Scot Moo J 1992; 37: 85-86
0036/9330/92/13491/085 $2.00 In USA 1992 Scottish Medical Journal
A CASE OF SPONTANEOUS HYPOTHERMIA
K.M. Ferguson. T Fyfe. *J Montgomery Department of Medicine, Southern General Hospital, Glasgow. *Govan Health Centre, Glasgow.
Abstract: It is important to recognise any condition. however rare, whichcan be cured. We report a case of chronic hypothermia and its dramatic response to treatment. Case report A 69 year old retired nurse presented with a year's history of intermittent episodes of unwellness associated with sweating, followed by rigors. These attacks usually occurred at night or early morning lasting up to four hours three times per day. She had had rheumatic fever SOyears ago and was hypertensive, controlled on atenolol and bendrofluazide. Systemic enquiry was unhelpful. On clinical examination she was obese with features suggesting acromegaly. She was in controlled atrial fibrillation with mitral stenosis. There was no clinical evidence of any infective process, including bacterial endocarditis. Neurological examination was normal. It was noted that her basal temperature on the ward was 34-3SoC but during attacks ofsweating her temperature fell, subsequently rising again during the shivering phase. (Fig1) The basal core temperature remained stable during immersion in cold water at 1SoC for 30 minutes and on raising the ambient temperature to 39°C for 30 minutes. Infection was considered as a cause for these attacks. Extensive investigations, including repeated blood cultures, mid-stream specimens of urine and chest X-ray failed to yield any focus of infection. The white cell count and ESR were normal. Cardiac ultrasound showed mitral stenosis with no vegetations. Endocrine investigations, including basal pituitary hormones, combined anterior pituitary function tests, glucose tolerance tests with growth hormone assays and dexamethasone suppression tests were all normal. EEGs were normal on two occasions. Full autonomic function testing showed intact parasympathetic and sympathetic systems. Cranial CT scanning demonstrated a normal pituitary fossa and hypothalamic area, the only abnormality being agenesis of the corpus callosum, confirmed by MRI scanning. (Fig 2) A trial of bromocriptine (2Omgs daily) followed by a trial of phenytoin produced no benefit. Treatment with chlorpromazine (initially 2S mgs. q.i.d., increased to SO mgs t.i.d.) at once raised the patient's baseline temperature to 37"C and abolished the attacks of hypothermia with sweating and subsequent shivering. On this treatment she has had no further attacks in the last four years.
Discussion This patient's basal temperature was subnormal and attacks of profuse sweating led to lowering of the temperature. Thereafter shivering produced a return to the basal subnormal level. Shapiro first described this clinical triad in 1969 1 hypothermia, sweating and chills with agenesis of the corpus callosum. Since then twelve cases have been reported in the literature2•3 with a male:female ratio of2: 1 and ages ranging from nine months to 34 years. All were found to have EEG abnormalities and four had epileptic seizures. Interestingly this patient presented when much older and had normal EEGs and no seizures. The key to diagnosis is careful detailing of the history of sweating followed by shivering, low basal temperature and the temperature fall during an attack. Agenesis of the corpus callosum, which is found in approximately 1 in 1000 cranial CT scans, should lead to the diagnosis. The pathogenesis of recurrent spontaneous hypothermia and its relation to agenesis of the corpus callosum is not known. Diencephalic epilepsy, a change in the set point of the "thermostat", or a localised lesion in the hypothalamus have been postulated? In this patient a change in the set point of the "thermostat" seems most likely." The normal EEG and lack of response to phenytoin make diencephalic epilepsy unlikely. The CT scan and normal endocrine tests ruled out a localised hypothalamic lesion or hypothalamic-pituitary dysfunction. This raises the question - why should the set point of the "thermostat" change in a 69 year old with congenital agenesis of the corpus callosum?
36
35 Temperature' 34 33
I=::::~l
32
3\ 30
29
08.00
16.00
00.00
08.00
Time in hours
Fig 1. 24 hour temperaturemeasurements
Correspondence to: Dr K M Ferguson, Department of Medicine, Southern General Hospital, Glasgow.
Fig 2. Cranialmagneticresonance imagingscan showing complete absenceof the COlpUS callosum.
85
Spontaneous hypothermia
The role of the corpus callosum in temperature regulation is uncertain. Temperature regulation is a task for the hypothalamus which is represented in both cerebral hemispheres.i Communication between the two hypothalamic areas occurs through the corpus callosum. Temperature on both sides of the body is obviously equal, suggesting that the corpus callosum plays a co-ordinating role. However, surgical section of the adult corpus callosum does not lead to any recognised anomaly of temperature regulation'' and not all people with agenesis of the corpus callosum have abnormal temperature control. The importance of this syndrome is that it can be cured by chlorpromazine therapy and the patient's life transformed, although the mechanism is uncertain. It may in fact be commoner
86
Ferguson, Fyfe and Montgomery
than is recognised, especially in older people, and is therefore worth considering in the differential diagnosis of attacks of sweating and shivering. REFERENCES 1 Shapiro WR, Williams GH, Plum F. Brain 1969; 92: 436. 2 Mooridan AD, Morley GK, McGeachie R, Lundgren S. Morley IE. Neurology 1984; 34: 82. 3 Le Witt PA, Newman RP, Greenberg HS, Rocher LL, Calne DB, Ehrenkranz 1RL Neurology 1983; 33: 1129. 4 Summers GD, Young AC, Little RA, Stoner HB, Forbes WSTC, Jones RAC. Jrnl Neurol, Neurosurg & Psych 1981; 44: 1099. 5 Editorial Lancet 1973; 2: 246. 6 Noel P, HubertJP, Ectors M, Franken L, Flament-Durand J. Brain 1973; 26: 368.
