Biomedical subjects
Publications and source records attributed to A Destée.
Bilateral cavernous sinus syndrome is usually due to a vascular disease, such as thrombophlebitis or arteriovenous malformation. In a 29-year old woman this syndrome revealed a malignant non-Hodgkin's lymphoma of the Burkitt type. In this case, the physiopathological mechanism was metastatic extension to the dura mater of a systemic lymphoma. The contribution of MRI to the diagnosis is emphasized.
Relations between linguistic deficits and cerebral blood flow (CBF) were studied in 20 cases of thalamic aphasia due to hemorrhage. Language analysis was based on BDAE, verbal intelligence quotient and verbal subtest of the memory quotient (Wechsler). CBF analysis (and of asymmetry index: AI) was done with 133 Xenon by SPECT technique in tomographic slices and in 15 areas of interest, i.e. cortical and deep areas. Relationships were analyzed by multiple correlations procedure and stepwise regression. Significant correlations were observed between linguistic results and AI of cortical but also deep areas (lenticular). Dynamic anomalies (fluency) were correlated with the IA and/or CBF of the frontal cortex. Verbal comprehension, naming and paraphasia were related to the AI of deep structures (insula and lenticular nucleus) and the AI of posterior cortex (temporo-occipital). Several correlations were found significant between results on verbal IQ of the WAIS and IA of the insula and lenticular nucleus.
Benign, monomelic juvenile amyotrophy of the hand (Hirayama type): new data.--Within the space of a few months a 21-year-old man developed isolated amyotrophy and paresis of the right hand; thirteen years later the clinical picture was unchanged. Medullo-cervical MRI was normal. The EMG confirmed the involvement of the anterior horn. Unilateral chronic distal juvenile amyotrophy located in an upper limb (Hirayama type) was diagnosed on clinical findings. But the spread of electrical abnormalities to the lower limbs suggested a transitional form between this syndrome and other disorders of the anterior horn.
Lesions of other structures than corpus Luysii, may rarely cause hemiballism. A case is reported of hemiballism due to infarction of the territory supplied by the lenticulo-striatal arteries. CT and MR imaging showed lesions of the caudate nucleus and putamen. This suggested that the cause of the hemiballism was the suppression of the regulatory activity of the caudate nucleus on the pallidum.
Long survival in subacute sclerosing panencephalitis (SSPE), including total disappearance of clinical signs, is rare. Two cases are reported. They concern a girl and a boy who, at age 13 and 15, developed SSPE and are still in remission 6 and 5 years later. After a typical onset and course over periods of 12 and 18 months, clinical improvement was observed and periodic EEG complexes disappeared. However, the electrophoretic oligoclonal pattern of CSF proteins and the elevated measles titers persisted (in one case specific CSF IgM were still increased 6 years after the onset). MRI showed asymmetrical areas of high-intensity signal in both white and gray matter, predominant in the temporal, parietal and occipital regions. The age at which SSPE begins and the interval between measles and SSPE onset are not prognostic factors. On the other hand, in reported cases with lasting remission SSPE did not progress beyond Jabbour's stage II. The second typical feature of these long-term improvements is disappearance of EEG periodic complexes and emergence of a normal basic background activity. No other prognostic factor has been reported.
A 31-year old man with chicken-pox encephalitis presented with a cerebellar syndrome and disorders of memory. The latter consisted of severe anterograde amnesia with normal retrograde memory, without confabulation or anosognosia. This suggested that the hippocampus was involved, probably functionally, since the outcome was rapidly favourable and the MRI was normal.
The natural course of untreated neuroleptic malignant syndrome (NMS) does not usually exceed 3 weeks. Its duration is reduced by dantrolene and/or bromocriptine. We report a patient in whom NMS developed after an injection of haloperidol decanoate and fluctuated during 3 months. This was due perhaps because the initial treatment with oral neuroleptic was pursued, or because the doses of dantrolene were insufficient.
On three occasions over a 21-month period, a woman with multiple sclerosis presented with hypothermia accompanied by altered consciousness, neurological signs and inappropriate antidiuretic hormone secretion. One of the episodes included hypoglycaemia. Although repeated MRI examinations, one of them with gadolinium injection, gave negative results, hypothalamic demyelination was suspected. The 4-year follow-up of this patient suggests that this lesion has no prognostic value.
