J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.5.487-a on 1 May 1985. Downloaded from

Letters 487 G GOZETt accompanied by a feeling of stiffness and routine analytical and radiological tests. M RIBET1 pain in the forearm and wrist. Any other The patient refused specific studies. B HUBSCHMAN§ manual activity was carried out normally. These patients were considered to suffer P LESTAVEL§ The difficulty in writing had remained from simple writer's cramp.'5 According to stable since the onset of the symptoms but Marsden,s even simple writer's cramp is a Neurological service. Hopital B, its intensity varies at times and is "minor" form of dystonia. Occasionally, *Neuroradiological service, t influenced by the emotional state of the some patients with writer's cramp provide General surgical servicet patient. On examination, moderate obesity data suggestive of familial involvement,'6 Centre hospitalier Universitaire de Lille. and very light postural tremor in upper but there is no clear evidence of genetic 59037 Lille Cedex France limbs were noted; in addition, there was a factors in most patients with writer's Internal medicine service. § minimal tremor in the finger-nose-finger cramp.'47 Writer's cramp was obviously Centre hospitalier d'Arras test. His writing was legible, but he wrote familial in our cases, as in other variants of References with moderate difficulty, with the wrist focal dystonia.'9 This observation favours tending to extension from the start, while an organic origin of the disorder. 'Killen DA, Furster JH, Gorbel WG Jr, et al. the fingers bent together clutching the pen. P MARTINEZ-MAR1N' The subclavian steal syndrome. J Thorac The pressure on the paper was slightly F BERMEJO PAREJAt Cardiovasc Surg 1966;51:539-60. increased. There was no micrographia. Servicio de 2 Testut L. Angdiologie. Systeme nerveux cen- Neurologia tral. In: Trait6 drAnatomie Humaine. 5th These signs lasted as long as writing con- Hospital Nacional de Enfermedades ed. Paris, Doin 1905, 2. tinued. The remainder of the examination Infecciosas* 3 Haughton VM, Rosenbaum AE. The normal was normal. Routine haematological and C/Sinesio Delgado, 6 and anomalous aortic arch and biochemical studies, uric acid, LE 28029 - Madrid brachiocephalic arteries. In: Newton TH, phenomenon, rheumatoid factor, radio- and Potts DG, eds. Radiology of the Skull and graphs of the chest and skull, ECG, brain Servicio de Neurologia Brain: Angiography. Saint Louis; CV high-resolution CT with contrast, cerulo- Ciudad Sanitaria "1° de Octubre"t Mosby, 1974;2 (2):1145-63. plasmin and cornea were normal. guest. Protected by copyright. Lesoin F, Bousquet C, Thomas CE, Quandalle Madrid, Spain P, Ribet M, Jomin M. Transient ischaemic The father of the propositus is a 70- attacks in patient with endothoracic goitre year-old retired lawyer, right-handed. References and congenital anomaly of the carotid Since the age of 35 he had experienced a Sheehy MP, Marsden CD. Writer's cramp. A arteries. Lancet 1982;2:98. non-progressive severe difficulty in writing, focal dystonia. Brain 1982; 105:461-80. consisting of abnormal posture of the hand, 2 Editorial. Writer's cramp. Lancet 1982;iH:969. Accepted 22 September 1984 inability to maintain the pen in the correct 3 Simmons VP. Writers cramp (letter). Lancet position, involuntary extension of the index 1982;Hl: 1220. finger ("it escapes"), and tremor. All these Hudgson P. Writeres cramp. Br Med J symptoms were present only during writing 1983;286:585. Familial writer's cramp or detailed drawing. The resulting script Marsden CD. Dystonia: The spectrum of the disease. In: was almost illegible, and the difficulty in lia. New York:YahrRavenMD, Press,ed. The1976:351-67.Basal Gang- Sir: The aetiology of writer's cramp is still writingwriting so markedmarked'thatthat he has used a 6Gowers WR. A Manual of Diseases of the controversial.'