420 Letters to the Editor

The physiological response to hypother- headache and fever. Walking was difficult, ments. Apart from the myoclonus, no other J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.61.4.420 on 1 October 1996. Downloaded from mia is controlled by the hypothalamus, although she felt better when standing. She evidence of spinal cord disease was appar- involving peripheral vasoconstriction and had a mild motor deficit in the proximal part ent. The treatment of the Borrelia rapidly shivering. In hypothalamic hypothermia of the lower limbs, patellar tendon reflexes relieved the pain and dramatically sup- these systems fail with loss of reactive abolished on the right, diminished on the pressed the myoclonus. peripheral vasoconstriction to reduce heat left, normal ankle reflexes, plantar reflexes V DE LA SAYETTE S SCHAEFFER loss and loss of the shivering response to flexor, and axial muscles and neck were not C QUERUEL produce heat. It is the failure of these sys- rigid. Results from routine laboratory inves- F BERTRAN tems that contributes to the hypothermia tigations were normal. Ketoprofene, G DEFER Service de Neurologie and also produces diagnostic difficulty, with haloperidol, clorazepate, and then tiaprid, P HAZERA the patient feeling warm to the touch and paracetamol, and buprenorphine were tried E GALLET not shivering. The ECG showing the with negligible relief. On 19 October, she Service de Rianimation midicale et maladies pathognomonic J waves, with absence of was transferred to the intensive care unit. infectieuses, CHU Caen, France shiver waves mirrored the hypothalamic The painful jerks were flexor, simultaneous Correspondence to: Dr V de la Sayette, Service de cause of the hypothermia. in all the muscles, and spontaneous or neurologie, CHU C6te de Nacre F 14033 Caen This is the first description of hypother- induced consistently by flexion of the neck, cedex, France. mia in a patient with multiple sclerosis with without involvement of the face and superior 1 Viader F, Poncelet AM, Chapon F, et al. Les a proved hypothalamic plaque and no other limbs. The intervals between the jerks formes neurologiques de la maladie de identifiable cause for hypothermia. became so short that the paroxyms gave the Lyme. Rev Neurol 1989;145:362-8. impression of being attacks of sustained 2 Stiernstedt GT, Granstom M, Hedrstedt B, We thank David Hughes for the preparation of the Skoldenberg B. Diagnosis of spirochetal histopathological photographs. truncal flexion. An EEG during jerking was meningitis by enzyme-linked immunoasor- unremarkable. Finally, the patient was bent assay and indirect immunofluorescence S EDWARDS anaesthetised and ventilated artificially. The assay in serum and cerebrospinal fluid. J Clin G LENNOX Microbiol 1985;21:819-25. K ROBSON treatment was propofol, fentanyl, and mus- 3 Brown P, Thompson PD, Rothwell JC, Day A WHITELEY cle relaxant pancuronium. Ceftriaxone (2 g BL, Marsden CD. Axial myoclonus of pro- Department ofNeurology, intravenously daily) was given for 14 days. priospinal origin. Brain 1991;114: 197-214. Queen's Medical Centre, Nottingham The CSF contained 398 mononuclear 4 Brown P, Thompson PD, Rothwell JC, Day BL, Marsden CD. Paroxysmal axial spasms Correspondence to: Dr S Edwards, Department of cells/,ul, numerous atypical cytological fea- of spinal origin. Mov Dis 1991;6:43-8. Academic Neurology, Queen's Medical Centre, tures, normal glucose and chloride ratios, 5 Martin R, Meinck HM, Schulte-Mattler W, Nottingham, NG7 2UH, UK. increased protein content (1 -2 g/l), intrathe- Ricker K, Mertens HG. Borrelia Burgdorferi and three myelitis presenting as a partial stiff man syn- 1 Benarroch EE. The central autonomic net- cal synthesis of IgG and IgM, drome. JNeurol 1990;237:51-4. work: functional organisation, dysfunction, oligoclonal bands were detected. The titre of and perspective. Mayo Clin Proc 1993;68: antibodies to Borrelia burgdorferi was raised 988-1001. in the CSF (1/64: normal < 1/4) by indirect 2 Geny C, Pradat PF, Yulis J, Walter S, Cesaro D, Degos JD. Hypothermia, Wemicke's immunofluorescence, both for IgM (1/16) Metamorphopsia and visual hallucina- encephalopathy and multiple sclerosis. Acta and IgG (1/16), 1-352 (normal < 0-16) by tions restricted to the right visual hemi- Neurol Scand 1992;86:632-4. enzyme linked immunosorbent assay field after a left putaminal haemorrhage 3 Sullivan F, Hutchinson M, Bahandeka S, borrelia Moore RE. Chronic hypothermia in multiple (ELISA) (Immunowell Lyme- sclerosis. J Neurol Neurosurg Psychiatry 1987; BMD); their detection in serum was nega- Metamorphopsia is a rare neurological phe- 50:813-5. tive three weeks later. On 22 October the nomenon in which objects appear distorted 4 Lammens M, Lissoir F, Carton H. patient was extubated. The jerks had totally in form. Many reports have attributed the Hypothermia in three patients with multiple sclerosis. Clin Neurol Neurosurg 1992;91: disappeared and the pains