Spontaneous periodic hypothermia with lipoma of the corpus callosum

Abstract

AManchester SUMMARY A patient with spontaneous periodic hypothermia who had both a lipoma and agenesis of the corpus callosum is described. Spontaneous periodic hypothermia associated with corpus callosum abnormalities is a distinct entity and although the mechanism underlying the hypothermic episodes is unexplained, the term "diencephalic autonomic epilepsy" does not seem appropriate. Profuse sweating for several days had been noted during one of them.When two years old he had sustained a fractured skull in a road traffic accident. His physical development was normal but he was mentally slow and had attended a special school. He had worked in a furniture factory for nearly 20 years, eventually being forced to retire by ill health. For at least 6 years he had major seizures treated with Phenytoin 100 mg twice daily. On examination he was thin with a normal male distribution of body hair. He was alert and orientated but below average intelligence (WAIS IQ= 75). He had mild dysarthria with exaggerated reflexes in the left arm and bilateral extensor plantar responses. After three weeks in hospital, during which he was well and ambulant with an oral temperature between 35 0°C and 36 5°C, blood pressure 120/80 mmHg and pulse rate 60 per minute, he suddenly developed profuse sweating accompanied by cutaneous vasodilatation which affected the whole body surface. He complained of feeling hot and after 2 hours continuous sweating his oral temperature was 31 0°C. Any rise in body temperature provoked further attacks of sweating lasting 30 minutes to several hours and with each attack the rectal temperature fell, often as low as 29 5°C (fig 1). Two days after the onset of sweating the patient became lethargic and withdrawn. His dysarthria increased, he had marked truncal ataxia and flapping tremor of the outstretched hands. After 4 days he was unresponsive and incontinent. Systolic blood pressure varied between 75-100 mmHg with marked bradycardia, sometimes as slow as 40 per minute. The ECG showed typical J-waves. Five days later his body temperature returned abruptly to his normal level. His physical and mental state recovered after two days. Seven days later he had a similar sudden episode of sweating and hypothermia which lasted 8 days. His hospital course was marked by alternating periods of relative normality and sweating with hypothermia. Shivering was never seen. Several major and left sided focal motor attacks occurred during

Symptoms and signs of progressive hydrocephalus.

“…They are older than the first subgroup, and their signs are even more varied with no obvious signs on clinical examination in a quarter. (17) 16 (15) 15 (14) 14 (13) 13 (12) 11 (10) 9 (8) 8 (7) 8 (7) Other less frequent symptoms were: jitteriness, sleep disturbance, sluggish valve, fits, abdominal pain, deteriorating school performance, pallor, and failure to thrive. Other less frequent signs were: tonic fits, pallor, sluggish valve, decerebration, optic atrophy, nystagmus, bradycardia, hypertension, profuse sweating, neurogenic stridor, macular rash, return of tonic neck reflex, hemiparesis, ataxia, sudden blindness, Cheyne-Stokes respiration, apnoeic or cyanotic episodes, cardiorespiratory arrest, ptosis, VII and XII cranial nerve palsies, and vasomotor changes.…”

Hypothermia in Hodgkin’s disease: unexpected state; case report and rewiew of the literature

“…Primary and metastatic brain tumors may cause hypothermia. [4,[12][13][14][15] Autonomic neuropathy was suggested to define the role of the autonomic nervous system by the authors. [5,9] Another view is that the different drugs are associated with hypothermia.…”

“…In literature, this clinical manifestation has been reported from several case reports. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Herein we presented a case of stage IIIB Hodgkin's disease, complicating with hypothermia and hypotension after naproxen sodium administration.…”

Episodic hyperhydrosis with corpus callosum agenesis: A rare case of Shapiro syndrome

“…[1] Proposed hypotheses to explain the clinical features of this syndrome include changes in the set point of the hypothalamic thermostat, [2] increased norepinephrine (NE) release and decreased plasma NE clearance. [1] Proposed hypotheses to explain the clinical features of this syndrome include changes in the set point of the hypothalamic thermostat, [2] increased norepinephrine (NE) release and decreased plasma NE clearance.…”