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Biomedical subjects

I Dehaene

Publications and source records attributed to I Dehaene.

At least 19 recordsLinked to original sources
PubMed1996

Unilateral internuclear ophthalmoplegia and ipsiversive torsional nystagmus.

We report four patients with internuclear ophthalmoplegia and torsional nystagmus, ipsiversive to the side of the medial longitudinal fasciculus (MLF) lesion. The MLF lesion could be responsible for an inactivation of the ipsilateral interstitial nucleus of Cajal inducing a contraversive slow phase. The presence of a corrective ipsiversive quick phase implicates an intact rostral interstitial nucleus of the MLF.

Aged↗
PubMed1996

Malignant hyperthermia susceptibility in a patient with concomitant motor neuron disease.

Laboratory confirmation of a clinical suspicion of malignant hyperthermia (MH) susceptibility by the standard in vitro contracture test remains inconclusive in patients reacting only to caffeine or halothane (called 'Equivocal') or in patients with concomitant neuromuscular disease. The detection of point mutations in the ryanodine receptor gene potentially provides additional information in these cases. The diagnostic value of the Gly341 Arg mutation in a patient reacting in vitro only to caffeine was reported previously by Quane et al. (1994). The present report describes a patient with motor neuron disease carrying the Gly341 Arg mutation, expanding the diagnostic value of this mutation to the group of patients with neuromuscular diseases.

Adolescent↗
PubMed1996

Benign paroxysmal positional vertigo of the horizontal canal.

OBJECTIVES: To review the clinical features, electronystagmography findings, the possible mechanism, and a possible therapeutic approach to benign paroxysmal positional vertigo (BPPV). METHODS: Sixty-three cases of BPPV of the horizontal canal type have been reviewed. It is characterised by horizontal nystagmus and an intense vertigo, provoked by rotation of the head in a supine patient. The horizontal nystagmus beats towards the ground on both sides, becomes more pronounced when lying on the pathological side, and then the nystagmus often changes direction. RESULTS: Forty-eight patients underwent electronystagmography. On the pathological side, the first phase nystagmus had a mean latency of three seconds and a mean duration of 31.6 seconds. Nystagmus inversion occurred in 36 patients after a nystagmus free interval. The mean second phase nystagmus duration lasted 33.4 seconds. On the healthy side, the nystagmus had a mean latency of 3.4 seconds and a mean duration of 39.5 seconds. Fatigue was seen in six patients. Simultaneous involvement of the posterior canal was present in 16 patients. A liberatory manoeuvre was successful in six patients. CONCLUSIONS: The liberatory manoeuvre should be tried in patients with horizontal canal vertigo. It should not be performed in patients with severe cervical arthrosis, vertebrobasilar insufficiency, or when the patient has neck pain during the manoeuvre.

Adult↗
PubMed1996

The stroke syndrome of cortical vein thrombosis.

Cortical vein thrombosis without sinus involvement is rarely diagnosed, although it may commonly be overlooked. We report four cases of cerebral venous thrombosis limited to the cortical veins. The diagnosis was made on surgical intervention in one patient and by angiography in three patients. Together with a survey of the published cases, the clinical and neuroimaging patterns of our patients allow delineation of several features suggestive of cortical venous stroke. Focal or generalized seizures followed by hemiparesis, aphasia, hemianopia, or other focal neurologic dysfunction in the absence of signs of increased intracranial pressure should suggest this possibility. Neuroimaging (CT, MRI) shows an ischemic lesion that does not follow the boundary of arterial territories and often has a hemorrhagic component, without signs of venous sinus thrombosis. Conventional angiography demonstrates no arterial occlusion but may show cortical vein thrombosis corresponding to the infarct, although these may also be nonspecific findings. The role of MR angiography, which is well-established in sinus thrombosis, remains to be assessed in patients with brain ischemia due to isolated cortical vein occlusion.

Adult↗
PubMed1996

The anti-Hu syndrome: a clinical and immunological study of 7 cases.

The authors describe the clinical and biological data of seven patients with anti-Hu antibodies. Six of them displayed a small cell lung carcinoma (SCLC), but no cancer was detected in the 7th patient in spite of an extensive workup. The clinical heterogeneity of the anti-Hu syndrome is emphasized. The major symptoms were linked to a severe sensory neuropathy in three cases, to cerebellitis in two cases, to dysautonomia in one case, and to gastro-intestinal pseudo-obstruction in one case. One patient also displayed EMG abnormalities characteristic of the Lambert-Eaton myasthenic syndrome. Two patients developed opsoclonus or ocular flutter associated with severe confusion in the late stage of their disease. In four patients, the neurological signs and symptoms preceded the discovery of the SCLC, and in two cases the initial detection of anti-Hu antibodies prompted the successful search for this tumor. Immunopathological events injuring the peripheral and central nervous system are briefly discussed.

Aged↗
PubMed1995

Central nervous system disease in primary Sjögren's syndrome.

Primary Sjogren's syndrome is a systemic autoimmune disorder whose main characteristics are dryness of the eyes and mouth, caused by lymphocytic infiltration of the exocrine glands. Patients may also show signs of extraglandular involvement of lung, liver, kidney and vessel walls, as well as of central and peripheral nervous system, muscles and joints. We present a review of the literature on the involvement of the central nervous system in primary Sjögren's syndrome, which may mimic many other neurologic disorders, especially multiple sclerosis. Features that may help in distinguishing these two entities are presented.

Central Nervous System Diseases↗
PubMed1994

Deletion in the CMT1A locus on chromosome 17p11.2 in hereditary neuropathy with liability to pressure palsies.