-4 In spite of the facts that typewriter from the onset of trouble. He Nervous system. Vol. 2. London: Churchill, favour an organic origin, genetic influence has practised artistic painting without prob- 1888:656-74. ,on this anomaly has not been demon- lems except for delicate drawings, when aid 7 Marsden CD. Writer's cramp (letter). Br MedJ strated. We report a patient with writer's with the left hand becomes neccesary. For 1983;286:1057. cramp, whose father had presented the 2-3 years before consultation, he has had Gilbert GJ. Familial spasmodic torticollis. same disorder 35 years previously. cephalic tremor and postural tremor in Neurology (Minneap) 1977;27:11-3. The propositus was a 41-year-old male limbs. He had no other symptoms. 9 Nutt JG, Hammerstad JP. Blepharospasm upper oromandibular dystonia (Meige's syndrome)and revealed s ght headtremor, right-handed lawyer. He had a previous ExaminationuppEramination revealed slight head tremor, in sisters. Ann Neurol 1981;9:189-91. http://jnnp.bmj.com/ history of hyperuricaemia and gout treated mild postural tremor in upper limbs and with allopurinol, 100 mg/day. He had not very slight tremor on finger-nose testing. A received any other specific treatment moderate increase in tone in wrists, a little Accepted 12 October 1984 before, was not exposed to contact with more marked on the right, with contralat- toxic substances, did not consume illegal eral activity was noted. Writing and draw- drugs and had not suffered any psychiatric ing were very difficult: from the start, the or neurological illness. There was no con- right wrist was placed in extension and Asterixis due to pontine haemorrhage sanguinity between his parents who did not ulnar deviation, with its ventral aspect on belong to a particular ethnic group. The the table and fingers forced in flexion. Sir: Asterixis is a common sign of on September 27, 2021 by patient had had postural tremor since While writing, the posture worsened, a metabolic encephalopathy and arises from youth. At the age of 33 years he suffered remarkable tremor was superimposed and various causes.' 2 This involuntary move- epicondilitis in the right elbow which remit- irregular jerks separated the index finger; ment has also been observed in a focal ted with local therapy. Since then, he had the pen became sustained between thumb, brain lesion.37 In this letter, we describe experienced difficulty in writing with the middle and ring fingers and, eventually, a patient with asterixis due to pontine right hand, that adopted an abnormal pos- escaped. When the attempt to write ceased haemorrhage. ture such that it attracted the attention of all these signs disappeared. The remainder A 65-year-old woman was admitted other persons. The disturbance was of the examination was normal, as was because of left motor weakness and dip- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.5.487-a on 1 May 1985. Downloaded from

488 Letters lopia on lateral gaze. There had been no grams showed symmetrical 8-9 Hz alpha gesting that the haemorrhagic lesion had history of hepatic, renal or pulmonary dis- activity over the occipital region with occa- interrupted the lemniscal sensory volley in ease. The patient denied habitual use of sional diffuse dysrhythmic bursts of 0.5-1.0 the right half of the pons. The components any kind of drugs. The initial examination ms duration. of the SEP following right median nerve- revealed a blood pressure of 200/100 mm Subsequent examination over the next stimulation were detectable with normal Hg and slightly confused mentation. several weeks disclosed improvement in latencies and amplitudes suggesting thaw Neurological examination showed a mild both mental state and ocular movement, the lemniscal sensory pathway remained left motor weakness of the upper and lower which was full in all directions. Diplopia intact.8 These findings was consistent with extremities and left hemisensory loss of all and nystagmus disappeared. However, the the CT scan finding and clinical sensory modalities. Pupils were isocoric and the mild left motor weakness, the severe left testing. light reaction was prompt and complete. hemisensory loss and the severe ataxia on Several reports have appeared of Optic disks were normal. Eye movements the left arm remained. Deep tendon asterixis due to focal brain lesions. Plumrr were full on up and downward gaze. How- reflexes were exaggerated on both sides and Posner9 described a patient