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A 21 year-old man presented with a history of sudden onset of aphasia and headache. CT showed a left parietal hypodensity and pallidal calcifications. The ECG showed a Wolff-Parkinson-White's syndrome. The patient then developed successively focal epileptic seizures, temper disorders, a cardiomyopathy, a pepper and salt retinopathy with hemeralopia, a left hemiplegia, deafness, and fever of unexplained origin. Left carotid angiography showed thin, irregular or occluded branches of the middle and anterior cerebral arteries. Blood muscle enzymes, lactate and pyruvate, were elevated with acidosis. Muscle biopsy revealed a mitochondrial myopathy and blood chemistry showed a severe deficiency of respiratory chain enzymes. Death occurred after 28 months. This case showed the diagnostic features of Melas, with some elements of the Kearns-Sayre syndrome. To our knowledge, this is the first case were serial angiographies allowed demonstration of arterial changes capable of explaining cerebral infarctions.
A 40 year-old glass-blower suddenly experienced a painless weakness of his left shoulder and arm, more severe in C5 and C6 territories. The disorders regressed. Il was suspected that this brachial plexus neuropathy was of ischemic origin. However the absence of pain was surprising.
Cerebrospinal fluid (CSF) otorrhea is the usual presenting symptom of spontaneous tegmen tympani defects. A case associated with recurrent meningitis and CSF rhinorrhea without otorrhea is described. The coexistence of an hydrocephalus had led initially to ventriculoperitoneal shunting, which did not prevent meningitis recurrence. Previously reported in cases of anterior fossa and sella defects, an aqueduct stenosis was here associated with the tegmen tympani defect.
A 41-year-old woman presented with a history of sudden onset of a dementia with frontal signs and antero-retrograde amnesia. CT showed a bilateral paramedian thalamic infarct which was confirmed by MRI. The study of regional cerebral blood flow showed a decrease in both frontal regions. Results of imaging suggested lesions of the ventro-oral, latero-polar and intralaminar nuclei as well as of the mammillo-thalamic tract. The dorso-median nucleus appeared to be relatively spared.
A 43 year old woman with a strong likelihood of familial history, developed progressively a spastic tetraparesis associated with intellectual deterioration and terminal epileptic fits. She died 11 years after onset of the clinical disorders. Neuropathological study revealed an orthochromatic leukodystrophy. Macrophages and glial cells of the white matter contained a brown-yellow, autofluorescent pigment which stained positively with PAS, Perls stain for iron and Masson-Fontana. Electron microscopy showed electron dense, membrane bound intracytoplasmic lamellar inclusions with curved or straight parallel arrangement or fingerprint pattern, in white matter macrophages, astrocytes and oligodendrocytes. Cortical cells contained lipofuscin which was normal in type and amount. This suggests that the material in white matter glial cells and macrophages is ceroid pigment; however, the distribution is not that seen in ceroid-lipofuscinosis. Similar inclusions have been found in oligodentrocytes in other forms of orthochromatic leucodystrophy. Ten similar cases of pigmentary type of orthochromatic leucodystrophy have been reported previously; only one had had an ultrastructural study.
Three patients developed chorea while receiving oral contraceptives. Two were young patients whose chorea developed long after treatment had been started and disappeared soon after it had been discontinued. The third patient had acute amphetamine-induced chorea after prolonged oral contraception. Prolonged administration of female sex hormones is a possible cause of chorea in women who have not previously had chorea or rheumatic fever.
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Two cases of intraspinal meningeal hemangiopericytomas are reported, one of which cervical and the other one dorsal with relapse. The anatomopathological features of the hemangiopericytomas are recorded and the nosological problems of their meningeal localizations are discussed. As in the case of intracranial meningeal hemangiopericytomas, the clinical, radiological and macroscopic features of intraspinal hemangiopericytomas--to the exception of a few particular cases--resemble those of other localizations of these tumours more than those of meningiomas, in the light of the review of twenty-eight cases to be found in the relevant literature. Treatment of such tumours hasn't as yet been well circumscribed. Surgical treatment may be facilitated by preoperative embolization. Radiotherapy must be associated to it, at least when removal is incomplete, because of frequent recurrence.