Asymptomatic cardiac arrhythmias in periodic paralysis

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154 therapy.4 6 6 Co-trimoxazole has been used successfully as a sole agent in resolving dementia and intestinal involvement in Whipple's disease,6 23 and in conjunction with ampicillin and chloramphenicol in reversing another case of CNS disease.5 Cotrimoxazole penetrates well the blood-brain barrier, confirmed as in this case by CSF antibiotic assay.8 The role of steroids with antibiotics is more controversial,4 18 24 but prednisolone may have been responsible for the rapid improvement in right knee effusion, and in mobility, and the swift resolution of left wrist weakness. Prednisolone has been suggested previously as responsible for recovery from meningeal involvement in Whipple's disease.'" As disordered immunity may play a role in Whipple's disease,25 the use of steroids is perhaps indicated if neurological damage is threatened. Full recovery from nervous system disease however may be limited by gliosis and neuronal loss.'6 Finally, peripheral nervous system involvement should be considered as part of the spectrum of Whipple's disease. We thank Dr G Wakefield, Consultant Neurologist at Royal United Hospital, Bath, for permission to submit this report; also Dr P Burton, Consultant Pathologist and Dr D Reeves, Consultant Microbiologist at Southmead Hospital; and Dr T Moss, Consultant Neuropathologist at Frenchay Hospital, Bristol.

Letters 3 Pallis CA, Lewis PD. Neurology of gastrointestinal disease. In: Vinken PJ, Bruyn SW, eds. The Handbook of Neurology. Amsterdam: North Holland Publishing Co., 1980: Vol. 39(1I), 449-68. 4 Comer GM, Brandt LJ, Abissi CJ. Whipple's disease: a review. Am J Gastro 1983;78: 107-14. 5 Feurle GE, Volk B, Waldherr R. Cerebral Whipple's disease with negative jejunal histology. N Engi J Med 1979;300:907-8. 6 Ryser RJ, Locksley RM, Eng SC, Dobbins WO, Schoenknecth FD, Rubin CE. Reversal of dementia associated with Whipple's disease by trimethoprim-sulfamethoxazole, drugs that penetrate the blood brain barrier. Gastroenterology, 1984;861745-52. 7 Medical Research Council. Aids to the Examination of the Peripheral Nervous System. Memorandum No. 45, 1976. (HMSO, London). 8 Wang EEL, Prober CG. Ventricular cerebrospinal fluid concentrations of trimethoprimsulphamethoxazole. J Antimicrob Chemother 1983;11:385-9. 9 Romanul FCA, Radvany J, Rosales RK. Whipple's disease confined to the brain: a case studied clinically and pathologically. J Neurol Neurosurg Psychiatry 1977;40:901-9. 10 Finelli PF, McEntee WJ, Lessell S, Morgan TF, Copetto J. Whipple's disease with predominantly neuroophthalmic manifestations. Ann Neurol 1977;1:247-52. 11 Halperin JJ, Landis DMD, Kleinman GM. Whipple disease of the nervous system. Neurology 1982;32:612-7. 12 Knox DL, Bayless TM, Pittman FE. Neurologic disease in patients with treated Whipple's disease. Medicine 1976;55:467-76. 13 Maizel H, Ruffin JM, Dobbins WO. Whipple's disease: a review of 19 patients from one hospital and a review of the literature since 1950. Medicine 1970;49:175-205. 14 Schochet SS Jr, Lampert PW. Granulomatous encephalitis in Whipple's disease. Electron microscopic observations. Acta Neuropathol 1969;13:i-1 1. 15 Enzinger FM, Helwig EB. Whipple's disease: a review of the literature and report of fifteen patients. Virchows Arthiv (Pathol Anat) 1 963;336:238-69. 16 Keinath RD, Merrell DE, Vlietstra R, Dobbins WO. Antibiotic treatment and relapse in Whipple's disease: long-term follow-up of 88 patients. Gastroenterology 1985;88:1867-73. 17 Thompson DG, Ledingham JM, Howard AJ, Brown CL. Meningitis in Whipple's disease. Br Med J 1978;ii: 14-5. 18 Pollock S, Lewis PD, Kendall B. Whipple's disease confined to the nervous system. J Neurol Neurosurg Psychiatry 1981;44:1104-9. 19 Koudouris SD, Stern TN, Utterback RA. Involvement of central nervous system in Whipple's disease. Neurology 1963; 13: 397-404. 20 Masson R, Bouillat G, Clavell M. Une observation d'encephalopathie myoclonique au cours de la maladie de Whipple. Rev Neurol (Paris)

