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cell, antithryroid, antigliadin, and anti- the basal ganglia and thalami. Immunohisto- endomysial antibodies, serum electrophore- chemistry for prion protein (PRP KG9 LETTERS TO sis, immunoglobulins, bone marrow aspirate, 1:150, monoclonal antibody, courtesy of the and trephine. Several EEGs showed diVuse CJD surveillance centre in Edinburgh) THE EDITOR slowing of background rhythms. showed prominent staining in the plaques Postmortem neuropathological and histo- and diVusely in the neuropil of cerebral and logical examination confirmed the diagnosis cerebellar cortices. The olfactory tract of vCJD. In addition, there was evidence of showed prominent and diVuse staining for bronchopneumonia. Sections of brain (brain prion protein (PrP) associated with vacuola- New variant Creutzfeldt-Jakob disease weight 1322 g) showed extensive tissue tion (fig 1). presenting with loss of and smell involvement with prominent neuronal loss, It is diYcult to determine the precise cause astrocytosis, spongiform change, and numer- of our patient’s olfactory and gustatory Abnormalities of smell and taste have been ous Kuru-type plaques, including florid dysfunction. There were significant his- described in some neurodegenerative dis- plaques. These changes were seen in the cor- topathological changes in the basal forebrain eases including Alzheimer’s dementia, idio- tices of frontal, parietal, temporal, and where both taste and smell are represented.2 pathic Parkinson’s disease, Huntington’s cho- occipital lobes, basal ganglia, thalami, periv- Deposits of prion were, however, also found rea, KorsakoV’s syndrome, Pick’s disease, the entricular grey matter, brain stem, olfactory in many other parts of the brain involved in parkinsonian dementia complex of Guam, areas of the cerebrum, and the cerebellar cor- the neural processing of these . and amyotrophic lateral sclerosis.1 Hyposmia tex. Spongiform change and plaques were Changes were particularly prominent in the and hypogeusia are a feature of normal aging most prominent in the cortical regions and olfactory tract. Our patient illustrates that our but they have not been recorded as a promi- cerebellum. Plaques were particularly dense understanding of the clinical range of vCJD nent early feature in previous reports of vari- in the molecular and granular layers of the remains incomplete. Loss of taste and smell ant Creutzfeldt-Jakob disease (vCJD).2–5 We cerebellar cortex. Neuronal loss, gliosis, and are not at all specific to vCJD. These describe a patient with vCJD whose first spongiform change were most conspicuous in symptoms can have many causes including symptoms included deficits of taste and smell. At the time of his initial neurological assessment, this 54 year old ceramic tiler had a 12 month history of loss of taste and smell, anxiety, low mood, and unusually short tem- per. He first became aware that something was wrong when he lost the ability to diVerentiate the taste of tea from that of beer. Loss of taste and personality change pro- gressed gradually. He began to crave vanilla ice cream although he had never liked sweet foods in the past. He became fearful of leav- ing his house. Six months into his illness he became increasingly sleepy. On average he copyright. would sleep for 12 hours a day although he could sleep for 20 hours at a time. He devel- oped slurred speech, unsteady gait, upper limb tremor, and impotence. Neither the patient nor his wife noticed any change in his memory. On examination the patient had scanning dysarthria. His abbreviated mini mental test score was 7/10 (unable to remember an address, give his age, or name the year). He had a staring look with limitation of upgaze. Limb tone was increased with brisk tendon reflexes, ankle clonus, and bilaterally extensor http://jnnp.bmj.com/ plantar responses. There was mild upper limb ataxia and severe ataxia of gait. Although spi- nothalamic sensation was intact, joint posi- tion was impaired in both lower limbs. Olfactory testing disclosed evidence of im- paired smell detection and recognition. The patient thought that he could smell some- thing when tested with lavender and tar essence but was unable to recognise or on September 26, 2021 by guest. Protected describe the smell. Over the next 4 months the patient deteriorated rapidly with progressive ataxia, confusion, and agitation. He developed myo- clonic jerks 2 weeks before dying of bron- chopneumonia 16 months after the onset of his first symptoms. Our investigations in vivo supported a diagnosis of vCJD (suggestion of high signal within the thalamus on T2 weighted cranial MRI, negative immunoassay for 14–3–3 pro- tein but raised S100b protein in the CSF (0.91 ng/ml, reference range <0.38 ng/ml), no known mutations in the prion protein gene, methionine homozygous at codon 129). DiVerential diagnoses were excluded by nor- mal or negative full blood count, erythrocyte Figure 1 Bottom: normal (control) olfactory tract of a 61 year old man showing no prion protein sedimentation rate, B12, folate, thyroid func- (PrP) deposition after immunostaining with PrP.Top:olfactory tract showing vacuolation and tion tests, treponemal serology, antinuclear prominent diVuse staining for PrP.PrP plaques and spongioform changes were also found in other factors, glucose, antineuronal, antiPurkinje parts of the , the cortex, basal ganglia, cerebellum, and brain stem.

