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Biomedical subjects

A Destee

Publications and source records attributed to A Destee.

At least 19 recordsLinked to original sources

[Internal carotid occlusion and essential thrombocythemia. 2 cases].

We report 2 cases of occlusion of the internal carotid artery in young, non-atherosclerotic patients with essential thrombocythemia (ET). This complication of ET seems to be rare, but is probably underdiagnosed since transient ischemia, which is frequent in ET patients, is seldom explored by angiography. The excess of thrombocytes and the thrombopathy may account for the microvascular complications, but in the absence of any other contributory factor, occlusions of large arteries, such as the ICA, is more difficult to explain.

Adult

[Obsessive-compulsive behavior and progressive supranuclear palsy].

A case of progressive supranuclear palsy characterized by a loss of self-activation and a compulsive behaviour of the obsessive type is reported. The pathological examination was remarkable for the intensity of pallidal lesions and their diffusion to both the external and internal segments. While the loss of self-activation seemed to result from a damaged cortico-subcortical circuit forming a limbic loop, the compulsive behaviour of the obsessive type may have resulted from the interruption of a frontal-caudal-pallidal-luysian circuit.

Globus Pallidus

[Secondary and late development of hematoma of the brain stem].

The functional and social outcome in 15 patients with brainstem hematomas surviving on the 15th day is reported. At the end of the first year post stroke, the main neurological deficits were sensory, postural and paresis of the 6th and 7th cranial nerves. This resulted from the preferential location of the lesions in the lateral and posterior pons. On a functional point of view, 8 patients were independent in daily living. Seven of them were regularly engaged in more elaborated activities, at home and outside: 1 had recovered his previous professional activities. Those functions evolved more slowly during the 2nd year and later on. A significant correlation was found between the initial neurological status and the functional outcome at 1 year, and also between the volume of the hematoma on C.T. scan and this outcome. In this series, the age of the patients was not correlated to the other variables.

Activities of Daily Living

[Malignant intraocular non-Hodgkin's lymphoma].

The authors presented two personal cases of ocular involvement by non hodgkin's malignant lymphomas. The first one concerned a 55 year-old man; initial symptoms were oedematous optic neuropathy associated to vitritis, uveitis and chorio-retinal infiltrates. The ocular symptoms did not respond to cortico-steroid therapy and lymphoma was diagnosed eight months later by histologic examination of a cerebral tumor discovered by C.T. scan. Systemic examination was normal. In spite of a cerebral radiotherapy associated to chemotherapy, uveitis and vitritis occurred in the other eye and vitreous biopsy of the most affected eye showed no cell abnormalities. The patient died two years after the onset of ocular symptoms. The second case concerned a 67 year-old woman who presented an association of absolute glaucoma secondary to uveitis with retinal detachment and neurological symptoms due to a cerebral tumor detected by C.T. Scan. Histological examination of the enucleated eye confirmed the diagnosis of centroblastic lymphoma: choroid, retina and optic nerve were involved. A data review consisted of a study of 93 cases with the same pathological conditions and permitted a discussion about the main clinical features of this affection and the different histological aspects. The following classification of the different forms of ocular lymphomas was proposed: isolated associated to cerebral and/or to systemic localizations. The management of a treatment by radiotherapy was discussed, based on the previous reports, and a prognosis of oculo-cerebral lymphomas was given.

Aged

[Borrelian meningoencephalomyelitis. A case].

A 22-year old man had spastic paraparesis and cerebellar syndrome of 5 months duration. CSF showed lymphocytosis, elevated protein content, hypoglycorachia, hypochlorurachia and oligoclonal banding. CT scan and MRI were normal. Extensive laboratory procedures disclosed no bacterial, viral, fungic, parasitic or inflammatory disease. Anti-Borrelia burgdorferi antibodies were present in blood (1/4000) and in CSF (1/1024). With antibiotics (penicillin G 20 millions units per day for 10 days, followed by latamoxef 1.5 g per day for 3 months) and prednisone (50 mg per day for 2 months), the cerebellar signs disappeared, the paraparesis improved and the CSF abnormalities disappeared; blood and CSF anti-Borrelia antibody levels decreased. This case is an example of a severe form of CNS impairment by Borrelia burgdorferi. In Europe, most reports show progressive para- or tetraparesis with, sometimes, intellectual, cerebellar or cranial nerve impairment. In some cases, the signs are less diffuse or more acute. Lymphocytic meningitis is present with hyperalbuminorachia and oligoclonal banding; hypoglycorachia is mentioned in only one other report. Diagnosis is made by high blood and CSF antibody titers and demonstration of local synthesis. As in syphilis, borrelian meningoencephalomyelitis could be the third stage of the disease. Its treatment, often disappointing, consists of antibiotics (penicillin G or latamoxef) and in some cases corticosteroids.

