course was distinctly more malignant but the US$125.00). Published by the Humana Press, relevant studies. The discussion around the disease has also been successfully treated to New Jersey, 2002. ISBN 0-89603-827-0 usefulness (or not) of clinical diagnosis of date. In the last 13 years, this patient has had lacunar syndromes, carefully updated with four metastases surgically removed and a fur- This book is unusual among books about sei- the information from recent clinicoradiologi- ther nine treated with stereotactically guided zures because it focuses on acute symptomatic cal studies, is both thoughtful and logical, percutaneous single dose convergent beam (“situation-related”) seizures, rather than with plenty of clinical and epidemiological irradiation therapy (stereotactic modified lin- “epilepsy” (although there is inevitably some common sense. ear accelerator, 6–15 MV photons, 18–20 Gy overlap between the two). It provides defini- In common with all recently published prescribed to the 80% isodose). Apart from tions and describes the epidemiology and medical textbooks, this one is already a little slight mnemonic deficits, the patient is in pathophysiology of acute symptomatic sei- out of date. This is most noticeable for the good health. zures in the initial section, which is followed chapter on therapy, where recent advances The following factors which affect the by chapters detailing the specific circum- 2–4 (for example, new evidence on blood pressure prognosis were all met by our patient: stances in which such seizures are likely to lowering from the PROGRESS trial and on • The interval between the diagnosis of renal occur, often (although not invariably) includ- cholesterol reduction with a statin from the cell carcinoma and the first detected brain ing points of management specific to the situ- Heart Protection Study) are likely to have metastasis exceeds 17 months (our patient, ation. Subjects covered include seizures occur- most impact on clinical practice. If the editors 18 months; the patient described by Roser ring in the context of multisystem disease, have the energy to produce a third edition, et al,1 36 months); infection, hypoxic-ischaemic cardiopulmon- there is (as always) some room for improve- ary conditions, endocrine disorders, cancer, ment. The series of chapters on infarcts in Age below 60 years at the time of initial • and other conditions. Situation-related sei- specific subcortical territories would be en- diagnosis; zures occurring as a result of drugs or hanced by some figures illustrating the vascu- • Primary tumour of the left kidney, initial misuse are also addressed, as are those occur- lar anatomy that is discussed in the text. In nephrectomy; ring in the intensive care situation, and the addition, the quality of the discussion of study difficult, but important, differentiation of sei- methodology varies considerably between • Diameter of primary metastasis < 2 cm; zures from syncope. The book ends with a very chapters, and some would benefit from a more • Not more than one brain metastasis at the practical chapter entitled “Anticonvulsants in systematic and accurate approach to statisti- time of initial treatment; acute medical illness”, in which the consid- cal and epidemiological concepts. erations affecting the choice of antiepileptic • Solely intracranial metastases; drug in the acute situation are reviewed. Cathie Sudlow • Karnofsky > 70%; Although situation-related seizures are • No systemic symptoms such as fever or usually discussed in books about epilepsy, Medical risks in epilepsy weight loss at the time of diagnosis; they do appear to constitute a distinct group in a number of respects including prognosis. Edited by Svein I Johannessen, Torbjörn • Blood sedimentation rate under 50 mm/h To a certain extent the topics discussed in the Tomson, Matti Sillanpää, and Birthe Pedersen at diagnosis of renal cell carcinoma. book form a rather disparate group linked (Pp 140, £29). Published by Wrightson Patients in whom prognostic factors predict only by their tendency to cause such seizures Biomedical Publishing Ltd, Hampshire, 2002. a good outcome should be treated with intent as a reflection of ISBN 1- 871816-46-7 to cure. disturbance. Nevertheless, they are all condi- tions likely to be encountered at various times This is a very useful, reasonably comprehen- S Heckl by general physicians, neurologists, and those sive yet succinct multiauthor small book on Department of Neuroradiology, University of working in the accident and emergency medical risks associated with epilepsy. Areas Heidelberg Medical School, Kopfklinik, Im department, and