www.jnnp.com J Neurol Neurosurg Psychiatry 2001;71:412–418 413 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.71.3.412 on 1 September 2001. Downloaded from other neurodegenerative disorders. However, subjects, in addition to normal healthy Although according to the classic literature the diagnosis of vCJD should be considered if control subjects. the patients with writer’s cramp often have hypogeusia and hyposmia are accompanied Twelve consecutive patients with writer’s obsessive personality features,2 the present by changes of personality and other, more cramp who visited the dystonia outpatient study is the first to confirm the clinical find- “typical” features of vCJD. clinic of the Department of at ing using objective psychometric measures. M REUBER Kyoto University from May 1998 to Decem- The disease control group consisted of A S N AL-DIN ber 1998, were evaluated with the Yale- patients with peripheral nerve lesions, who Department of Neurology, Pinderfields General Brown obsessive-compulsive scale (Y- had discomfort in writing comparable with Hospital, Aberford Road, Wakefield WF1 4DG, UK BOCS). All patients had been treated with the patients with writer’s cramp in this study, 3 A BABORIE muscle aVerent block for more than 3 who were treated with muscle aVerent block. 3 A CHAKRABARTY months. As a consequence, their writing dis- Thus the current result does not support the Department of Neuropathology, General Infirmary at abilities were moderately improved. All of idea that physical distress and social disability Leeds, Great George Street, Leeds LS1 3EX, UK them could write their names in four to six due to writing impairment is the main cause Correspondence to: Dr A S N Al-Din Chinese characters at the time of assessment. of the obsessive-compulsive features of pa- A disease control group consisted of seven tients with writer’s cramp, but supports the patients with carpal tunnel syndrome, three notion that writer’s cramp and obsessive- 1 Mesholam RI, Moberg PJ, Mahr RN, et al. compulsive symptoms would develop due to Olfaction in neurodegenerative disease: a meta- with cervical spondylotic radiculopathy, one analysis of olfactory functioning in Alzheimer’s with chronic inflammatory demyelinating common pathophysiological mechanisms. and Parkinson’s diseases. Arch Neurol polyneuropathy, and one with myasthenia However, it should be noted that our patients 1998;55:84–90. gravis. All these patients showed disabilities with writer’s cramp had been under the treat- 2 SchiVman SS. Taste and smell losses in normal ment for more than 3 months and the aging and disease. JAMA 1997;278:1357–62. in finger movements due to peripheral nerve 3 Will RG, Zeidler M, Stewart GE, et al. Diagno- lesions of an upper limb, and were recruited duration of disease was relatively short. In sis of new variant Creutzfeldt-Jakob disease. from the outpatient clinic of the Department most cases of writer’s cramp without treat- Ann Neurol 2000;47:575–82. ment, it is known that writing disability is 4 Allroggen H, Dennis G, Abbott RJ, et al.New of Neurology at Kyoto University. A healthy variant Creutzfeldt-Jakob disease: three case control group, age and sex matched to the extremely severe, causing a significant eVect reports from Leicestershire. J Neurol Neurosurg writer’s cramp group, consisted of oYce on their lifestyle. The present result does not Psychiatry 2000;68:375–8. clerks or secretaries of Kyoto University and deny the possibility that the condition could 5 Zeidler M, Stewart GE, Barraclough CR, et al. cause abnormal psychopathology in writer’s New variant Creutzfeldt-Jakob disease: neuro- Shiga University. None of the subjects had logical features and diagnostic tests. Lancet received antidepressive or neuroleptic medi- cramp. 1997;350:903–7. cation for at least 6 months before the study. Recent studies reported abnormal meta- None of the subjects had a history of any psy- bolic activity in basal ganglia together with frontal and anterior cingulate areas in pa- Obsessive-compulsive characteristics in chiatric diseases. For these three groups, obsessive- tients with obsessive-compulsive disorder. patients with writer’s cramp compulsive symptoms were evaluated by These data suggest basal ganglia dysfunction trained psychiatrists, based on the semistruc- as a neural basis of obsessive-compulsive Writer’s cramp is characterised by a muscular 4 tured interviews assessing Y-BOCS. The symptoms (for a review, see Saxena et al ). spasm in the hand of the writing arm, and is Therefore, we suspect that basal ganglia dys- often provoked during specific tasks such as scores of the writer’s cramp group and two control groups on Y-BOCS were compared function is the common neural basis of writ- writing. Because of its highly task specific er’s cramp and obsessive-compulsive symp- copyright. nature, some neurologists believed that writ- using a two tailed Kruskall-Wallis test for non-parametric data. p Values lower than toms. In other forms of focal dystonia such as er’s cramp was of psychogenic origin. How- spasmodic torticollis and blepharospasm, ever, recent electrophysiological and neu- 0.05 were considered to be significant. When the result was significant, the analysis was high obsessive-compulsive symptoms were roimaging studies unanimously confirmed reported.5 Thus, the basal ganglia hypothesis repeated with each pair of groups, and the p basal ganglia dysfunction in patients with of obsessive-compulsive symptoms may be values were multiplied by three to correct for writer’s cramp. As a consequence, writer’s applied to focal dystonia in general. As multiple comparisons (two tailed Mann- cramp is currently regarded as a form of focal obsessive-compulsive symptoms of the pa- Whitney tests). Informed consent was ob- dystonia with neurophysiological pathoge- tients in this study were not severe enough for 1 tained from all the participants after the pro- netic mechanisms. the diagnosis of obsessive-compulsive disor- cedure of the study had been fully explained. On the other hand, in the classic psychiat- der, and due to the small sample size, these The 12 patients with writer’s cramp, 12 ric literature, clinicians sporadically pointed findings should be interpreted with caution. patients in the disease control group, and 12 out obsessive-compulsive personalities in Larger studies are necessary in clarifying the normal controls did not diVer in age or sex patients with writer’s cramp. Bindman and pathophysiology of writer’s cramp and associ- ratio (table 1). Mean Y-BOCS scores were http://jnnp.bmj.com/ Tibbetts described 10 patients with writer’s ated obsessive-compulsive symptoms. cramp, nine of whom had obsessional person- 5.8 (SD 5.5, range 0–18) for the writer’s alities.2 These findings have never been cramp group, 1.0 (SD 1.4, range 0–4) for the Y KUBOTA confirmed using reliable psychometric meas- disease control group, and 1.0 (SD 1.0, T MURAI range=0–2) for the normal control group, T OKADA ures. However, because of the above men- A HAYASHI tioned evidence of basal ganglia pathophysi- respectively. There was a significant eVect of Department of Neuropsychiatry, Faculty of Medicine, ology in writer’s cramp, together with the diagnosis on the total scores of Y-BOCS Kyoto University, 54 Shogoin-Kawaharacho, Kyoto 2 growing evidence of basal ganglia involve- (Kruskal-Wallis test, ÷ =5.99, p=0.012), with 606, Japan ment in obsessive-compulsive disorder, it is of significant diVerences between the writer’s M TOICHI great interest to elucidate the relation be- cramp group and disease control group Division of Child and Adolescent Psychiatry, Case on September 26, 2021 by guest. Protected tween writer’s cramp and obsessive- (p=0.030, corrected for three comparisons) Western Reserve University, University Hospitals of compulsive symptoms. or normal control group (p=0.036) but not Cleveland, Cleveland, USA In the present study, we evaluated between the disease control group and the M SAKIHAMA obsessive-compulsive symptoms in patients normal control group (p>0.99). The compul- Minakuchi Hospital, Shiga, Japan with writer’s cramp. Obsessive-compulsive sive symptoms of the writer’s cramp group symptoms may simply be a psychological were mainly related to cleaning and ordering. T SAKAMOTO K ASANUMA reaction to the writing impairment. To rule The result of our study showed that S MATSUMOTO out this possibility, patients with writing patients with writer’s cramp had higher R KAJI impairment due to peripheral nerve lesions obsessive-compulsive symptoms than the dis- Department of Neurology, Faculty of Medicine, Kyoto were included as a second group of control ease control group or normal control group. University, Kyoto, Japan Correspondence to: Dr Y Kubota Table 1 Ages, disease duration, and sex ratio of writer’s cramp group and control groups 1 Kaji R, Shibasaki H, Kimura J. Writer’s cramp: Disease a disorder of motor subroutine? Ann Neurol Age range Mean age duration Mean duration Sex ratio 1995;38:837–8. (y) (SD) (y) range (y) (SD) (y) (male/female) 2 Bindman E, Tibbetts RW. Writer’s cramp: a rational approach to treatment? Br J Psychiatry Writer’s cramp 21–70 42.6 (16.5) 1–9 4 (2.6) 8/4 1977;131:143–8. Disease control 22–66 44.9 (16.2) 1–5 2 (1.6) 7/5 3 Kaji R, Rothwell JC, Katayama M, et al. Tonic Normal control 22–70 43.1 (16) — — 6/6 vibration reflex and muscle aVerent block in writer’s cramp. Ann Neurol 1995;38:155–62.