with right ever, there were a mild left hemifacial sen- and plantar response was equivocal bilat- parietal empyema who showed asterixis. sory deficit deviation of the jaw to the right erally. There was prominent asterixis of However, as their patient was stuporous, it when the mouth opened, bilateral abdu- hyperextended hands and fingers on both could not be determined whether the dis- cence palsy, mild left facial motor weak- sides. turbance of consciousness was due to mass ness and horizontal rotatory nystagmus on Electromyographic recordings were car- effect of the empyema on the reticular lateral gaze to left and right. Deep tendon ried out and other electrophysiological formation of the brainstem caused reflexes were hyperreactive on both sides. studies including somatosensory evoked asterixis, or to the focal lesion itself. On the- Planter response was extensor bilaterally. potentials (SEPs) were undertaken. Sur- other hand, the disturbance of conscious- Sensory testing revealed severe left face EMG recordings showed intermittent ness of the patient which Tarsy et aP hemisensory disturbance of all modalities. electrical cessations of ongoing EMG dis- reported was considered as a focal sign of There was a marked swing of outstretched charges of both the agonist and antagonist disturbance of reticular formation of the left arm and severe ataxia on finger-to- muscles simultaneously (asterixis). This midbrain. Other reported cases of asterixisguest. Protected by copyright. nose-testing of the left arm. A CT scan asterixis was observed bilaterally. The SEP due to a focal lesion suggest that asterixis revealed a circumscribed high density area component following left median nerve could result from a dysfunction of sen- in the midpons (fig). Electroencephalo- stimulation was completely abolished sug- sorimotor integration occurring in the parietal lobe and the midbrain.4' Unilat- eral asterixis without disturbance of con- sciousness also has been reported. Young et al'° showed that asterixis could be pro- duced by stereotactic thalamotomy. These reports and descriptions indicate that asterixis can be due to a focal lesion in the parietal lobe, thalamus, or midbrain. It is emphasised here that the circumscribed pontine lesion in our case did not extend tce the midbrain or the thalamus. Asterixis has never been observed in a spinal lesion. We thank Dr Naoyasu Motomura who kindly allowed us to examine this patient. YUTAKA KUDO

MITSUHIRO FUKAIhttp://jnnp.bmj.com/ ATSUSHI YAMADOR' Neurology Service, Hyogo Brain and Heart Center at Himeji 520 Saisho-Ko, Himeji, 670 Japan

References on September 27, 2021 by 'Adams RD, Foley J. Neurological changes in more common types of severe liver disease. Trans Am Neurol Ass 1949; 74:217-9. 2 Conn H. Asterixis in non-hepatic disorders. Am J Med 1960;29- 647-61. Fig CT scan without contrast 3 Tarsy D, Lieberman B, Chirico-Post J, Benson enhancement. A circumscribed high density F. Unilateral asterixis associated with a area was seen at the right dorsal part ofthe mesencephalic syndrome. Arch Neurol pontine tegmentum. 1977;34:446-7. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.5.487-a on 1 May 1985. Downloaded from

Letters 489 Degoes JD, Verrous J, Bouchareine A, Ser- 2 Sandyk R. The effects of dopamine-HCL on daru M, Barbizet J. Asterixis in focal brain Matters arising glucose transport in isolated rat adipocytes. lesions. Arch Neurol 1979; 36:705-7. Thesis presented in partial fulfillment for the Donat JR. Unilateral asterixis due to thalamic Cerebral glucose utilisation in Parkinson's degree of MSc (Med) at the University of the hemorrhage. Neurology (Minneap) 1980; disease Witwatersrand, Johannesburg 1984. 30:83-4. Lenzi GT, Jones T, Reid JL, Moss S. Regional '76 Massey EW, Goodman JC, Stewart C, Bran- Sir: We were interested in the paper by impairment of cerebral oxidative metabol- non WL. Unilateral asterixis: Motor integ- Rougemont et all in which no alteration of ism in Parkinson's disease. J Neurol rative dysfunction in focal vascular disease. local cerebral glucose utilisation was found Neurosurg Psychiatry 1979;22:59-62. Neurology (Minneap) 1979;29: 1188-90. between treated and non-treated Parkin- 4Hakim AM, Mathieson G. Dementia in Par- Ericson G, Warren SE, Gribik M. Unilateral kinson's disease: a neuropathologic study. asterixis in a dialysis patient. JAMA sonian patients. However, the same para- Neurology (Minneap) 1979;29: 1209-14. 