this book, which is both covered include methodological aspects; acci- Neuenheimer Feld 400, 69120 Heidelberg, readable and comprehensively referenced, dents and risks in everyday life; traffic Germany will be of interest to all these groups. accidents; driving regulations; mortality, in- K Braun, J Debus cluding SUDEP; psychiatric comorbidity and Yvonne Hart suicide; fatal adverse drug reactions reporting Department of Radiation Oncology, German data (which are rather difficult to interpret); Cancer Research Centre, Heidelberg Subcortical stroke, 2nd edition -warning systems and risk prevention; S Kunze as well as insurance related issues. It also Department of Neurosurgery, University of Edited by Geoffrey Donnan, Bo Norrving, highlights many areas where further research Heidelberg John Bamford, and Julien Bogousslavsky is required. The book generally provides an (Pp 450, £79.50). Published by Oxford overview of the more recent research and Correspondence to: Dr med Stefan Heckl; University Press, New York, 2002. ISBN publications in this area and includes some [email protected] 0-19-263-157-8 regulatory issues. Inevitably it has a Nordic emphasis; it includes very useful advice on References This book is a must read for clinicians and precautionary measures to minimise risk of 1 Roser F, Rosahl SK, Samii M. Single cerebral researchers with an interest in stroke. The injury for people with uncontrolled epilepsy, metastasis 3 and 19 years after primary renal four editors are all specialist stroke clinicians including safer sauna. Some chapters, by cell carcinoma: case report and review of the who have been thinking about and leading necessity, serve purely as a compilation of literature. J Neurol Neurosurg Psychiatry research in subcortical stroke for many years, available incomplete data. Others are written 2002;72:257–8. 2 Decker DA, Decker VL, Herskovic A, et al. and they have put together a well constructed by key researchers directly involved in the Brain metastases in patients with renal cell and comprehensive multiauthor work. This area addressed and provide a very balanced carcinoma, prognosis and treatment. J Clin second edition is longer and more extensive review of current knowledge. On psychiatric Oncol 1984;2:169–73. than the first, reflecting the considerable and comorbidity, while agreeing that “the positive

www.jnnp.com 142 PostScript effects of drug therapy on cognitive and affec- within their particular subspecialty. There is and easy to read manner. Moreover, it tive functioning because of the reduction in increased reliance on colour illustrations, line addresses basic clinical issues rarely seizure activity are usually far greater than diagrams and tables to illuminate the text, addressed in a single publication. Its major the negative effects”, more information would and these are of excellent quality throughout. drawback is the lack of reference backing for have been welcome in an otherwise very well As to be expected, all chapters are written many of its statements, some of which are balanced chapter. The book would well serve authoritatively with clarity and style, compre- questionable. I found, for example, some of those for whom it is intended, namely epilep- hensively illustrated, and lavishly referenced. the “facts” unfamiliar, and would have tologists, neurologists, paediatric neurolo- Judging by the content of the chapters on welcomed knowledge of their source. Bits of gists, psychiatrists, and other professionals ageing and dementia, disease, and the material are dated (for example, the role who deal with patients with epilepsy. The edi- movement disorders, it is my guess that if of IP3 receptors in olfactory function, the tors rightly stress the “official line” that the anything is not included in each chapter, it’s nature of olfactory receptor cell regeneration) majority of patients with epilepsy can achieve probably not worth including anyway. The and several sections of the book seem lengthy, good control, with low associated risks. accompanying CD rom is user friendly, and uncritical, and of little practical value. Thus, the images are downloadable—a boon to nearly seven pages are devoted to the topic of Lina Nashef those wishing to produce a ready made odour memory, a topic with inherent theoreti- lecture or presentation of distinction. The cal issues and problems that are not addressed Greenfield’s neuropathology, book is a must for practicing and trainee by the author. However, the book is not 7th edition pathologists, but is equally compelling for intended to be a research book and, despite workers in other clinical neuroscience such shortcomings, it