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4 Saxena S, Brody AL, Schwartz JM, et al. motion induced oscillopsia, ataxia, head- Neuroimaging and frontal-subcortical circuitry aches, cervical , or Valsalva induced diz- in obsessive-compulsive disorder. Br J Psychia- 1 try 1998;35(suppl):26–37. ziness. 5 Broocks A, Thiel A, Angerstein D, et al. Higher Various ocular motor abnormalities have prevalence of obsessive-compulsive symptoms been reported in patients with ACM I. in patients with blepharospasm than in patients Among these, downbeat nystagmus and peri- with hemifacial spasm. Am J Psychiatry 1998; 155:555–7. odic alternating nystagmus are the most common. Other often encountered ocular signs, such as gaze evoked nystagmus, Cervical spondylotic myelopathy and rebound nystagmus, and impaired smooth Kennedy syndrome mimicking pursuit, reflect cerebellar involvement.23 amyotrophic lateral sclerosis of vestibular origin, being periph- eral or central, is usually accompanied by A 62 year old male patient presented with a nystagmus and nausea, or vomiting, and is longstanding history of slowly progressive often influenced by head position.34 The limb weakness, speech, and swallowing diY- entity of central positioning vomiting with- culties. In 1983 a diagnosis of amyotrophic out, or little, vertigo and nystagmus (pos- lateral sclerosis had been made. At that time turally evoked vomiting (PEV)) was first his physical examination showed tongue reported by Drachman et al and later atrophy with fibrillations, proximal limb recognised by Brandt and Baloh and weakness, and brisk lower limb tendon Halmagyi.3–5 reflexes. Electromyography showed abnormal Posturally evoked vomiting is generally “spontaneus activity” with fibrillations and poorly known as a warning symptom of a positive sharp waves in muscles of all limbs. posterior fossa lesion and is often misinter- His further medical and family history was preted.5 Whereas it has been documented in unremarkable. patients with posterior fossa tumours, it has In January 2000 not been reported in patients with develop- Figure 1 Spondylotic ridging at the C3-C6 showed mild facial weakness, marked atrophy mental abnormalities. We report on a patient and fibrillations of the tongue, severe dysar- level causing spinal stenosis is shown in the sagittal T2 weighted MR image. Additionally with ACM I where PEV was the most promi- thria and dysphagia, atrophy, and weakness of increased signal intensitiy is seen within the nent presenting symptom. the shoulder girdle and arm muscles, and an spinal cord at the level of C5. This 57 year old woman was seen in our unsteady and broad based gait. Apart from vertigo clinic because of gait unsteadiness brisk knee jerks, deep tendon reflexes were and postural vomiting. Her history included repeat (44; normal range 16–33) thus con- absent and plantar responses were negative. an aortic valve replacement for aortic insuY- firming the diagnosis of Kennedy syndrome. Sensory testing was normal. General physical ciency, nephrolithiasis, and peptic disease. The present patient with coexisting cervi- examination showed slight gynaecomastia. She was treated with warfarin. cal spondylotic myelopathy and Kennedy Laboratory testing showed raised creatine For years, she had dizziness and severe

syndrome highlights the diagnostic value of copyright. kinase (305 U/l) and lactate dehydrogenase nausea while looking up. During the past an intensified investigation including cervical (195 U/l) concentrations. Needle EMG dem- months severe nausea and vomiting appeared MRI and androgen receptor gene analysis in onstrated positive sharp waves and fibrillation when she tilted her head to either side and patients with an unusual clinical presentation potentials and long duration polyphasic down. Lately, she had become unsteady. She motor unit potentials with increased ampli- of motor neuron disease. also complained about left high pitched tudes in muscles of all limbs. By contrast, D FISCHER tinnitus and intermittent pain in the left motor and sensory nerve conduction studies U WÜLLNER shoulder. gave normal results. T KLOCKGETHER On examination her eyes were properly As cervical myelopathy is an important dif- R SCHRÖDER aligned with a full range of movements. No ferential diagnosis in patients with suspected Neurologische Klinik und Poliklinik, Rheinische primary or gaze evoked nystagmus was seen, motor neuron disease, cervical MRI was per- Friedrich-Wilhelms-Universität, with and without Frenzel’s glasses. The formed. As shown in figure 1, MRI disclosed Sigmund-Freud-Strasse 25, 53105 Bonn, Germany saccadic eye performance was normal, but marked cervical spondylosis with appreciable K WILHELM Radiologische Klinik, Rheinische the smooth pursuit in both the horizontal and narrowing of the spinal canal between C3 and http://jnnp.bmj.com/ the vertical plane was saccadic. The C6. In addition, T2 weighted images showed Friedrich-Wilhelms-Universität, Sigmund-Freud-Str. 25, 53105 Bonn, Germany vestibulo-ocular reflex, examined by a doll’s intramedullar changes with foci of high signal eyes movement, head thrust test, and dy- intensity at the level of C5 indicating Correspondence to: Dr D Fischer [email protected] namic visual acuity test, was normal. Mild myelopathy. Although these changes may readily explain the weakness in his upper dysmetria on finger-nose testing and finger- limbs, the cause of bulbar symptoms and finger testing was found bilaterally. The deep 1 La Spada A, Wilson EM, Lubahn DB, et al. tendon reflexes were brisk in the upper and denervation in his lower limbs remained Androgen receptor gene mutations in X-linked unclear. spinal and bulbar muscular atrophy. Nature normal in the lower limbs. The plantar toe 1991;352:77–9. responses were flexor. Sensation was normal.