22 23

24

25

Pollock DJ. Myopathy in Whipple's disease. Gut 1977;18:800-4. Smith WT, French JM, Gottsman M, Smith AJ, Wakes-Miller JA. Cerebral complications of Whipple's disease. Brain 1965;88:137-50. Tauris P, Moesner J. Whipple's disease: Clinical and histopathological changes during treatment with sulphamethoxazole-trimethoprim. Acta Med Scand 1978;204:423-7. Bayless TM. Whipple's disease: newer concepts of therapy. Adv Intern Med 1970;16:171-89. Haeney MR, Ross IN. Whipple's disease in a female with impaired cell-mediated immunity unresponsive to co-trimoxazole and levamisole therapy. Posigrad Med J 1978; 54:45-50.

Accepted 29 August 1987

Asymptomatic cardiac arrhythmias in periodic paralysis

Sir: After the original report by Klein et all in 1963, several new cases of periodic paralysis associated with cardiac arrhythmias have been described27 (see table). In all of the reported cases, alterations ofcardiac rhythm were principally characterised by isolated and paired ventricular ectopic beats and biTJ COOPER directional ventricular tachycardia. This BIRD G serious and rare arrhythmia may be considB WHITE* ered a possible sign of periodic paralysis IT FERGUSON when found in a young patient.7 Department of Neurology, These cases of periodic paralysis with Bristol Southmead Hospital, associated arrhythmias have been regarded *Department of Neurology, as exclusively ventricular in origin with poor Royal United Hospital, Bath prognosis. We present what we believe is the first report of a case of periodic paralysis associated with a benign cardiac arrhythmia Address for correspondence: Dr IT Ferguson, MD, of atrial origin. FRCP(E), Consultant Neurologist, Southmead A 16 year old white male with no family Hospital, Southmead Rd., Westbury-on-Trym, history of neuromuscular disease suffered Bristol, BS10 5NB, UK. acute episodes of paralysis involving upper and lower limbs, lasting 2-4 days twice a year, since the age of 9 years. During hospitalisation in our Department because of one of these crises at the age of 13, laboratory data, including myoglobin, CK, aldosterone and thyroid function tests were all normal. References Serum and urinary potassium and other I Pallis CA, Lewis PD. Whipple's disease and the electrolytes were measured daily and were nervous system. In: The Neurology of Gasconsistently normal. A biopsy of the left trointestinal Disease. London: WB Saunders, quadriceps muscle showed no structural 1974:207-14. alteration and no glycogen accumulation 2 Sieracki JC. Fine G. Whipple's diseasewith PAS. Trichromic stain showed a slight observations on systemic involvement. (II) increase in interstitial connective tissue and 1976;132:415-8. Gross and histologic observations. Arch 21 Swash M, Schwartz MS. Vandenburg MJ, the electron microscopy showed a few fibres Pat/hol 1959;67:81-93.

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Letters

155

Table Periodic paralysis with arrhythmias: cases reported

Paralysis onset (years) Paralysis frequency (per month) Arrhythmias onset (years) Type of arrhythmias Serum K during paralysis KCI test-paralysis KCL test-arrhythmias Glucose test-paralysis Glucose test-arrhythmias

Klein1

Klein'

