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BACKGROUND: Newborn infants are particularly prone to hypothermia, a condition with a high mortality. OBJECTIVE: To study the CT brain patterns in infants with hypothermia and neurological symptoms. MATERIALS AND METHODS: We reviewed the brain CT of nine infants with neonatal hypothermia, multiple organ failure, seizures and coma. RESULTS: Two infants had normal CT scans, acutely and at follow-up, and were clinically normal at follow-up. In seven infants, CT showed diffuse cerebral oedema, with reversal of the normal density relationship between grey and white matter and a relative increased density of the thalami, brainstem and cerebellum - the 'reversal sign'. In six surviving infants with severe developmental delay, follow-up CT revealed cerebral atrophy with multicystic encephalomalacia. CONCLUSIONS: The 'reversal sign' has been described in the abused child, birth asphyxia and anoxia due to drowning. Neonatal hypothermia is offered as a further cause.

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We describe MRI findings in 13 persons with typical clinical, EEG, CT and biochemical features of cerebrotendinous xanthomatosis (CTX). MRI showed brain (13/13) and cerebellar (12/13) atrophy and diffuse white matter hypodensity (4/13) presumably reflecting sterol infiltration with demyelination. Focal lesions were rare (2/13). Mass effect, edema or enhancement were not observed. Treatment with chenodeoxycholic acid (CDCA) 750 mg/day orally improved neurological and biochemical abnormalities. MRI appears to be of little value in following improvement after treatment has begun. Otherwise, the MRI studies are very sensitive and useful in diagnosing early incomplete forms of CTX.

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Significant osteoporosis determined by skeleton radiography and bone densitometry was found in 15 patients with cerebrotendinous xanthomatosis (CTX) whose mean age was 31 +/- 11 years. In three CTX patients, bone biopsies confirmed osteoporosis. Nine patients also sustained bone fractures following minimal trauma. Serum 25-hydroxyvitamin D ([25-OHD] 14.6 +/- 6.6 ng/mL v [normal] 30.4 +/- 8.0 ng/mL; P < .001) and 24,25-dihydroxyvitamin D ([24,25(OH)2D] 1.2 +/- 0.4 ng/mL v [normal] 2.7 +/- 0.8 ng/mL; P < .001) levels were low. Serum concentrations of 1,25(OH)2D, calcium, inorganic phosphorus, alkaline phosphatase, parathyroid hormone, and calcitonin were normal. Patients showed classic manifestations of CTX, including dementia, pyramidal and cerebellar insufficiency, peripheral neuropathy, cataracts, and tendon xanthomas associated with elevated serum cholestanol concentrations. These results demonstrate that extensive osteoporosis and increased risk of bone fractures are components of this inherited disease.

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Ocular bobbing, dipping and "reversed" ocular dipping were observed and recorded in two patients presenting a severe neurological symptomatology: quadruplegia and coma in one and locked-in syndrome in the other. CT scan showed a pontine infarction in both these patients. This is the first time that the ocular dipping and "reversed" ocular dipping are related to such an anatomic structure.

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Tendinous xanthomata with maximum expression on Achilles tendons are a characteristic feature of cerebrotendinous xanthomatosis, a rare autosomal recessive lipid storage disease. Twenty patients are under review; three of them, at different stages of the disease, underwent computed tomographic (CT) examination. CT demonstrates with a high degree of accuracy the increased size of the tendon and its heterogeneous structure resulting from cholesterol and cholestanol crystal deposits.

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Angiomatous malformations of the central nervous system are relatively rare and have a serious prognosis. Clinical manifestations of such lesions of the spine include back pain associated with motor and sensory weakness, progressing to complete paraplegia. In angiomatous involvement of the brain, the symptoms may progress from headaches to coma and death. Computerized tomography and magnetic resonance imaging are new noninvasive modalities used in the diagnosis of such malformation, but they have not replaced myelography and angiography. Up to the past decade, the management of angiomatous lesions of the CNS was only surgical. Angiographic embolization is now widely accepted as an alternate method of management. Experience with embolic treatment of 3 cases is reported. In a 15-year-old boy with two adjacent thoracic vertebral hemangiomas (D7 and D8) compressing the spinal cord, embolization was used before surgery. A 23-year-old man with acute myelopathy due to extraretro-medullary arteriovenous malformations of the spinal cord (D9-D11) was successfully treated by selective embolization via catheter of the right 10th intercostal feeding vessel. An 82-year-old man had proptosis, bruit and pulsation in the right eyeball and loss of vision. Arteriovenous malformation of the dura of the anterior cranial fossa was demonstrated and was completely cured by bilateral selective embolization of the feeding branches of the internal maxillary arteries.

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A 32-year-old man had generalized tonic-clonic epileptic seizures associated with episodes of recurrent high fever for 6 years. Repeated physical examinations including neurological status, EEG and nuclear brain scan were negative. Brain CT showed a non-homogeneous parasellar cyst of low density and fat-fluid levels in the lateral ventricles. The diagnosis of intracranial dermoid cyst was confirmed at surgery and histopathologically. Recurrent febrile convulsions and chemical meningitis may be the only clinical manifestations of ruptured dermoid cyst. The CT features of intracranial dermoid cyst are pathognomonic.

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Linear brain parameters were measured by CT in 19 patients with morbid obesity (mean weight 126.4 +/- 20.5 kg) and 20 age and sex matched normal weight subjects (mean weight 62.6 +/- 14.9 kg). Ventricular parameters were slightly smaller and cortical parameters were slightly larger in the preoperative obese than in control subjects. However, only the four cortical sulci ratio was significantly different in the two groups (P = 0.02). After gastric restriction surgery and drastic weight loss (mean postoperative weight 82.9 +/- 27.4 kg), all the ventricular and cortical parameters increased, with significant change in the frontal interhemispheric fissure ratio (P less than 0.05). Obese patients followed for 23 months after surgery had less striking changes than those followed for 6 months. Morbidly obese subjects have altered brain CT dimensions which are partly reversible after weight correction.

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In nine patients with cerebrotendinous xanthomatosis (CTX), computed tomography (CT) demonstrated diffuse white matter hypodensity above and below the tentorium. This was attributed to sterol infiltration with secondary demyelination. In one patient, a focal right cerebellar hypodense lesion reflected a true xanthoma. These findings suggest that the neurologic symptoms, no matter how longstanding, result from metabolic encephalopathy rather than irreversible destruction of brain tissue by xanthomas.

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