The presence of slight bilateral gynaeco- on September 26, 2021 by guest. Protected 2 Kennedy WR, Alter M, Sung JH. Progressive The gait was atactic and the Romberg test mastia prompted us to look for androgen proximal spinal and bulbar muscular atrophy receptor gene mutations, which cause X of late onset: a sex-linked recessive trait. negative. linked spinal bulbar muscular atrophy.1 This Neurology 1968;18:671–80. On testing the eyes in the Dix-Hallpike 3 Sobue G, Hashizume Y, Mukai E, . X-linked disorder, also known as Kennedy syndrome, et al position to either , as well as in the head recessive bulbospinal neuronopathy: a clinico- down position, the patient reported severe is caused by an unstable expansion of a CAG pathological study. Brain 1989;112:209–32. repeat in exon 1 of the androgen receptor 4 Brooks BP, Fischbeck KH. Spinal and bulbar nausea, and became pale and perspired. gene (Xq11–12). The androgen receptor is muscular atrophy: a trinucleotide-repeat ex- However, no nystagmus was seen either by pansion neurodegenerative disease. TINS direct observation, or with Frenzel’s glasses. highly expressed in motor neurons of the 1995;18:459–61. brain stem and spinal cord. The CAG repeat The symptoms persisted with repetition of expansion is thought to confer a toxic gain of the positioning. function to the androgen receptor protein Posturally evoked vomiting without An electronystagmogram (ENG) docu- resulting in irreversible damage of brain stem nystagmus in a patient with mented saccadic eye tracking in the horizon- and spinal cord motor neurons. In addition, Arnold- tal plane. The optokinetic nystagmus was the impaired ability to transactivate androgen asymmetric with little increment after in- sensitive genes of the mutated receptor may Arnold-Chiari malformation type I (ACM I) creased speed velocity of the target. When account for endocrine features such as is a developmental anomaly of the rhomben- supine and with her head turned to the left, gynaecomastia or testicular atrophy in spinal cephalon characterised by displacement of nystagmus of 7°/s, beating to the left, was bulbar muscular atrophy.2–4 the cerebellar tonsils into the foramen recorded. No nystagmus was recorded on Genetic analysis in our patient showed one magnum and elongation of the medulla. It Hallpike testing.The caloric test was within allele carrying an abnormally expanded CAG usually presents in adult life with head normal limits.

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A behavioural showed mild reticular formation, sparing relatively the headaches were sharp and throbbing with the bilateral sensorineural loss in the fre- vermis-ocular motor circuity.6 maximum pain behind the right eye. The quencies of 2000 Hz-8000 Hz with normal Severe positioning nausea and vomiting headache then radiated to the back of the speech discrimination, interpreted as pres- with inconsistent dizziness and without nys- head without crossing the midline. The pain byacusis. The brain stem auditory evoked tagmus, was present in this patient with ACM was felt in paroxysms each lasting several potentials were normal. I. Initially, nausea occurred only on head minutes. He did not experience any visual Brain T1 and T2 weighted MRI with extension, but later on all head movements symptoms, nausea, nasal congestion, lacrima- gadolinium enhancement disclosed low lying but anteflexion. An ENG showed only low tion, or ptosis. Initially the total duration of cerebellar tonsils with elongation of the speed positional—that is, sustained nystag- the headaches was about 10 hours but it medulla and pons, compatible with ACM I. A mus in one head direction. On Hallpike posi- became progressively longer, remaining until brain stem or cervical syrinx was not demos- tioning testing nystagmus was not recorded. Wednesday on some occasions. They were trable (fig 1). Other ocular motor findings (saccadic pur- not related to the consumption of food and he In view of the progressive symptomatology, suit and abnormal optokinetic nystagmus ) did not go out drinking on Sunday nights. posterior fossa decompression was consid- were compatible with a cerebellar lesion.34 At the age of 20, he had had four episodes ered, but postponed because of the cardiac In ACM, a normal vestibular input after of severe headache on the right on four con- situation of the patient. Clonazepam was changes in head position might be misproc- secutive days after breaking rest during a oVered to the patient for alleviation of the essed by transient brain stem compression, week of night shifts as a warehouseman. In posturally evoked symptoms, but was refused leading to dissociation of the vomiting centres between the Monday “lunchtime head- because of possible sedation. from other parts of the vestibular reflex. The aches”, he remained well. He had no personal Central paroxysmal positioning vertigo relatively mild signs of positional vomiting in or family history of . He was taking occurs transiently on changing the head posi- our patient might be due to the benign nature propranolol for mild . His exam- tion, as opposed to central positional nystag- of the underlying disease, as opposed to the ination was normal. There was mild cervical mus, which persists as long as the head posi- invasiveness of tumours or acute neurotoxic- spondylosis on plain radiology. Brain CT 34 56 tion is sustained. Central paroxysmal ity, leading to severe PEV. showed minor atrophy. positioning vertigo can be diVerentiated from This patient with ACM seems to be unique A diagnosis of chronic paroxysmal benign paroxysmal positioning vertigo by its in the literature, as PEV has not been hemicrania was made, and he was prescribed shorter latency, longer duration of the attack, reported in this clinical setting. indomethacin (50 mg three times on Mon- and a direction changing nystagmus which is Further neurophysiological studies are days). With this his headaches were delayed not attributable to stimulation of a single needed to elucidate the pathogenesis of PEV. until Tuesday morning and the duration of 34 canal. It usually indicates a lesion around L POLLAK the headaches was reduced to less than 24 the fourth ventricle, dorsal vermis, or diVuse C KLEIN hours. The headaches also became less 3 cerebellar pathology. Central positional nys- J M RABEY predictable. A more protracted course of tagmus is often bilateral, of low constant fre- Department of Neurology, Assaf Harofeh Medical indomethacin rendered him headache free. quency, and occurs in association with lower Centre, AYliated to the Sackler School of Review of this man’s headache history 3 brain stem or vestibulocerebellar lesions. Medicine,Tel-Aviv University, Israel suggests that the episode he had when aged In 1977 Drachman et al described the E REICHENTHAL 20 was probably an isolated “cluster” head- 5 entity of PEV. In their two patients (one with Department of Neurosurgery, Ben Gurion University, ache. The more recent, highly predictable, a metastasis and the other with a glioblastoma Beer Sheva, Israel right sided