Andersen2

Lisak3

Levitt4

Gould7

et al

et al

et al

et al

Stubbs' et al

Yoshimura6

et al

et al

et al

1963

1963

1971

1972

1972

1976

1983

1985

7

11

7

18 months

7

28

13

1 8 VEB-BVT 5-37

1-4 14 BVT

2-3/year 2 BVT 3-1

No

1 10 BVT 384-8 Yes NSR

6-8

-

2 3 months VEB No -

I 6 VEB-VT 31-44 Yes NSR No NSR

5.1

Unchanged NSR No

-

No -

7 VEB-VT 2-9 -

Yes Unchanged -

No

-

Improved

-

4 -

10 BVT

Yes NSR No Worsened

Our case

9 2/year 13 SVEB 4-8 No Unchanged Yes

Unchanged

VEB, ventricular ectopic beats; BVT, bidirectional ventricular tachycardia; VT, ventricular tachycardia; SVEB, supraventricular ectopic beats; NSR, normal sinus rhythm.

with centralised nuclei. The ECG performed during the crisis showed a sinus rhythm with sporadic atrial ectopic beats with aberrant conduction. The dynamic ECG (Holter) after recovery of muscle strength showed a high incidence of atrial ectopic beats (mean = 105/h), many of which were conducted with varying degrees of aberrance. During the stress test, atrial ectopic beats were frequent up to 75 W, disappeared until the end of the test and were again frequent in the recovery phase. Bidimensional echocardiogram and radio-isotope cineventriculography with Tcssm revealed a slight enlargement of the right ventricle (diameter 30mm) with normal contractions. A load test with 6 grams of potassium chloride by mouth provoked no reduction in strength or modification in the dynamic ECG pattern. A load test with 100 grams of glucose by mouth and 10 IU insulin brought on paralysis of the lower limbs after 2 h; 2 grams of KCI were administered by mouth with full recovery of motor function within 2 h. A prolonged ECG performed during the test showed 570 (mean = 114/h) atrial ectopic beats of which the majority were aberrant. The patient was then discharged, being given acetazolamide 250mg twice a week and a low carbohydrate diet. During follow up (3 years) the patient experienced only one episode of paralysis of the legs which ceased 2 h after administration of KCI. The Holter ECG (3 recordings at intervals of 12 months for a total of 68 h) showed a high incidence of atrial ectopic beats (mean = 95/h), most of which were conducted with various degrees of aberrance, and numerous episodes of bigeminy with many paired beats. The peculiarity of this case lies in the arrhythmias observed in the numerous ECG and Holter recordings. Our patient had a

constantly high incidence of atrial arrhythmias often characterised by aberrant conduction. The atrial arrhythmias did not seem to be influenced by serum potassium concentrations, or by the presence or absence of neurological signs. Periodic paralysis associated with cardiac ventricular dysrhythmias may be modified by hypokalaemia or potassium load (see table). However, although arrhythmias accompanying an attack of muscle weakness have been known to occur, in all these cases arrhythmias were clearly independent of attacks of paralysis and plasma potassium levels. Thus the most widely accepted hypothesis to explain these rare cases of periodic paralysis associated with cardiac ventricular arrhythmias is that there is a structural or functional alteration in the cardiac muscle at cell membrane level, as there is in the skeletal muscle.4 8 9 Excitable muscle cells have proved to be constantly hypopolarised, and even a slight depolarisation at cardiac level could be sufficient to generate arrhythmias.811-l However, if we accept this hypothesis, it is not easy to explain the rare occurrence of cardiac arrhythmias associated with periodic paralysis. That our case is a familial cardiac supraventricular rhythm disturbance associated with periodic paralysis cannot be tested, since the parents and relatives showed no attacks of paralysis and constantly normal ECG. Since supraventricular arrhythmias are not uncommon in healthy young people, though not with such a high number of ectopic beats as found in our case,'2 we cannot exclude an incidental association. A more intensive cardiac survey of patients with periodic paralysis should be conducted to estimate the true incidence of asymptomatic arrhythmias in this population.

