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Biomedical subjects

N Giard

Publications and source records attributed to N Giard.

14 recordsLinked to original sources

Hereditary ptosis of late onset: early observations on oculopharyngeal muscular dystrophy in Quebec by Roma Amyot.

In 1948, Roma Amyot, a well-known French-Canadian neurologist, observed in ten families a late onset syndrome consisting of hereditary ptosis which was sometimes associated with dysphagia but rarely with limb weakness. At that time, Taylor's original work dating back to 1915 was still unknown to him. Nonetheless, these reports constitute the two earliest publications about this syndrome prevalent in the French-Canadian population. Amyot recognized the myopathic nature of this disease which was later called oculopharyngeal muscular dystrophy (OPMD) by Victor et al.

Age of Onset↗

Short-term and long-term memory deficit following intracarotid Amytal injection: further support for the memory consolidation hypothesis.

To test the three main hypotheses of the human amnesic syndrome (encoding, consolidation, retrieval), we designed an original protocol for memory assessment under Amytal that included, in addition to a retrograde memory measure, both short-term and long-term anterograde memory measures. Twenty epileptic patients with SEEG-confirmed unilateral temporal lobe foci were given right and left injections on successive days. Only the long-term memory measure for material presented under Amytal was significantly related to the presence of a temporal contralateral epileptogenic focus, even if it was assessed when the hemisphere had completely recovered from the effect of Amytal. Short-term memory deficits were observed equally often after injections ipsilateral and contralateral to the epileptogenic focus, and no retrograde amnesia was observed. These results are consistent with the consolidation hypothesis.

Adolescent↗

Anterior callosotomy and chronic depth electrode recording in the surgical management of some intractable seizures.

Chronic depth electrode recording has proven to be a safe and indispensable tool for the investigation of intractable seizures. Anterior callosotomy appears as an excellent palliative treatment at least in bifrontal foci. It is hoped that chronic depth electrode recording will be used more widely before callosotomy in order to establish more clearly the best indication for such a surgical procedure.

Corpus Callosum↗

Balint's syndrome.

Balint's syndrome is usually attributed to bilateral parieto-occipital lesions. In several reported cases involvement of the frontal lobes was also documented and could be responsible for the "spasmodic fixation" often recorded in these patients. We report a case of Balint's syndrome in a patient with bilateral frontal and parieto-occipital metastases demonstrated by CT scan and confirmed by postmortem examination.

Brain Diseases↗

Immunological features in multiple sclerosis.

A clinical and laboratory profile of the immunological system of patients with multiple sclerosis (MS) strongly suggested that many specific immune deficiencies exist in MS. The immunological history showed that patients with MS had had more tonsillectomies, appendicectomies, and childhood infections than matched controls, which suggested that there had been problems in controlling various types of childhood infections. The cell-mediated immune response and the circulating antibody titres were specifically impaired against a variety of antigens. Patients with MS had significantly lower serum antibody titres than controls against many naturally occurring antigens-namely, diptheria and tetanus toxoids, adenovirus, and mumps viruses. Raised serum antibody titres were found against measles and varicella zoster viruses while no difference was found towards other antigens. The delayed hypersensitivity reaction and the immunological memory of patients with MS were also greatly reduced against the mumps skin test antigens. There were normal amounts of circulating T and B lymphocytes, and the phytohaemagglutinin, concanavallin A, pokeweed mitogen, and encephalitogens lymphocyte transformation was not different from that in controls. These results indicated that patients with MS have more infectious problems than normal people and that both their T and B cell systems cannot mount a fully normal immunological response to some viral and bacterial antigens, while they give an increased response to others.

Adult↗

Cerebrospinal fluid proteins in multiple sclerosis.

Various CSF proteins were studied in 255 definite multiple sclerosis patients at various disease stages and compared with corresponding values obtained from 174 controls. The CSF changes in acute multiple sclerosis patients included a significant increase of total proteins and of gamma globulin, IgG, IgA, IgM, alpha-2 ceruloplasmin, 7S-gamma-1, and cytotoxic index for nerve cells in tissue culture, and significant decreases of pre-albumin, alpha-1, and alpha-2 and of the beta/gamma globulin ratio. The CSF levels of IgG, IgA, and IgM remained significantly higher in steroid-treated multiple sclerosis patients than in controls, but the levels often were significantly reduced while patients were on treatment or in remission. During remission or treatment with ACTH and/or steroids, the alpha-2 ceruloplasmin, 7S-gamma-1, and cytotoxic index were significantly reduced and the pre-albumin, alpha-1, and alpha-2 globulin classes and the beta/gamma ratio showed a tendency to return to normal.

Adolescent↗

Electrical stimulation of the human brain in epilepsy.

The concordance between the epileptogenic area as determined by intracranially recorded spontaneous seizure onset and that determined by electrically induced auras or seizures in 126 patients with medically intractable epilepsy was studied. Four to 11 multicontact depth electrodes were chronically implanted in patients as part of a preoperative investigation procedure. Localization of the epileptic area was obtained from the recording of three spontaneous seizures and from stimulation data including self-reported auras, seizures, and intracranial EEG afterdischarges recorded during an incremental stimulation sequence at all cortical pairs of contacts. Positive responses to stimulation were obtained in 63% of patients. Concordance between spontaneous and induced auras or seizures was greater than 90% with single unilateral foci and less with multiple foci. Stimulation data reliably predicted the resection area in unilateral and bilateral temporal foci but not in other locations. Afterdischarge thresholds were not reliable predictors of the spontaneous seizure focus. The intracranial stimulation procedure used appears to provide useful confirmatory and complementary localizing information in relation to that obtained from spontaneous seizures.

Brain↗