headaches on Monday afternoons of the cerebellar vermis) PEV was a promi- Correspondence to: Dr L Pollak, Neve-Nir 1, Nes- were unusual. The predictability of these was copyright. nent clinical sign, whereas vertigo and Ziona 74042, Israel such that he could time it precisely to 1300 nystagmus diminished with the progression [email protected] hours. Their characteristics were not classic of the pathological process. Positional vomit- for chronic paroxysmal hemicrania, in which ing became so severe that the patients had to 1 Stevens JM, Serva WAD, Kendall BE, et al. Chi- attacks last between 20 and 30 minutes and hold their head in a forced position to prevent ari malformation in adults: relation of morpho- are accompanied by ipsilateral nasal conges- vomiting. The symptoms cleared after treat- logical aspects to clinical features and operative tion and lacrimation.1 Attacks of chronic par- ment with vestibular suppressants. Drach- outcome. J Neurol Neurosurg Psychiatry oxysmal hemicrania can, however, have man et al explained the condition of PEV by 1993;56:1072–7. 2 Bronstein AM, Miller DH, Rudge P, et al.Down clockwork regularity and are abolished by dissociation of the vomiting centres from beating nystagmus: magnetic resonance imag- indomethacin.1 Headaches in this case are oculomotor and receptive pathways of the ing and neuro-otological findings. J Neurol Sci also diVerent from chronic cluster headache 1987; :173–84. complex vestibular syndrome by a brain stem 81 as the paroxysms occurred many times infiltrating lesion. 3 Brandt T. Vertigo. Its multisensory syndromes, 2nd ed. London: Springer-Verlag 1999:199–213, throughout the day (repetition rate 12–16/ Arbusow described a patient with et al 291–8. day) and the total duration of an “attack” http://jnnp.bmj.com/ amiodarone induced positional vomiting with 4 Baloh RW, Halmaghi GM. Disorders of the could be up to 72 hours. Recently another mild vertigo, downbeat nystagmus, and limb . New York: Oxford University Press, 1996:446–53, 488–93. variant of chronic paroxysmal hemicrania— ataxia, responsive to . Ac- 5 Drachman DA, Diamond ER, Hart CW. hemicrania continua—has been described2 cording to their concept, which is in keeping Posturally evoked vomiting: association with and these headaches are non-paroxysmal and with that of Drachman et al, amiodarone dis- posterior fossa lesions. Ann Otol Rhinol Laryn- continuous. The degree of cervical spondylo- inhibits selectively the connections between gol 1977;86:97–101. 6 Arbusow V, Strupp M, Brandt T. Amiodarone sis in this patient was mild, and furthermore the dorsal cerebellar vermis and the vomiting induced severe prolonged head positional the nature of the attacks was not that of cer- centres in the postremal area and the lateral vertigo and vomiting. Neurology 1998;51:917. vicogenic headache. SUNCT syndrome is a

type of paroxysmal unilateral headache con- on September 26, 2021 by guest. Protected Lunchtime headache sisting of short lasting, unilateral, neuralgi- form headaches with conjunctival injection 34 Chronic primary unilateral headaches fall and tearing. Patients have up to 30 into one of five categories: chronic cluster paroxysms/hour, occurring once or twice a 3 headache, chronic paroxysmal hemicrania, day. This diagnosis is also highly unlikely in hemicrania continua, cervicogenic headache, our patient, with his clinical presentation and and SUNCT syndrome. Overlap between therapeutic response. It was thought that this types is recognised. Although the diVerentia- man’s headaches were most probably due to tion of these is sometimes diYcult, there are chronic paroxysmal hemicrania evolving from important therapeutic implications—for ex- a possible early “cluster” attack, supporting ample, indomethacin has a dramatic eVect on the suggestion that a common pathophysiol- chronic paroxysmal hemicrania but is less ogy underlies these “trigeminal-autonomic 4 eVective in chronic cluster headache. Here, a cephalalgias”. The precise predictability of patient with paroxysmal unilateral headaches, the Monday afternoon headaches is fascinat- occurring precisely on the same day of the ing, and unexplained. week and at the same time, is described. B SENANAYAKE A 57 year old man presented with episodic, RDESILVA Figure 1 Brain MRI shows low lying right sided, moderate to severe headaches of Essex Centre for Neurology and Neurosurgery, cerebellar tonsils and elongation of the pons and 9 months’ duration. He developed these Oldchurch Hospital, Romford, Essex RM7 0BE, UK medulla, compatible with ACM 1. always on a Monday at 1300 hours. The Correspondence to: Dr R de Silva

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1 Sjaastad O, Dale I. A new (?) clinical headache NINCDS-ADRDA criteria. According to eVects of spatial resolution. Ann Nucl Med entity “chronic paroxysmal hemicrania”. Acta Butters et al, they distinguished, on the basis 1995;9:215–23. Neurol Scand 1976;54:140–59. 5 Corey-Bloom J, Sabbagh MN, Bondi MW, et al. 2 Sjaastad O, Spierings ELH. Another headache of neuropsychological tests, patients with a Hippocampal sclerosis contributes to dementia absolutely responsive to indomethacin. Cepha- diVuse pattern of cognitive deficits (dAD) in the elderly. Neurology 1997;48:154–60. lalgia 1984;4:65–70. from those with focal temporal lobe dysfunc- 6 Crystal HA, Dickson D, Davies P, et al. The 3 Pareja JA, Sjaastad O. SUNCT syndrome, a tion (FTLD).2 Patients with FTLD are relative frequency of “dementia of unknown clinical review. Headache 1997;37:195–202. etiology” increases with age and is nearly 50% 4 Goadsby PJ, Lipton RB. A review of paroxysmal reported by both studies as having a better in nonagenarians. Arch Neurol 2001;57:713– hemicranias, SUNCT syndrome and other cognitive prognosis. Whereas the dAD group 19. short lasting headaches with autonomic fea- had diVuse perfusion deficits, the patients tures including new cases. Brain 1997;120: 193–209. with FTLD showed a circumscribed reduc- tion of tracer uptake in the left or right, or Cappa, et al reply: both temporal lobes. We are grateful to Bien, Helmstaedter, and We comment on the problem of suggesting Elger as they allow us to clarify a point of our AD in these patients with dementia. In the study that was not explicitely discussed in the study of Butters et al, seven of the patients of paper recently published in this Journal.1 CORRESPONDENCE the FTLD group underwent necropsy. Six We obviously agree with them that, in the had the typical histopathological signs of AD. absence of histopathological confirmation, One patient, however, showed the features of the diagnosis of “probable” Alzheimer’s hippocampal sclerosis (HS) and not those of disease (AD) can be questioned on method- AD (Cappa et al wrongly state that all ological grounds, particularly in patients with Painful stimulation necropsied cases of Butters et al had AD). focal temporal lobe dysfunction (FTLD). Two major groups of patients with the However, we think it rather implausible Once again, the meaningless term “response histopathological signs