UBALDO BONUCCELLI ANGELO NUTI LUCA PAPERINI* MARIA GRAZIA BONGIORNI* ANTONINO ARRIGO CARLO CONTINIt ALBERTO MURATORIO

Institute of Clinical Neurology, University of Pisa, Clinical Physiology Institute CNR,* Pisa, Institute of Medical Pathology,t University ofPisa, Italy Address for correspondence: Dr Ubaldo Bonuccelli, Institute of Clinical Neurology, University of Pisa, via Roma, 67, 56100 Pisa, Italy. References I Klein R, Ganelin R, Marks JF, Usher P, Richards C. Periodic paralysis with cardiac arrhythmia. J Pediatr 1963;62:371-85. 2 Andersen ED, Krasilinikoff PA, Overvad H. Intermittent muscular weakness, extrasystoles and multiple developmental anomalies: a new syndrome? Acta Paediatr Scand 1971;60:559-64. 3 Lisak RP, Lebeau J, Tucker SH, Rowland LP. Hyperkalemic periodic paralysis and cardiac arrhythmia. Neurology 1972;22:810-5. 4 Levitt LP, Rose LI, Dawson DM. Hypokalemic Periodic Paralysis with Arrhythmia. N Engl J Med 1972;286:253-4. 5 Stubbs WA. Bidirectional ventricular tachycardia in familiar hypokalemic periodic paralysis. Proc R Soc Med 1976;69:223-4. 6 Yoshimura T, Kaneuji M, Okuno T, et al. Periodic paralysis with cardiac arrhythmia. Eur J Pediatr 1983;140:338-43. 7 Gould RJ, Steeg CN, Eastwood AB, Penn AS, Rowland LP, De Vivo DC. Potentially fatal cardiac dysrhythmia and hyperkalemic periodic paralysis. Neurology 1985;35:1208-12. 8 Creutzfeldt OD, Abbott BC, Fowler WM, Pearson CM. Muscle membrane potentials in episodic adynamia. Electroencephalogr Clin Neurophysiol 1963;15:508-19.

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156

Letters

9 McComas AJ, Mrozek K, Bradley WG. The nature of the electrophysiological disorder in adynamia episodica. J Neurol Neurosurg Psychiatry 1968;31:448-52. 10 Norris FH Jr. Micropipette recording from human striated muscle. J Neurol 1976;213:1-15. 11 Hofmann WW, Smith RA. Hypokalaemic periodic paralysis studied in vitro. Brain

1970;93:445-74. 12 Brodsky M, Wu D, Denes P, Kanakis C, Rosen KM. Arrhythmias documented by 24 hour continuous electrocardiographic monitoring in 50 male medical students without apparent heart disease. Am J Cardiol 1977;39:390-5. Accepted 27 July 1987

Kluver-Bucy syndrome associated with delayed post-anoxic leucoencephalopathy following carbon monoxide poisoning Sir: Despite intensive investigation, the clinico-pathological correlates of cerebral demyelination remain a neuropsychiatric enigma. Disorders of higher cortical function associated with primary or exclusive pathological change in the hemispheric white matter are not common. When they are observed, the involvement of myelin is often so extensive as to preclude specific anatomic-behavioural correlation. We report a patient with an unusual acute demyelinating process in which the neuropsychiatric features correlated with the neuropathological substrates. A 32 year old right-handed man with a prior diagnosis of bipolar affective disorder, attempted to commit suicide by car exhaust inhalation. After an unknown period of exposure, he was taken to a nearby hospital emergency room where he was found to be unresponsive to pain with decerebrate posturing. The patient was intubated and maintained on 100% oxygen while being transferred via helicopter to a hyperbaric treatment chamber. Upon arrival, his blood pressure was 148/100 mm Hg, pulse 125 per minute, and pupils were approximately 2 mm, equal, round and reactive to light. The patient improved rapidly following hyperbaric therapy. Approximately 6 hours following treatment, he was awake and able to follow commands. Within 24 hours, neurological examination was reported to be entirely normal. Four days following hyperbaric treatment, he drove about 320 km and sought care for his depression. Upon admission, the patient was found to be suffering a major depression with no evidence of neurologic deficit.

