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Malignant Hyperpyrexia We Report a Case of Neuromyotonia in a Patient

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Malignant Hyperpyrexia We Report a Case of Neuromyotonia in a Patient 556 Letters to the Editor J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.5.556 on 1 November 1995. Downloaded from mmol/l), total bilirubin (1 59 mg/dl), >- Neuropathological studies in patients with glutamyl transferase (73 U/1), and pro- Wemicke's encephalopathy have shown thrombin time (1-37 international symmetric lesions in the paraventricular normalised ratio, normal values from 1-00 to regions of the thalamus and hypothalamus, 1*27). Examination of CSF one day after in the mamillary bodies, periaqueductal admission showed a raised CSF/serum albu- region of the midbrain, floor of the fourth min ratio (12-5), but a normal cell count (no ventricle, and midline structures of the cere- malignant cells were detected) and glucose bellum.2 Histologically, the acute lesions concentration; fluid was sterile on culture. were found in and around blood vessels. Oligoclonal IgG bands were absent in the Hypertrophic endothelial cells, demyelina- CSF. A cranial CT on admission as well as tion, loss of neuropil, proliferation of astro- bilateral carotid angiography three days after cytic and microglial cells, and relative admission were normal. Five days after preservation of neurons have been reported admission her mental state deteriorated. She in neuropathological studies. Periventricular was intubated and transferred to the neurol- haemorrhages, usually petechial in size, may ogy intensive care unit. On admission there be found in up to 20% of cases. Two she was comatose without reaction to patients were reported to have died of exten- painful stimuli. Oculocephalic responses sive haemorrhagic brainstem lesions were absent. The isocoric pupils had slight detected on necropsy.3 bilateral reaction to light. Corneal reflexes Wemicke's encephalopathy remains a were symmetrically present. An EEG clinical diagnosis, because of the insen- showed diffuse slowing. She had hypo- sitivity of neuroimaging in this setting. Initial tension requiring fluid administration, CT in our patient was normal. Hae- dopamine, and noradrenaline. She had a morrhages due to Wemicke's encephalo- fever (39°C) and a raised C-reactive protein pathy detected on CT or MRI are seldom concentration (13-8 mg/dl); antibiotic treat- reported in the medical literature. In one ment (imipenem plus erythromycin) was ini- report scattered haemorrhages in the thalami tiated. Repeat cranial CT was normal. and posterior diencephalon in a patient with Twenty four hours after admission at the Wernicke-Korsakoff syndrome were shown intensive care unit she acutely developed by CT.4 In a case report a small haemor- bilateral dilated areactive pupils. Immediate rhagic lesion adjacent to the body of the lat- CT showed severe intraventricular haemor- eral ventricle was discovered on MRI.5 The rhage (figure, A). Coagulation studies (pro- severe intraventricular haemorrhage due to thrombin time, partial thromboplastin time, Wernicke's encephalopathy as seen in our platelet count) determined on the same day patient raises the question of whether spon- as well as the day before intraventricular taneous bleeding of Wernicke's encephal- haemorrhage were normal. The patient had opathy was accentuated by an associated no clinical signs of cutaneous or mucous coagulopathy. At the time of ventricular bleeding. Despite immediate insertion of haemorrhage the data on clotting studies bilateral intraventricular drains the patient were normal. Moreover, the patient had not died 14 hours later. received antiplatelet agents. At necropsy the brain weighed 1440 g and HANS-WALTER PFISTER FRIEDRICH VON ROSEN showed some arteriosclerosis of the great Department ofNeurology arteries at the base of the brain. An KARL BISE aneurysm was not found in the circle of Institute for Neuropathology, Ludwig-Maximilians-University ofMunich, (a) Demonstration ofhaemorrhage by CT Willis. Coronal sections showed pronounced Germany (without contrast) in the third ventricle, ambi- haemorrhages at the walls of the third ven- ent, and quadrigeminal cistern. (b) Coronal tricle bilateral, which extended into the third Correspondence to: Professor Hans-Walter section showed a pronounced haemorrhage in ventricle, the aqueduct, and the floor of the Pfister, Ludwig-Maximilians-University, Depart- ment of Neurology, Klinikum GroBIhadern, the third ventricle (arrow). In addition, small fourth ventricle (figure, B, C). There was MarchioninistraBe 15, 81377 Munich, Germany. http://jnnp.bmj.com/ areas of haemorrhagic discolouration in corti- destruction of the mamillary bodies by callsubcortical regions secondary to agonal haemorrhage. In addition to petechial haem- 1 Naidoo DP, Bramdev A, Cooper K. Wemicke's raised intracranial pressure are shown. (c) A encephalopathy and alcohol-related disease. necrotising haemorrhagic lesion in the floor of orrhage, microscopy showed prominent and Postgrad Med J 1991;67:978-81. the fourth ventricle (arrow), small areas of dilated capillaries, reactive astrocytes in the 2 Victor M, Adams RD, Collins GH. The haemorrhagic necrosis in cortical regions sec- periventricular regions around the third and Wernicke-Korsakoff syndrome and related disor- ondary to agonal raised intracranial pressure, fourth ventricles and aqueduct, and mild ders due to alcoholism and malnutrition. 