0036/9330/92/13491/085 $2.00 In USA 1992 Scottish Medical Journal
A CASE OF SPONTANEOUS HYPOTHERMIA
K.M. Ferguson. T Fyfe. *J Montgomery Department of Medicine, Southern General Hospital, Glasgow. *Govan Health Centre, Glasgow.
Abstract: It is important to recognise any condition. however rare, whichcan be cured. We report a case of chronic hypothermia and its dramatic response to treatment. Case report A 69 year old retired nurse presented with a year's history of intermittent episodes of unwellness associated with sweating, followed by rigors. These attacks usually occurred at night or early morning lasting up to four hours three times per day. She had had rheumatic fever SOyears ago and was hypertensive, controlled on atenolol and bendrofluazide. Systemic enquiry was unhelpful. On clinical examination she was obese with features suggesting acromegaly. She was in controlled atrial fibrillation with mitral stenosis. There was no clinical evidence of any infective process, including bacterial endocarditis. Neurological examination was normal. It was noted that her basal temperature on the ward was 34-3SoC but during attacks ofsweating her temperature fell, subsequently rising again during the shivering phase. (Fig1) The basal core temperature remained stable during immersion in cold water at 1SoC for 30 minutes and on raising the ambient temperature to 39°C for 30 minutes. Infection was considered as a cause for these attacks. Extensive investigations, including repeated blood cultures, mid-stream specimens of urine and chest X-ray failed to yield any focus of infection. The white cell count and ESR were normal. Cardiac ultrasound showed mitral stenosis with no vegetations. Endocrine investigations, including basal pituitary hormones, combined anterior pituitary function tests, glucose tolerance tests with growth hormone assays and dexamethasone suppression tests were all normal. EEGs were normal on two occasions. Full autonomic function testing showed intact parasympathetic and sympathetic systems. Cranial CT scanning demonstrated a normal pituitary fossa and hypothalamic area, the only abnormality being agenesis of the corpus callosum, confirmed by MRI scanning. (Fig 2) A trial of bromocriptine (2Omgs daily) followed by a trial of phenytoin produced no benefit. Treatment with chlorpromazine (initially 2S mgs. q.i.d., increased to SO mgs t.i.d.) at once raised the patient's baseline temperature to 37"C and abolished the attacks of hypothermia with sweating and subsequent shivering. On this treatment she has had no further attacks in the last four years.
Discussion This patient's basal temperature was subnormal and attacks of profuse sweating led to lowering of the temperature. Thereafter shivering produced a return to the basal subnormal level. Shapiro first described this clinical triad in 1969 1 hypothermia, sweating and chills with agenesis of the corpus callosum. Since then twelve cases have been reported in the literature2•3 with a male:female ratio of2: 1 and ages ranging from nine months to 34 years. All were found to have EEG abnormalities and four had epileptic seizures. Interestingly this patient presented when much older and had normal EEGs and no seizures. The key to diagnosis is careful detailing of the history of sweating followed by shivering, low basal temperature and the temperature fall during an attack. Agenesis of the corpus callosum, which is found in approximately 1 in 1000 cranial CT scans, should lead to the diagnosis. The pathogenesis of recurrent spontaneous hypothermia and its relation to agenesis of the corpus callosum is not known. Diencephalic epilepsy, a change in the set point of the "thermostat", or a localised lesion in the hypothalamus have been postulated? In this patient a change in the set point of the "thermostat" seems most likely." The normal EEG and lack of response to phenytoin make diencephalic epilepsy unlikely. The CT scan and normal endocrine tests ruled out a localised hypothalamic lesion or hypothalamic-pituitary dysfunction. This raises the question - why should the set point of the "thermostat" change in a 69 year old with congenital agenesis of the corpus callosum?
36
35 Temperature' 34 33
I=::::~l
32
3\ 30
29
08.00
16.00
00.00
08.00
Time in hours
Fig 1. 24 hour temperaturemeasurements
Correspondence to: Dr K M Ferguson, Department of Medicine, Southern General Hospital, Glasgow.
Fig 2. Cranialmagneticresonance imagingscan showing complete absenceof the COlpUS callosum.
85
Spontaneous hypothermia
The role of the corpus callosum in temperature regulation is uncertain. Temperature regulation is a task for the hypothalamus which is represented in both cerebral hemispheres.i Communication between the two hypothalamic areas occurs through the corpus callosum. Temperature on both sides of the body is obviously equal, suggesting that the corpus callosum plays a co-ordinating role. However, surgical section of the adult corpus callosum does not lead to any recognised anomaly of temperature regulation'' and not all people with agenesis of the corpus callosum have abnormal temperature control. The importance of this syndrome is that it can be cured by chlorpromazine therapy and the patient's life transformed, although the mechanism is uncertain. It may in fact be commoner
86
Ferguson, Fyfe and Montgomery
than is recognised, especially in older people, and is therefore worth considering in the differential diagnosis of attacks of sweating and shivering. REFERENCES 1 Shapiro WR, Williams GH, Plum F. Brain 1969; 92: 436. 2 Mooridan AD, Morley GK, McGeachie R, Lundgren S. Morley IE. Neurology 1984; 34: 82. 3 Le Witt PA, Newman RP, Greenberg HS, Rocher LL, Calne DB, Ehrenkranz 1RL Neurology 1983; 33: 1129. 4 Summers GD, Young AC, Little RA, Stoner HB, Forbes WSTC, Jones RAC. Jrnl Neurol, Neurosurg & Psych 1981; 44: 1099. 5 Editorial Lancet 1973; 2: 246. 6 Noel P, HubertJP, Ectors M, Franken L, Flament-Durand J. Brain 1973; 26: 368.