Hereditary neuropathy with liability to pressure palsies (NHPP) is an autosomal dominant disease of peripheral nerves, characterized by recurrent focal neuropathies often with an underlying asymptomatic polyneuropathy. We report the clinical, electrophysiological, and histopathological findings in three families with HNPP and confirm the presence of a deletion on chromosome 17p11.2, including all the markers known to be duplicated in Charcot-Marie-Tooth disease type 1A. This deletion appears to be the underlying molecular deficit in this disease and provides additional evidence for the importance of this locus for peripheral nerve function.

Adolescent↗
PubMed1994

Self-treatment of acute migraine with subcutaneous sumatriptan using an auto-injector device: comparison with customary treatment in an open, longitudinal study.

In a multicenter open longitudinal clinical trial where 479 patients suffering from migraine with or without aura were recruited, patients treated at home one to three migraine attacks with their customary treatment, and subsequently, over a 3-month period, one to three migraine attacks with 6 mg sumatriptan sc using an autoinjector. The headache response to customary treatment was 19% at 1 h and 30.5% at 2 h, and was not significantly different when only attacks treated "adequately" according to accepted treatment recommendations were considered: 16% at 1 h and 35% at 2 h. In contrast, 69% and 82% of patients treated with 6 mg sumatriptan sc had mild headache or no headache at 1 and 2 h respectively, regardless of migraine type or duration of symptoms prior to treatment. Other migraine symptoms (nausea, vomiting, photo- and phonophobia) were effectively treated with sumatriptan. Recurrence of migraine was observed in 31% of patients and was well controlled by a second injection of sumatriptan. It is concluded that 6 mg sumatriptan sc, self-administered using an autoinjector, is well tolerated and more effective than most currently used acute treatments for migraine in a population of severely affected patients consulting a neurologist.

Acute Disease↗
PubMed1994

Jerk-waveform see-saw nystagmus due to unilateral meso-diencephalic lesion.

See-saw nystagmus is an uncommon but highly characteristic eye movement disorder comprising intorsion and elevation of one eye, with synchronous extorsion and depression of the other. It generally has a pendular waveform and is due to a midline, extrinsic, suprasellar mass lesion compressing or invading the brainstem bilaterally at the meso-diencephalic junction. This report deals with the clinical and MRI findings in three patients (and binocular three-dimensional quantitative oculographic findings in one patient) with a jerk waveform see-saw nystagmus due in each case to a unilateral meso-diencephalic lesion. In each patient the torsional component of the nystagmus fast phases rotated the upper poles of the eyes toward the side of the lesion. Jerk see-saw nystagmus can be clinically indistinguishable from pendular see-saw nystagmus and from the torsional-vertical nystagmus which occurs with medullary lesions. We propose that jerk see-saw nystagmus is due to unilateral inactivation of the torsional eye-velocity integrator, thought to be in the interstitial nucleus of Cajal, with sparing of the torsional fast-phase generator, thought to be in the adjacent rostral interstitial nucleus of the medial longitudinal fasciculus.

Adult↗
PubMed1994

Magnetic resonance examination of the inner ear and cerebellopontine angle in patients with vertigo and/or abnormal findings at vestibular testing.

The inner ears of 167 patients with vertigo and/or abnormal findings at vestibular testing were studied using magnetic resonance (MR). Pathology potentially explaining vertigo was found in 54 patients, and was detected in the posterior fossa (28%), the internal auditory canal (28%) and the membranous labyrinth (44%). The overall percentage of pathology and the percentage of pathology found in the membranous labyrinth was high and was probably influenced by the referral pattern in our hospital where high resolution MR of the inner ear (three dimensional Fourier transformation-constructive interference in steady state sequence; 3DFT-CISS sequence) is available. Unenhanced, and especially gadolinium (Gd)-enhanced T1-weighted spin-echo images, are needed to detect most of the pathology inside the internal auditory canal, and some of the lesions inside the membranous labyrinth. 3DFT-CISS images are the only images that can show fibrous obliteration of the intralabyrinthine fluid spaces, and are therefore necessary to recognise most of the intralabyrinthine pathology. Finally, T2-weighted spin-echo images are best suited to demonstrate cerebellar or brain stem infarction. An additional MR-angiography sequence (three dimensional Fourier transformation-fast imaging with steady precession; 3DFT-FISP) is used when vascular compression of the cochleovestibular nerve is suspected. MR is the method of choice to look for pathology in patients with vertigo, and allows detection of pathology that remains invisible with other imaging techniques. However, well adapted sequences are needed to detect these lesions.

Cerebellopontine Angle↗
PubMed1992

Combined trochlear nerve palsy and internuclear ophthalmoplegia.

We report a case of left superior oblique palsy combined with a right internuclear ophthalmoplegia. A right mesencephalic lesion involving the trochlear nerve nucleus (or its fibers prior to decussation) and the medial longitudinal fascicle was hypothesized. Magnetic resonance imaging showed a lesion at the suspected level.

Adult↗
PubMed1991

Paralysis of saccades and pursuit: clinicopathologic study.

We report a 73-year-old patient with an eye movement disorder characterized by paralysis of saccades and pursuit. At autopsy, there were small cortical lesions in the middle frontal gyrus immediately anterior to the precentral gyrus and in the inferior parietal lobule on both sides.

Aged↗
PubMed1991

Acute hemorrhagic leukoencephalitis.

We report an autopsy proven case of acute hemorrhagic leukoencephalitis. There was a very rapid evolution leading to death in less than 24 hours. CT of the brain revealed widespread hypodensities in both frontal lobes. In the cerebrospinal fluid as well as in the peripheral blood, there was a moderate leucocytosis. The literature is reviewed.

Acute Disease↗