2nd in ed. Philadelphia; Davis, 1989:1-117. and subarachnoid haemorrhage, especially neuronal loss. 3 Harper C. Wemicke's encephalopathy: a more the basal cisterns and the leptomeninges of the The clinical diagnosis of Wemicke's common disease than realised. A neuro- cerebellar vermis secondary to intraventricular pathological study of 51 cases. .7 Neurol encephalopathy is often overlooked' and was on September 23, 2021 by guest. Protected copyright. haemorrhage. Neurosurg Psychiatry 1979;42:226-31. not suspected in our non-alcoholic patient 4 Roche SW, Lane RJM, Wade JPH. Thalamic during her lifetime. Her mental abnormali- hemorrhages in Wemicke-Korsakoff syn- and cystectomy with construction of an ileal ties had aroused clinical suspicion of carci- drome demonstrated by computed tomogra- nomatous meningitis, paraneoplastic phy. Ann Neurol 1988;23:312. conduit were performed. Two and a half 5 Schroth G, Wichmann W, Valavanis A. Blood- weeks before admission the patient devel- disorder, cerebral vasculitis, or sinus throm- brain barrier disruption in acute Wemicke oped a rectovaginal fistula; the rectum and bosis. A recent necropsy study showed that encephalopathy: MR findings. 7 Comput sigmoid colon were resected and an end 80% of patients with the Wemicke- Assist Tomogr 1991;15:1059-61. colostomy and another ileal conduit were Korsakoff syndrome were not diagnosed constructed. Oesophagogastroscopy, per- during life (Harper et al, 1986, cited in formed because of vomiting four days before Naidoo et all). Apart from patients with a admission, showed candidiasis of the history of chronic alcoholism, Wemicke's Neuromyotonia in association with oesophagus. Neurological examination encephalopathy can be found in other med- malignant hyperpyrexia showed that the patient was disoriented in ical settings-for example, malnutrition, time, had a right sided abducens paresis, prolonged intravenous feeding, cancer, gas- We report a case of neuromyotonia in a horizontal gaze evoked nystagmus to the left, tric plication, and acquired immuno- patient with malignant hyperpyrexia. and a vertical gaze evoked nystagmus deficiency syndrome. ' 2 The extensive The patient, a 19 year old woman, has upward. The tendon reflexes were normal. abdominal surgery and vomiting before had several discrete episodes of muscular She had no paresis of the extremities, but admission may have led to decreased food stiffness over her lifetime and several years slight dysmetria of the arms and legs. There uptake and thiamine deficiency in our of twitching in the hand and calf muscle. were no sensory abnormalities. Routine patient. Parenteral nutrition in the intensive Episodes of calf muscle contracture and pain laboratory tests were normal except for care unit probably aggravated Wernicke's had occurred from the age of 5 with two serum glucose (243 mg/dl), potassium (2-4 encephalopathy. severe episodes at the ages of 5 and 16. Letters to the Editor 557 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.5.556 on 1 November 1995. Downloaded from associ- P K NEWMAN University Department ofClinical Neurology, Some of the episodes may have been Neurology Department, Institute ofNeurology, ated with fever. A third severe episode Middlesbrough General Hospital, Queen Square, occurred at the age of 19 two weeks after Ayresome Green Lane, London WClN 3BG, UK normal vaginal delivery of a healthy child. Middlesbrough, Cleveland, developed rapid onset of spasms affect- 1 Smith CAD, Cough AC, Leigh PN, Summers She TS5 SAZ, UK BA, Harding AE, Maraganore DM, et al. ing her legs and arms which progressed so M C BATESON Debrisoquine hydroxylase gene polymor- that she had difficulty breathing and swal- Bishop Auckland General Hospital, phism and susceptibility to Parkinson's dis- lowing. She was also vomiting. Examination Cockton Hill Rd, ease. Lancet 1992;339:1375-7. Bishop Auckland, 2 Armstrong M, Daly AK, Cholerton S, showed a temperature of 42°C with tachy- County Durham, Bateman DN, Idle JR. Mutant debrisoquine cardia and tachypnoea and generalised BL14 6AD, UK hydroxylation genes in Parkinson's disease. muscular spasm. Her creatine kinase was Lancet 1992;339: 1017-8. to Dr Newman. raised at 740 IU/l and investigation for an Correspondence 3 Kurth
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