of HS have been that some of our patients with FTLD were in to pain” has been allowed to appear in the described in the literature: (1) HS is the most reality aVected by other non-AD diseases, in Journal.1 common morphological substrate of medial particular by those suggested by Bien et al: In this particular instance, the patient was temporal lobe epilepsy (MTLE). The af- medial temporal lobe epilepsy (MTLE) or unconscious and the brain stem was sepa- fected patients are known to show character- dementia sustained by hippocampal sclerosis rated from higher centres. The stimulus may istic memory impairment, which correlates (HS). have been noxious, but there is no evidence well with the degree of pathological changes. As for MTLE, which Bien et al consider a whatsoever that it was painful. Furthermore, Progress of memory impairment in MTLE is relevant source of possible errors in the diag- as it is insisted, rightly, that other sensory very slow and other cognitive functions are nosis of patients with AD and FTLD,2 we stimuli be fully and accurately described, why mostly preserved.3 Most (but not all) of them emphasise that none of our patients—with is “pain” permitted without qualification? have an abnormal MRI signal and HMPAO diVuse cognitive impairment (dAD) or with This often means pricking with a pin. Unless SPECT usually shows temporal hypoper- FTLD—was aVected by epilepsy. In our the pin or needle is driven in and twisted, the fusion.4 (2) Patients with HS have been paper, the exclusion of epileptic patients was modality tested is in fact sharpness discrimi- described in necropsy series of patients with implicit in the statement that all our patients nation and not pain—the two are dissociated dementia.5 It is unknown whether these met the NINCDS-ADRDA criteria for prob- in some lesions. Mechanical noxious stimula- patients could have been identified by MRI, able AD (according to these criteria, patients tion, which is interpreted as painful in the whether they had abnormal brain perfusion, who have epilepsy could at the most attain a copyright. intact and fully conscious person, may be and what the perfusion pattern would have diagnosis of possible AD). brought about by skinfold pinch or various been. Apart from MTLE, we are certainly aware other manoeuvres; thermal and chemical Cappa et al did not exclude patients with that HS is sometimes responsible for a noxious stimulation also, of course, exist. But epilepsy, and it is not clear if their MRI pro- dementia syndrome mimicking AD, as re- unless the nature of the stimulus is specified, cedure included high resolution T2/FLAIR cently discussed in several neuropathological the expression noxious stimulation (let pain sections angulated along or perpendicular to studies.3–8 and painful stimulation be forever abolished) is the hippocampus, which considerably in- On the other hand, a careful review of these not only almost devoid of relevance, but mis- creases the sensitivity for detection of studies leads to the following conclusions: (1) leading. MTLE-HS. They also had no histopathologi- in a general demented population, pure HS It also seems unfortunate that the recent cal evidence of AD. Thus, the authors may (HS without Alzheimer-type or other well Journal of Neurol ogy, Nurosurgery, and have included an unknown number of characterised degenerative or vascular patho- Psychiatry/Association of British Neurologists patients who actually did not have AD, but, logical changes) seems to be a very rare cause supplement on stroke did not mention for example, had temporal lobe epilepsy or 3 of dementia. Ala et al found only 0.4% of http://jnnp.bmj.com/ central poststroke pain. This diYcult neuro- dementia with the morphological substrate of pure HS in a retrospective study of 1771 logical sequel occurs in some 8%–10% of HS. It is noteworthy that in a recent 78 23 unselected demented patients and Jellinger survivors of stroke (6000–7000 new cases/ histopathological study on the causes of obtained similar results (0.53%) in a con- 6 year in the United Kingdom). Its correct dementia (with further references) it was secutive necropsy series of 746 demented diagnosis, treatment, and possible prevention noted that fewer patients than clinically subjects older than 55 years! (2) HS signifi- should be a matter of great concern to suspected fall into the histopathological cantly contributes to dementia only in old or neurologists. category of AD but—among others—fall into very old (>80 years of age) demented the category of HS. D BOWSHER patients with documented cardiovascular dis-

Pain Research Institute, University Hospital Aintree, CGBIEN eases (for example, ischaemic heart disease, on September 26, 2021 by guest. Protected Lower Lane, Liverpool L9 7AL, UK C HELMSTAEDTER arrhythmias, congestive heart failure in about Correspondence to: [email protected] C E ELGER 88% of cases) or with depression as another 46 University of Bonn, Department of Epileptology, frequent (63%) clinical feature. (3) When Sigmund-Freud-Str. 25, 53105 Bonn, Germany HS is associated with other neuropathologi- 1 Wijdicks EFM, Atkinson JLD, Okazaki H. Isolated medulla oblongata function after Correspondence to: Dr C G Bien cal changes, the precise boundaries with severe . J Neurol Neuro- [email protected] other forms of dementia are often less clear surg Psychiatry 2001;70:127–9. and the diVerential diagnosis may be contro- 2 Andersen G, Vestergaard K, Ingeman-Nielsen versial even at a pathological level. In the M, et al. Incidence of central post-stroke pain. 1 Cappa A, Calcagni ML, Villa G, et al. Brain per- 4 Pain 1995;61:187–94. fusion abnormalities in Alzheimer’s disease: necropsy series of Corey-Bloom et al, for 3 Bowsher D. Stroke and central post-stroke pain comparison between patients with focal tempo- instance, more than 50% of patients with HS in an elderly population. Journal of Pain 2001 ral lobe dysfunction and patients with diVuse had enough amyloid plaques to meet NIA (in press). cognitive impairment. J Neurol Neurosurg Psy- 9 chiatry 2001;70:22–7. criteria for AD; and even using the more 2 Butters MA, Lopez OL, Becker JT. Focal conservative criteria of CERAD,10 four pa- Is it really Alzheimer’s disease? temporal lobe dysfunction in probable tients out of eight could have been diagnosed Alzheimer’s disease predicts a slow rate of cog- as AD. (4) It is quite uncommon for nitive decline. Neurology 1996;46:687–92. We read the recent article of Cappa et al with 3 Helmstaedter C, Elger CE. The phantom of demented patients with postmortem evi- great interest.1 Using HMPAO SPECT, the progressive dementia in epilepsy. Lancet 1999; dence of HS to have previously received a authors studied 24 patients diagnosed as 354:2133–4. clinical diagnosis of probable AD (in most 4 Menzel C, Hufnagel A, Grunwald F, et al. The having (probable) Alzheimer’s disease (AD) relevance of interictal rCBF brain SPECT in cases, patients with HS had been diagnosed on the basis of DSM-III-R and the temporal lobe epilepsy: diagnostical value and as possible AD4).