dramatically responsible lesion to the occipitoparietal cor- 117-21. improved. At this time, EMG failed to tex and its related structures.l We report a 5 Ghawche F, Destee A. Hypothermie et scle- detect any myoclonic jerks. Recording of case of left putaminal haemorrhage followed rose en plaques. Un cas avec trois episodes evoked by metamorphopsia and visual hallucina- d'hypothermie transitoire. Rev Neurol Paris peroneal nerve somatosensory poten- 1990;146:767-9. tials and MRI of the spine were unremark- tions restricted to the right visual hemifield. able. On 24 October, the patient was free of The origin of this patient's symptoms was pain and then recovered full strength and considered to be the left optic radiation. normal tendon reflexes. A 63 year old right handed man with a

Lyme neuroborreliosis presenting with The clinical features of pain resistant to previous history of hypertension was admit- http://jnnp.bmj.com/ propriospinal myoclonus analgesic agents,' meningoradiculitis with a ted to the hospital with acute right hemi- history of tick bite, and erythema migrans paresis. On admission, his visual field A 60 year old white woman presented with strongly evokes a Lyme neuroborreliosis examination showed a right homonymous an erythema migrans after a tick bite on the confirmed by the CSF findings and detec- hemianopia. There was also a right inferior right thigh on 1 1 July 1995. On 2 October tion of antibodies to Borrelia burgdorferi.2 facial palsy and a right hemiparesis without 1995, she complained of a lumbar pain However, the most dramatic feature was the sensory involvement. The right homony- which radiated to the right thigh. She myoclonic jerks which support the clinical mous hemianopsia disappeared on the third received dextropropoxyphene, paracetamol, diagnosis of propriospinal myoclonus char- day. On the fourth day, he complained that thiocolchicoside, and tetrazepam and then acterised by repetitive, non-rhythmic jerks of the doctor's left cheek seemed to have been on September 27, 2021 by guest. Protected copyright. was additionally treated with codeine, the neck, trunk, both hips, and knees.3 scraped, that the doctor's left hand seemed chlormezanone, and tenoxicam. Despite Sometimes attacks of sustained truncal flex- tortuous, and that some of the fingers of the this, the pains, which prevented sleep, ion are generated by paroxysmal bouts of hand seemed to be missing. He drew a pic- rapidly radiated bilaterally to the low back axial jerks.4 In this type of myoclonus, the ture of what he saw (figlA). Visual field and the abdomen. On 1 lOctober 1995 she discharge arises from a limited segment of examination by confrontation was immedi- presented with flexor non-rhythmic symmet- the spinal cord and then spreads slowly up ately performed but no abnormalities were ric jerks of the trunk, the abdomen, both and down by the involvement of the long found, later confirmed by using Goldmann's hips, and knees evident both sitting and propriospinal pathways.3 The jerks had dis- perimeter. On the next day, he complained, standing, increasing when lying, without appeared at the time of the EMG investiga- "The right half of the curtain in front of me suppression by an effort of will or during tion in our patient. Accordingly, we could suddenly transforms into an animal's face. It voluntary movements. Although the pains not ascertain the possible origin in the tho- rotates there for a while and finally flows to and jerks were atypical, the patient was diag- racic segment of the spinal cord, corre- the right, and then disappears. At the next nosed as having a herniated disc; an epidural sponding to the abdominal and lumbar moment, another face springs up at the very infiltration of dexamethasone (10 mg) gave a muscles, which were painful throughout the portion and. . ." He then drew a picture to transient relief of the pains and jerks. A sec- course of the disease and constantly affected illustrate his experience (fig 1B). These phe- ond infiltration was not effective. On 13 by the jerks. To our knowledge, no case of nomena lasted three to four days and then October the patient was admitted to hospi- Lyme neuroborreliosis has been associated disappeared. One month later, he was able tal. The myoclonic jerks had reinforced, with a propriospinal myoclonus. Another to walk without assistance and was dis- occurring sometimes in bursts, occasionally patient had stiffness, painful cramps, and charged from hospital. involving the neck and the shoulders but spasmodic jerks confined to the left leg,5 The laboratory analysis of blood and never the face. The patient was agitated and which suggest a localised myelitis of the urine was within the normal range. Cranial exhausted, and cried on account of the con- spinal intemeurons. Our own strongly CT on admission showed a left putaminal tinuous distressing pains. She was free of evokes the involvement of many spinal seg- haemorrhage without ventricular extension