accomplishes its goal of disciplines and basic researchers interested in Edited by David I Graham and Peter L Lantos. educating the practitioner and providing him the roots of the dysfunctional nervous system. (2 Vol set (HB), Vol 1 pp1190, Vol 2 pp1140, or her with a practical roadmap for clinical Possession of the 7th edition is guaranteed £395). Published by Arnold, London, 2002. assessment and treatment. Indeed, the clini- lasting quality and full value, but before lash- ISBN 0-340-74231-3. CD rom (£145) ISBN cal information provided is comprehensive ing out make sure both your arms and shelv- 0-340-76-221-7. and well illustrated. This inexpensive book is a ing are strong enough to accommodate its must for any physician who has the occasion What can one say. The latest (7th) edition of presence. to see patients with chemosensory distur- Greenfield’s Neuropathology has hit the book- David MA Mann bances or has even a casual interest in shops, and indeed what a resounding thud it chemosensation, and should serve to elevate makes! The present edition is bigger than ever, Smell and taste complaints the level of appreciation of these senses again running into two volumes, but now within the medical community at large. totalling a staggering 2330 pages and costing Edited by Christopher H Hawkes (Pp 176, Richard L Doty an equally staggering £395. It comes equipped £16.99). Published by Butterworth- with a handy CD version of the illustrations, a Heinemann, Woburn, 2002. ISBN 0-7506- mere snip at £145. 7287-0. The 7th edition has undergone considerable CORRECTIONS changes in content, since the last edition five Despite the fact that problems with tasting years ago, reflecting the ever expanding and smelling are common in the general increase in knowledge of diseases of the nerv- population, few physicians have the knowl- The following errors occurred in the short ous system and muscle that has come from edge and training to authoritatively deal with report by Merlini L, Carbone I, Capanni C, et al. the exponential growth in neuroscience re- them. Christopher Hawke’s Smell and Taste Familial isolated hyperCKaemia associated search over the past decade. Areas of cellular Complaints provides a straightforward guide to with a new mutation in the caveolin-3 and molecular neurobiology, and the contri- the understanding and management of (CAV-3) gene. J Neurol Neurosurg Psychiatry butions that genetics and neuroimaging have chemosensory disturbances, reflecting the 2002;73:65–7. On page 66, left column, line 9, made towards improving our understanding first clinically oriented book of its kind since proline should replace leucine, line 12, protein of the causes of disease and our clinical inves- Ellis Douek’s The Sense of Smell and its Abnor- should replace enzyme, and in table 1, line 8 tigative and diagnostic skills, are more malities (Edinburgh: Churchill Livingstone, Del-TFT (63–65) should replace ?TFT (63–65). strongly featured. Hence, while greater em- 1974). This 180 page pocket sized book phasis has been placed on the basic science of provides a cogent overview of the anatomy We regret that an editing error occurred in the disease, the classic descriptive morphology for and physiology of the olfactory and gustatory correspondence from Jaster JH, Dohan FC, which Greenfield’s is renown is well main- systems, practical approaches towards their and O’Brien TF. Demyelination in the brain as tained. There are new chapters on “Metabolic assessment, and suggestions for therapy and a paraneoplastic disorder: candidates include and neurodegenerative diseases of childhood” management. Importantly, it provides the some cases of seminoma and central nervous and “Peroxisomal and mitochondrial dis- practitioner with the names and addresses of system lymphoma. J Neurol Neurosurg Psychia- eases”. The chapter on “Pathology of schizo- specialised taste and smell clinics throughout try 2002;73;352. The description of a patient phrenia” has been shrewdly expanded to the world, aiding the referral process. was altered, in the first line of the fourth cover “The pathology of psychiatric disor- Although there is little new in this guide, and paragraph the text should read “ . . .patient ders”. Other chapters have been retained as much of the material seems to have been who had a non-neurological malignancy, such, but many have been rewritten with new derived from second hand sources, it presents seminoma, and subsequently developed a authors reflecting the pre-eminence of each the available information in a well organised paraneoplastic syndrome . . .”.

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