1978;240:671. meter was found to be moderately Chiappa KH, Ropper AH. Evoked potentials increased in the basal ganglia of these in clinical medicine (second of two parts). N patients compared to controls. In a recent Engl J Med 1982306:1205-11. study2 one of us demonstrated that low 9 Plum F, Posner JB. The Diagnosis of Stupor concentrations of dopamine combined with Sympathetic skin response and Coma. Philadelphia: FA Davis Com- insulin in vitro increased glucose transport pany, 1980, 3rd ed. 129. in the isolated rat adipocytes. However '0 Young RR, Shahani BT. Unilateral asterixis Sir: Techniques for evoking the psycho- produced by a discrete CNS lesion. Trans high concentrations of dopamine combined galvanic response and determining conduc- Am Neurol Assoc 1976;101: 306-7. with high insulin concentrations inhibited tion velocity along autonomic nerve fibres glucose transport. If this occurred in vivo, have long been available' but have met then alterations in dopaminergic function with limited interest in electroneuronogra- (for example decreased dopaminergic phy. The simplicity of Shahani et aPls2 tech- Accepted 10 October 1984 activity) could result in impaired glucose nique of eliciting the sympathetic skin transport in neuronal cells. This would be response makes it particularly suitable in in agreement with the findings by Lenzi the study of the autonomic nervous system guest. Protected by copyright. et aP who demonstrated decreased glucose during routine EMG sessions. In effect, metabolism in the parietal lobe of patients psychogalvanic responses can be easily with hemi-Parkinsonism. Moreover induced by any internal or external Rougemont et al' demonstrated slightly stimulus of sufficient "novelty": compar- increased glucose metabolism in the basal Correction able sympathetic skin response in one hand ganglia of Parkinsonian patients. This, we can be obtained by electrical stimulation of postulate, could result from reduction of " Mitochondrial malic enzyme in Fried- the ipsi- or contralateral wrist, of the dopamine content in these areas with resul- glabella and by a sudden auditory burst reich's ataxia: failure to demonstrate tant compensatory enhancement of insulin reduced activity in cultured fibroblasts" J applied by earphones (fig). Thus, exploring activity in these areas. It is thus possible several eliciting modalities of sympathetic Neurol Neurosurg Psychiatry Vol 48 Page that increased glucose utilisation in the 70-74. skin response may have a localising value. basal-ganglia of Parkinsonian subjects The technique has however some draw- could reflect impaired dopaminergic activ- backs, which, if unrecognised, could result Page 71-Methods ity. The degree of the regional glucose util- isation could thus serve as a marker for loss Male 33yews Column 2, Line 8 should read ' The cells of dopaminergic activity in these areas. from a 175cm2 flask were harvested, Dementia is a common associated symp- a washed, frozen and thawed once in 20 ,ul of tom of Parkinson's disease.4 It is possible distilled water and sonicated on ice with that by normalising glucose transport into two 20 second bursts" the cortical cells which have been shown to b http://jnnp.bmj.com/ have decreased utilisation in Alzheimers type dementia, that the condition can be improved. This could possibly be achieved by administration of insulin, glucose and levodopa. R SANDYK MA GILLMAN South African Brain Research Institute d

Johannesburg, South Africa on September 27, 2021 by I mV References SOOms Fig Sympathetic skin response evoked in Rougemont D, Baron JC, Collard P, et al. Local cerebral glucose utilisation in treated the left hand by (a) stimulation ofleft and untreated patients with Parkinson's dis- median nerve; (b) stimulation ofright ease. J Neurol Neurosurg Psychiatry median nerve; (c) stimulation at glabella; 1984;47:824-30. (d) auditory burst.