Eight days following hyperbaric treat- and (5) increased and altered sexual behav-

ment, he received one right unilateral ECT iour. The human Kluver-Bucy syndrome treatment. He received 0-2 mg glycopyrolate (KBS) closely parallels the syndrome seen in IM one hour prior to the procedure. He was monkeys, involving (1) visual agnosias

anaesthesised with 2 mg/kg sodium pentathol IV and 40mg succinyl choline IV without complications. Within several hours, the patient began exhibiting prominent verbal sexual innuendos, undressing and exposing himself, and lunging at the breasts of female staff and groins of male staff. These outbursts of sexually aggressive behaviour lasted 10 to 15 minutes and were interspersed with otherwise lethargic and apathetic behaviour. The patient ate excessively, constantly complained of hunger, showed excessive handling of objects, and was easily distracted. He displayed prominent amnesia, both retrograde and anterograde, and denied any memory of his inappropriate behaviour. Disorientation to time and severe attention deficit were present. Neurological examination revealed ataxia, moderate rigidity, and brisk muscle stretch reflexes. Electroencephalography demonstrated low-to-moderate voltage 4 to 6 Hz semirhythmic theta and paroxysmal high voltage rhythmic 5 Hz theta activity. These diffuse paroxysmal theta bursts were associated clinically with blank staring followed by agitation and poor compliance with commands. The patient was placed on phenytoin. Haloperidol and lorazepam were required to control the outbursts of aggressive hypersexual behaviour that continued to alternate with long periods of somnolence. The inappropriate behaviour resolved over a course of 10 days and neuroleptics and benzodiazepines were gradually withdrawn. Repeat EEG 10 days after the initial recording revealed minimal-to-moderate generalised 6 to 7 Hz theta without paroxysmal irregularities. Cranial CT scan was normal. Mental status examination progressively improved in all spheres. At discharge 4 weeks following admission, neuropsychological testing revealed only mild-to-moderate deficits in naming and recent memory. There was no evidence of inappropriate behaviour or neurologic deficits. As the encephalopathy cleared, the patient's primary affective disorder again became more apparent. In 1937, Kluver and Bucy described an unusual syndrome appearing in rhesus monkeys following bilateral temporal lobecThese monkeys displayed (1) tomy.'X "psychic blindness," (2) hyperorality, (3) an increased tendency to touch and examine objects ("hypermetamorphosis"'), (4) loss of normal anger, fear, and pleasure responses,

(especially prosopagnosia), (2) hyperphagiabulimia, (3) hypermetamorphosis, (4) placidity, apathy, and blunted affect, and (5) alterations in sexual behaviour.' In addition, the human KBS is accompanied by more complex behavioural manifestations, such as aphasia, amnesia, dementia, and/or seizures.' Partial or complete human

KBS has been described in association with variety of encephalopathic processes invariably associated with bilateral temporal lobe involvement. This paper is the first description of human KBS occurring in association with delayed carbon monoxide a

leucoencephalopathy. Neuropsychiatric sequelae of carbon monoxide poisoning occur in 15-40% of surviving patients.6 These sequelae frequently develop after a period of apparent recovery from the carbon monoxide exposure. The clinical picture of delayed

neuropsychiatric deterioration following carbon monoxide poisoning has been described in numerous case reports.7 These patients are usually deeply comatose following carbon monoxide inhalation but regain consciousness within 24 to 48 hours. Subsequent improvement is rapid, and they are often discharged from the hospital within I week. Choi found that a period of apparent recovery between the carbon monoxide insult and the onset of neuropsychiatric sequelae occurred in 11 8% of patients requiring hospital admission.8 The latent period varied from 2 to 40 days with a mean of 22-4 days. Recovery from the delayed sequelae occurred in 75% of patients within 1 year. Similar descriptions of delayed postanoxic encephalopathy have been reported following anaesthetic complications, cardiac arrest, hypotension, and anoxic anoxia.' Several authors have reported that the delayed onset of neurological deterioration occurred immediately following an increase in patient activity, after discharge from the hospital, after emotional stress, and in one case, following ECT.69 10 While a period of bed rest following carbon monoxide poisoning has been recommended as a means of preventing delayed post-anoxic deterioration, it has yet to be determined that this therapeutic measure is effective. The usual neuropathological substrate of delayed neuropsychiatric deterioration following carbon monoxide poisoning is believed to be selective injury to the cerebral white matter.7 ' Cases with a delayed onset

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Asymptomatic cardiac arrhythmias in periodic paralysis. U Bonuccelli, A Nuti, L Paperini, M G Bongiorni, A Arrigo, C Contini and A Muratorio J Neurol Neurosurg Psychiatry 1988 51: 154-156

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