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Biomedical subjects

F Chain

Publications and source records attributed to F Chain.

At least 19 recordsLinked to original sources

[Central and peripheral neurologic lesions in primary Gougerot-Sjögren syndrome. Clinicopathological study of a case].

A 51 year old woman presented with cerebellar ataxia and paralysis of conjugate lateral movement of the eyes. She had xerophtalmia and xerostomia. Biopsy of minor salivary glands showed mononuclear infiltrates typical of Sjögren's syndrome. Peripheral neuropathy was of the axonal type, and nerve biopsy showed a vasculitis made of a mononuclear infiltrate with only a few polymorphonuclears. The patient deteriorated despite corticosteroid therapy, plasma exchange and cyclophosphamide. Ciclosporin seemed to be transitorily beneficial. Epileptic seizures (auditory hallucinations) occurred. MRI showed hypersignal in the right temporal lobe. At post-mortem examination, a small ischaemic lesion with a large lymphocytic infiltrate was present in the left putamen. Neuronal loss and gliosis with some rod cells and glial stars were found in the brainstem, dentate nucleus and internal part of the right temporal lobe. Inflammatory changes were limited. The posterior columns of the spinal cord were pale, and some residual nodules of Nageotte were present in the spinal ganglia. These lesions were similar to those seen in encephalomyelitic syndromes associated with carcinoma. Lesions of the peripheral nerves were associated with vasculitis but changes in the central nervous system were morphologically similar to those observed as remote effects of carcinoma.

Central Nervous System Diseases

Increased wave P 300 latency in progressive supranuclear palsy.

The visually evoked P 300 wave and related reaction times (RTs) were studied in 25 patients with progressive supranuclear palsy (PSP). Both the P 300 wave latency and the RTs were significantly increased compared with 14 control subjects, and were correlated with an intellectual deterioration index calculated from neuropsychological scores. It is suggested that the study of wave P 300 may contribute to the diagnosis of cognitive disorders in PSP.

Aged

Dissecting aneurysm of the vertebral artery and cervical manipulation: a case report with autopsy.

A 35-year-old woman with 3 weeks of cervical pain developed ischemia in the basilar artery territory following cervical manipulation. At autopsy, there was a dissecting aneurysm within the third segment of the right vertebral artery. The pathologic changes in the lower and the upper part of the dissecting aneurysm were different, indicating recurring bleeding. Cervical manipulation could have accounted for one recent dissection, but not for another, which was a few weeks old. This suggests that cervical pain, which prompted the manipulation, may have been the first symptom of the dissection, and manipulation of the neck precipitated the stroke by inducing bleeding within the dissecting aneurysm.

Adult

Manic-like state after bilateral orbitofrontal and right temporoparietal injury: efficacy of clonidine.

A manic-like state occurred in a 44-year-old right-handed woman with bilateral orbitofrontal and right temporoparietal traumatic contusions. In a brief trial, we assessed the effect of clonidine, carbamazepine, dopa therapy, and placebo on manic symptoms and cognitive functions. Clonidine rapidly reversed the manic syndrome. The patient's behavior did not change with carbamazepine and worsened with levodopa. We suggest that the manic-like syndrome was related to noradrenergic overactivity secondary to the fronto-orbital lesions.

Adult

[Retrochiasmatic lesions in multiple sclerosis. Demonstration by visual evoked potentials. Correlation with magnetic resonance imaging].

Monocular stimulation of each visual hemifield can show an interhemispheric asymmetry of VEP. Validity of this test needs a reproducibility of responses and exclusion of stimulation induced by eye movements. In a prospective study of 22 MS cases, it appeared that interhemispheric asymmetry was a criterion of dissemination is space and had a good diagnostic value: MS became clinically definite in 10/12 cases; in 10 other cases in which a correlative MRI-VEP study was possible, there were disseminated high signal areas in T2 weighted sequences on hemispheric MRI. In 7/10 cases, these areas were located on retrochiasmatic visual pathways. With MRI, VEP are the most performant tests for early diagnosis in MS. Technical progress will improve its fiability. Prospective correlative clinical, electrophysiological and MRI studies are necessary on a larger number of MS patients.