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Patients reported in our study as aVected 7 Jellinger K. Hippocampal sclerosis: a common Schizophrenia in children and pathological feature of dementia in very old by AD with FTLD were clearly diVerent adolescents. Edited by HELMUT REMSCHMIDT humans. Acta Neuropathol 1994;88:599. from those described in studies claiming a (Pp 924, £39.95). Published by Cambridge 4–6 8 Jellinger K. Pure hippocampal sclerosis: a rare significant contribution of HS to dementia. cause of dementia mimicking Alzheimer’s University Press, Cambridge, 2000. ISBN 0 Their mean age was 67.3 (SD 8.1). All of disease. Neurology 2000;55:739. them had received a diagnosis of probable 9 Hyman BT, Trojanowski JQ. Consensus recom- 521 79428 5. AD according to the NINCDS-ADRDA cri- mendations for the postmortem diagnosis of There are few psychiatric conditions as teria. None of them had a history of Alzheimer disease from the National Institute on Aging and the Reagan Institute Working devastating as childhood schizophrenia. Case or depression. At Group on diagnostic criteria for the neu- MRI—performed with high resolution T2/ descriptions became available soon after ropathological assessment of Alzheimer dis- schizophrenia was first recognised as an FLAIR sections, using transverse and coronal ease. J Neuropathol Exp Neurol 1997;56:1095– planes to obtain a good visualisation of the 7. entity but a childhood onset is fortunately mesial temporal cortex (hippocampal and 10 Gearing M, Mirra S, Hedren J, et al. The rare. Early samples probably included chil- consortium to establish a registry for parahippocampal gyri)—all of them exhibited dren with autistic spectrum disorders as these Alzheimer’s disease (CERAD). Part X. Neu- were only recognised in the 1940s. In spite of atrophic changes consistently involving and ropathology confirmation of the clinical diag- yet exceeding the hippocampal structures, in nosis of Alzheimer’s disease. Neurology 1995; shared deficits there are important clinical the absence of focal lesions attributable to 45:461–6. diVerences between autism and childhood vascular or other diseases. With regard to this 11 Hodges JR, Patterson K. Is semantic memory schizophrenia. The wealth of research into consistently impaired early in the course of autism has not been matched in the field of last point, it seems to us noteworthy that Alzheimer’s disease? Neuroanatomical and 2 childhood schizophrenia, perhaps because of Helmstaedter and Elger, studying 63 pa- diagnostic implications. Neuropsychologia 1995; tients with chronic pharmacoresistant MTLE 4:441–59. its rarity but also because clinicians can draw sustained by HS, found significant diYculties on the findings on adult disorders. This book with episodic memory tasks (learning, re- is written by experts in the field and helpfully trieval, and recognition of a word list), but not describes current thinking and knowledge in with semantic memory tasks (vocabulary). In childhood schizophrenia. It includes a chap- our FTLD group, conversely, the presence of ter on historical aspects, and specially useful semantic memory impairment in association for clinicians, developmentally informed with episodic memory deficit was in agree- BOOK REVIEWS guidelines on diVerential diagnosis and man- ment with the typical progression of AD agement. There is a full discussion on the use pathological process, spreading from the of traditional and newer anti-psychotic medi- mesial temporal areas to the adjacent tempo- cation. ral neocortex.11 Incidentally, in four patients Authors make well the case for the with FTLD who completed a clinical and Intractable focal epilepsy. Edited by JOHN similarities between childhood and adult neuropsychological follow up of at least 3 OXBURY, CHARLES POLKEY, and MICHAEL schizophrenia, they document the empirical years an evolution towards a more diVuse evidence indicating increased pre-morbid DUCHOWNY (Pp 878, £125.00). Published by pattern of cognitive impairment has now developmental and personality anomalies, WB Saunders, London, 2000. ISBN been found. In two other patients with perhaps a stronger genetic vulnerability and FTLD, who recently underwent neuro- 0-7020-2428-7. worse outcome with a childhood onset. psychological retesting after a 12–15 month Research findings are detailed on information copyright. interval from the baseline assessment, a This is quite a comprehensive multiple processing deficits and on anomalies of significant worsening of cognitive deficits, author textbook which sets out in detail clini- thought processes comparable to those seen which nevertheless still remained relatively cal, pathological, investigative, and medical in adult patients. The important on-going circumscribed to episodic and semantic and surgical management of patients with research by the National Institute of Mental memory, has been documented. focal epilepsy. The standpoint of the editors is Health is referenced. Outstanding areas for In conclusion—and also in the light of the to use the words “focal epilepsy” rather than future research are identified. This should above cited studies investigating the relations partial epilepsy because they think that it is attempt a better description of pre-morbid between HS and dementia—we think that the more descriptive of the probable underlying developmental and behavioural anomalies clinical characteristics of our patients with pathophysiological changes. As such, the currently subsumed under a bewildering FTLD were hardly explainable in terms of an book is of particular interest to epileptologists array of overlapping syndromes and their dif- underlying HS and more consistent with the and other specialists likely to be involved in ferentiation from those seen in autism. diagnosis of probable AD. developing an epilepsy surgery programme. This book represents a welcome update on G VILLA Many of the other epilepsies would of course research and clinical thinking in childhood http://jnnp.bmj.com/ A CAPPA have more widespread or multifocal underly- schizophrenia and will be useful to clinicians. C MARRA ing substrates or generator circuits. G GAINOTTI ELENA GARRALDA Servizio di Neuropsicologia, Istituto di Neurologia, The selection of the editors for individual Università Cattolica del Sacro Cuore, Policlinico A chapters incorporates a diverse range of spe- Gemelli, Largo Agostino Gemelli 8, I-00168 Roma, cialists principally from Europe and North Charcot-Marie-Tooth disease. A Italy America. As such there is a useful diversity of practical guide. Compiled by CMT Correspondence to: Dr G Villa views represented and many of the authors INTERNATIONAL UK (Pp 113, £10.00). [email protected] selected are acknowledged world experts in

Published by CMT International UK, on September 26, 2021 by guest. Protected the areas of their chapter. Penarth, 2000. ISBN 0 9533883 0 1. 1 Cappa A, Calcagni ML, Villa G, et al. Brain The text is clearly written with good perfusion abnormalities in Alzheimer’s disease: comparison between patients with focal tempo- illustrations and high quality photographs. Charcot-Marie-Tooth disease. A practical guide, ral lobe dysfunction and patients with diVuse There is a standard useful summary of key is a book compiled by CMT International cognitive impairment. J Neurol Neurosurg Psy- points with each chapter, and clearly there UK with the aim of providing an overview of chiatry 2001;70:22–7. 2 Helmstaedter C, Elger CE. The phantom of has been significant editorial input to pro- Charcot-Marie-Tooth disease (CMT) with a progressive dementia in epilepsy. Lancet 1999; duce an easily readable textbook with com- particular emphasis on providing practical 354:1225–30. mon themes. day to day advice for living with the disease. It 3 Ala TA, Beh GO, Frey WH 2nd. Pure hippocampal sclerosis: a rare cause of dementia The text covers many of the areas needed is aimed at doctors and patients and other mimicking Alzheimer’s disease. Neurology to support an epilepsy surgery programme, people involved with CMT . It is well written 2000;54:843–8. although it might be advantageous in a future and excellently presented and provides a 4 Corey-Bloom J, Sabbagh MN, Bondi MW, et al. Hippocampal sclerosis contributes to dementia edition to include further information about range of information that the intended audi- in the elderly. Neurology 1997;48:154–60. the rapidly expanding field of neurogenetics ence will find invaluable. 5 Crystal HA, Dickinson D, Davies P, et al. The and epilepsy. The overall quality of the mate- The book is divided into three main relative frequency of “dementia of unknown etiology” increases with age and is nearly 50% rial would more than justify its inclusion in a sections. The first section deals with genetic in nonagenarians. Arch Neurol 2000;57:713–7. specialist library or to support physicians or and medical issues. The known genetic 6 Dickinson DW, Davies P, Bevona C, et al. Hip- associated specialists working in the field of variants of the disease are well described and pocampal sclerosis: a common pathological accurate except for one mistake stating that feature of dementia in very old (> or = 80 years epilepsy surgery. of age) humans. Acta Neuropathol (Berl) 1994; the gene duplication that causes CMT1a is 88:212–21. DAVID FISH on chromosome 22 when it is actually on

