Deep cerebral venous thrombosis: an illustrative case with reversible diencephalic dysfunction.

BACKGROUND: Isolated thrombosis of the deep cerebral veins is rare and its diagnosis can be difficult. Mortality is often high and little is known about the long-term prognosis. CASE REPORT: We report a 24-year-old woman with akinetic mutism and extensive bilateral thalamic lesions. CT and MRI allowed early diagnosis by demonstrating thrombosis within the internal cerebral veins, without the need for angiography. Heparin treatment was used safely despite the presence of thalamic and intraventricular hemorrhage. After five weeks, the patient recovered rapidly and remains well at 18 months. Serial MRI showed dramatic resolution of the imaging abnormalities. CONCLUSIONS: The clinical features and characteristic neuroimaging appearance of deep cerebral venous thrombosis should be recognized by physicians caring for stroke patients. Deep cerebral venous thrombosis can produce extensive venous congestion and vasogenic edema without early infarction. Excellent clinical recovery is possible even after severe and prolonged neurological deficits.

Experience from a multidisciplinary "dizzy" clinic.

OBJECTIVE: To describe the experience of a combined otolaryngology and neurology multidisciplinary clinic in the evaluation, investigation, and management of patients with dizziness. STUDY DESIGN: Prospective clinical study. SETTING: Patients were seen in a tertiary referral, multidisciplinary clinic at The Toronto Hospital, University of Toronto, Ontario, Canada. INTERVENTION: A thorough history, formal otoneurologic examination, and appropriate laboratory investigations were performed. After their assessment, the patient's diagnoses were classified as peripheral, central, psychogenic, or undiagnosed and were then subdivided into specific clinical diagnoses. RESULTS: The first 812 consecutive patients seen in the multidisciplinary clinic from January 1, 1993 to December 31, 1998 are reported. Five hundred twenty-five (64.7%) patients were found to have a peripheral vestibular cause for their dizziness, 66 (8.1%) had a central cause, 108 (13.3%) had a diagnosis unknown, and 73 (9.0%) were thought to be psychogenic. In 40 (4.9%) patients, a peripheral and central cause were found. More than one type of peripheral disorder was noted in 17.9% of patients with a peripheral vestibular cause for their dizziness, and 12.3% of patients with a central cause for their dizziness had more than one specific type of central nervous system disorder. CONCLUSIONS: Most patients that were seen in a multidisciplinary clinic had a peripheral vestibular disorder. Central causes of dizziness were relatively uncommon. Serious diseases such as tumor, multiple sclerosis, and encephalitis were rare and unlikely to present with dizziness only. It is important to realize that a patient may have more than one type of disorder accounting for the symptoms, which may represent a spectrum of disease affecting the inner ear.

Evaluation of the dizzy patient: experience from a multidisciplinary neurotology clinic.

In 1993 a multidisciplinary neurotology clinic was established at the Toronto Hospital, University of Toronto, where patients with symptoms of dizziness were assessed by both otolaryngologists and neurologists. The results from the first 400 patients seen in consultation are described. The disease pathologies identified in this patient population with dizziness showed some significant differences from other published series, which we believe reflects the specialized tertiary nature of referrals to this clinic. A model for the collaborative investigation of the dizzy patient is provided consistent with the current trend towards multidisciplinary approaches in medicine.

Cerebral and cerebellar gangliocytomas: a morphological study of nine cases.

Hypothalamic gangliocytomas have been shown to contain immunoreactivity for hypophysiotropic peptides and some have been associated with endocrine dysfunction. Extrahypothalamic gangliocytomas are usually not associated with endocrine abnormalities. We studied nine cerebral or cerebellar gangliocytomas from six men and three women; none of the patients had detectable alterations of endocrine homeostasis. On histological examination, the tumor cells resembled hypothalamic neurons. Electron microscopy disclosed the presence of dense-core vesicles in neuronal cytoplasm and processes resembling Herring bodies, and there were synaptic contacts between tumor cells. All but two tumors contained immunocytochemical positivity for at least one peptide hormone or amine; these included somatostatin, corticotropin-releasing hormone, beta-endorphin, galanin, vasoactive intestinal peptide, calcitonin, serotonin, catecholamines or met-enkephalin. These tumors have been thought to represent neoplasms arising in ectopic autonomic neural tissue. Their morphological features, their similarity to hypothalamic gangliocytomas and the multiple immunoreactivities shown here suggest that they can be regarded as tumors of peptidergic neurons that are widely distributed throughout the central nervous system.

Regional cerebral glucose metabolism in SLE chorea: further evidence that striatal hypometabolism is not a correlate of chorea.

The pathophysiology of chorea in systemic lupus erythematosus (SLE) is uncertain. Pathologic examination has not identified a specific location for the causative lesion(s) and immunologic mechanisms have been suggested in its etiology. In other choreic disorders, such as Huntington's disease and benign hereditary chorea, glucose hypometabolism in the striatum has been demonstrated by positron computed tomography (PCT) using [18F]deoxyglucose. With this technique we have studied four patients with chorea secondary to SLE. In these patients the regional distribution of cerebral glucose metabolism was normal. In particular, striatal glucose metabolism was within the normal range, even though the ratio of striatal to cortical glucose metabolism was increased. Our results show that striatal hypometabolism, as seen in other disorders manifesting chorea, is not the PCT correlate of the dyskinesia.

Hemangioma calcificans of the spinal cord.

A 27-year-old woman presented with hemotomyelia during pregnancy. Needle drainage of the spinal cord was followed by relief of symptoms. Symptoms recurred during a second pregnancy 4 years later and she presented with an avascular mass in the midcervical spinal cord. This lesion proved to be hemangioma calcificans, a densely calcified and ossified variant of cavernous angioma.

Pilar tumor of the scalp.

An elderly woman presented with a large, fungating mass over the occipital portion of the scalp. Biopsy revealed this to be a pilar tumor of the scalp. The tumor was excised with a margin of normal tissue and the defect was closed with advancement of scalp and neck skin. Follow-up at 6 months revealed no recurrence of the lesion.