Adult

Pigmentary type of orthochromatic leukodystrophy (OLD): a new case with ultrastructural and biochemical study.

A 34-year-old woman with no family history of orthochromatic leukodystrophy (OLD) developed progressive intellectual deterioration, a frontal syndrome and spastic tetraparesis. She died four years after the onset of the clinical illness. Neuropathological studies included light and electron microscopy of cerebral and nerve biopsies, and a complete postmortem examination. Light microscopy demonstrated OLD with pigmented macrophages and glial cells. 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 oligodendrocytes in other forms of OLD. Biochemical study did not show evidence of demyelination. Galactolipids were normal. Polyunsaturated fatty acids were decreased. The most striking feature was an increase in plasmalogens.

Adult

Lateral gaze disturbance in a case of Wallenberg's syndrome.

A 63-year-old Caucasian man was admitted for Wallenberg's syndrome following a left vertebral artery thrombosis. In addition to the classical symptoms, an axial lateropulsion to the left and ocular motor disorders (vertical diplopia, tonic deviation of the gaze to the left, skew deviation and horizonto-rotatory nystagmus) were present. These clinical signs are unusual, but in common Wallenberg's syndrome, neurophysiological tests often reveal slight abnormalities of oculomotor function: impairment of jerks, skew deviation, lateral deviation of the gaze in darkness. Interruption of cerebellar pathways is thought to be the cause of these symptoms. Their existence does not seem to change the outcome of these cases.

Eye Movements

[Recurrent coma, papular mucinosis and benign dysglobulinemia].

A woman presented with a history of three regressive comas of undetectable etiology between the age of 52 and 57 years. An IgG lambda benign monoclonal dysglobulinemia was combined with a papular mucinosis (myxedematous lichen or the generalized form of Arndt-Gotton's scleromyxedema). In the 6 analogous cases documented in the literature the onset of coma occurred generally several weeks after an aggravation of the cutaneous lesions. The coma was preceded by an influenza-like syndrome followed by asthenia, malaise with vertigo and frequently epileptic seizures. During recovery, hallucinations and transient hepatic disorders were noted. Pruritus with pronounced hypereosinophilia preceded desquamation and regression of dermatologic lesions. These comas can lead to a fatal outcome (2 of 7 cases) or regress in 2 to 20 days usually without sequelae. The disease is probably of immunologic origin. The paraprotein or a serum factor could exert a direct toxic effect on brain. As in neurologic manifestations of malignant dysglobulinemia, explained initially by a "toxic encephalosis, clinical, angiography, biologic and immunologic data exist in favor of blood hyperviscosity. This hyperviscosity could result from polymer formation through intermediates immunoglobulins and other protein chains, or again from alteration of deformability of red cells by binding of paraprotein. Hyperviscosity syndromes are frequent in system diseases that are often associated with papular mucinosis. Whatever the exact mechanism of these "comas due to papular mucinosis", a logical choice is their treatment by immunosuppressants and plasmapheresis: in the case reported, the use of plasmapheresis as soon as premonitory signs had appeared probably prevented a fourth coma.

Alopecia

Mismatch between iodine-123 and xenon-133 SPECT rCBF measurement.

This report is concerned with the case of a man afflicted by multiple cerebral arteriovenous malformations. The rCBF measurement with Xe-133 inhalation was relatively elevated. However, I-123 iodoamphetamine imaging showed a relative reduction in radioactivity, and was a more accurate reflection of the clinical situation.

Amphetamines

Management of subdural intracranial empyemas should not always require surgery.

Seven patients with subdural empyema were initially treated by antibiotics without surgery. Six have recovered without sequelae. One required delayed surgery and has recovered with epilepsy. The authors emphasise the use of CT for the diagnosis and follow-up of subdural empyema, the principles and modalities of non-surgical treatment, and the good results, especially for late morbidity.

Adult