www.jnnp.com 418 J Neurol Neurosurg Psychiatry 2001;71:412–418 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.71.3.412 on 1 September 2001. Downloaded from chromosome 17. I thought the section cover- the advice given (for example, on driving), drawings and good illustrations of pathways ing CMT inheritance was particularly well somewhat distracting. Nevertheless, it should but some poor thumbnail sketches. Some presented and illustrated using simple dia- prove useful reading for those involved in the illustrations have been recycled from the grams to explain the various inheritance care of people with epilepsy. author’s anatomy text without adaptation for mechanisms. There was also a very useful their new context. Imaging material is varied YVONNE HART glossary of scientific and medical terms in and usually well chosen. The tables tend to be this section. congested with information and are not likely The second section deals with living with Clinical neuroanatomy for medical to be found helpful. CMT. This covers many important areas for students, 5th edition.ByRICHARD S SNELL The short time available for studying neu- the patient including coming to terms with (Pp 545, £29.95). Published by Lippincott roanatomy in most curricula means that stu- the diagnosis, care of the feet, pain control, Williams and Wilkins, Baltimore, 2001. dents tend to use textbooks to clarify the and secondary complications. Foot deformi- deficiencies of their lecture notes rather than ties and their surgical correction were par- ISBN 0 7817 2831 2. for systematic reading. The index is fairly ticularly well covered. comprehensive and will usually take the Richard Snell’s Clinical anatomy for medical The third section deals with practical issues reader to the right place. students is a successful clinically oriented text including finding work, having a baby, In summary, Professor Snell has not yet that contains essential facts and explanations driving, and CMT and aids to daily living. produced the compact, clear guide to the without excessive detail. His parallel neuro- This section is particularly useful in providing anatomical basis of clinical neurology needed anatomical text has similar aims. contact details for many diVerent organisa- by modern medical students; the message is Each chapter begins with chapter objec- tions who will help patients. All three sections still buried in excessive anatomical detail and tives; these are actually a brief explanation of are supported by informative appendices. terminology. However, his book deserves rec- broad aims for learning rather than specific This book is an excellent patient oriented ommendation for its clinical notes. These educational objectives. There follow the main guide, full of useful information and contacts. could provide the starting point for defining anatomical information of the chapter. New It will be a particularly useful book to recom- the core anatomical knowledge in a future terms are printed in bold text, a style that is mend to newly diagnosed patients. edition. less than eVective as attention is drawn to MARY REILLY these rather than to key points of understand- ing. Next comes an extensive section of clini- Clinical neuroanatomy. An illustrated cal notes that constitute the greatest strength review with questions and explanations, Epilepsy in clinical practice. A case of the book. They make clear the ways in .ByRICHARD S SNELL (Pp 256, study approach. Edited by ANDREW N which anatomical knowledge underpins diag- 3rd edition WILNER (Pp272, US$34.95). Published by nosis and management, and provide the stu- £19.95). Published by Lippincott Williams Demos Medical Publishing, New York, dent with valuable motivation to gain control and Wilkins, Baltimore, 2001. ISBN 0 7817 2000. ISBN 1-888799-34-x. of the key facts. Understanding is then tested 2989 0. by a set of clinical problem solving scenarios This is an interesting book, directed at a wide with answers. The last element is a set of This text recycles information and illustra- audience including neurologists, medical stu- review questions in a multiple choice format, tions from his larger textbook in a form dents, social workers, and pharmacists, which many of which test topographical neuro- designed for rapid revision and as a reminder sets out to demonstrate the management of anatomy at an excessively detailed level. of forgotten neuroanatomy for clinical attach- copyright. epilepsy in practice by presenting case histo- The first three chapters cover gross anat- ments and recent graduates. Use of clinical ries of people with seizures. The issues omy and cell biology of the nervous system notes and review questions parallels the larger addressed include such common clinical dif- and the fundamentals of nervous system text, though the chapter organisation is ficulties as diagnosis (and the problem of function. These take things fairly gently and a somewhat diVerent. The factual content has non-epileptic attacks), epilepsy in pregnancy, good deal of the material will be familiar to a been reduced in length, often to sequences of withdrawal of antiepileptic drugs, and status medical student who already has a reasonable short statements closer to lists than to expla- epilepticus. Alternative therapies and psychi- knowledge of anatomy and physiology. The nation. The quality of illustration is also con- atric issues are also discussed. Each history is material in the functional chapter in particu- siderably reduced by exclusion of colour. followed by questions about the management lar is rather variable in level and quality. The result is about half the length of the of the patient, which the author answers The next four chapters provide a fairly main text and two thirds of the price. It is according to his practice, with appropriate conventional ascending regional treatment of hard to imagine why a student or junior doc- explanations. The second part of the book the CNS from spinal cord through brain stem tor who has used the larger book or some consists of a sizeable Resources section provid- and cerebellum to cerebrum. The author’s other clinical anatomy text would wish to buy ing such diverse information as the classifica- intention to provide an essential core of this considerably inferior product, still far too http://jnnp.bmj.com/ tions of epileptic seizures and syndromes, sei- knowledge has here been overwhelmed by large to provide headlines for storage in short zure history , home safety , reluctance to leave anything out. term memory. lists of drugs and their modes of action, web- The next nine chapters are much better. ANTHONY FIRTH sites for physicians and patients, lists of These deal with the nervous system in a sys- epilepsy centres, and driving regulations tems based way, covering such themes as cor- within the United States. tical organisation, the reticular formation, the Both sections of the book contain a wealth limbic system, and the hypothalamus, with of information, and the histories, which are balance and clarity. The chapter on the auto-

easy to read, provide a useful insight into the nomic system will help students who find the CORRECTION on September 26, 2021 by guest. Protected problem areas of epilepsy, together with pos- principles diYcult, although it is disappoint- sible solutions. Inevitably the views given ing that the aVerent component of the describe the author’s personal practice, and parasympathetic system is not considered as Frisoni GB. Structural imaging in the clinical the nature of the book does not allow detailed an entity. The coverage of cranial nerves suf- diagnosis of Alzheimer’s disease: problems examination of the evidence underlying the fers from insuYcient explanation of the com- and tools. J Neurol Neurosurg Psychiatry decision making. However, it emphasises the ponents of a cranial nerve—the term “bran- 2001;70:711-18. The following acknowledg- importance of tailoring treatment to the indi- chiomotor” is used but not explained. The ments should have appeared in this editorial: vidual patient, and addresses the social issues final chapter, on development of the nervous “The ideas of this paper have arisen from in a manner often missing from larger texts. I system, seems to have been included for extensive discussion with Marco Trabucchi. I found the fact that the book was clearly completeness rather than out of any convic- am also indebted to Alberto Beltramello, directed at an American audience, which was tion that it matters. Charles DeCarli, Mikko Laakso, and Hilkka apparent not only in the Resources section, but Illustrations are very variable in quality, Soininen for helpful suggestions and com- also colouring the choice of medication and with excellent three dimensional half tone ments”

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