Adult

Serum and CSF humoral immunity in Guillain-Barré syndrome: clinical correlations.

Paired samples of CSF and serum obtained from 29 patients affected with Guillain-Barré syndrome (GBS) were analyzed for various protein levels, including immunoglobulins and complement components. An attempt was made to correlate these findings to the clinical stage, severity, and duration of the disease. Intrathecal IgG synthesis was detected and quantified by means of a previously reported formula. It is practically constant in the GBS during the stage of stabilized paralysis, and is significantly greater in this stage than in the stage of progressive paralysis. Moreover, it increases with severity and duration (longer than 3 months) of the disease. Evidence of intrathecal C3 consumption is also presented.

Adolescent

[Complete human Klüver-Bucy syndrome after encephalitis caused by herpes simplex type 2].

A case of complete Klüver-Bucy syndrome is reported. A 42 year-old man developed Herpes Simplex type II (H.S.V.II) encephalitis. Good sparing of language functions allowed thorough neuropsychological testing. The troubles usually described in Klüver-Bucy syndrome as psychic blindness, colour agnosia, prosopagnosia, auditive and tactile agnosia were present. The authors theorize that these symptoms are mainly correlated with the amnestic syndrome, which is constantly reported in human Klüver-Bucy syndrome. Unfortunately, after 8 weeks, the encephalitis recurred and the patient was left demented and untestable.

Adult

Unilateral visual neglect and wave P 300. A study of nine cases with unilateral lesions of the parietal lobes.

Wave P 300 was studied in nine patients with parietal lobe lesions who had visual neglect but no hemianopia. Stimulation of the hemispace contralateral to the lesion showed a lengthening in latency and a decrease in amplitude of P 300, specifically related to visual stimulation. Comparison with results obtained in 14 normal subjects and four patients with localized hemispheric lesions without visual neglect showed that changes in P 300 were specifically related to visual neglect. The parietal lobe lesions may disturb cerebral processes involved in discrimination and visual stimulus evaluation required to perform a task, inducing disturbance of the system responsible for P 300.

Adult

Pontine metastasis with dissociated bilateral horizontal gaze paralysis.

A clinicopathological case of pontine metastatic tumour is reported with an oculomotor syndrome including bilateral horizontal gaze paralysis affecting saccades and foveal pursuit. During full-field pursuit, oculocephalic movement, and after caloric stimulation, the right eye alone was able to move slowly only 30 degrees to the right of the midline. Convergence and vertical eye movements were unaffected in either eye. The lesion lay in the whole left pontine tegmentum and partly in the right pontine tegmentum which was also strongly compressed and displaced to the right. The bilateral horizontal gaze paralysis resulted from damage to both paramedian pontine reticular formations. The unusual combination of an absence of foveal pursuit with the persistence of a rightward full-field pursuit analysed in the light of recent experimental work, suggested a clear separation between the brainstem pathways of these two types of pursuit movement. Lastly, according to our data and other clinicopathological findings previously reported, it appeared also that the paramedian pontine reticular formation role in the triggering of voluntary vertical saccades is less significant in man than in the monkey.

Brain Neoplasms

Miller Fisher syndrome: clinical and electrophysiologic evidence of peripheral origin in 10 cases.

Clinical and electrophysiologic data recorded in patients with Fisher syndrome characterized by ophthalmoplegia, ataxia, and areflexia are presented. Cases with limb weakness or pleocytosis in the CSF were excluded, according to Fisher. Ten patients were selected. All had hand and foot numbness. A large amount of protein without cell reaction was found during the third week of illness in serial CSF examinations. EMGs showed very slight limb involvement without spontaneous activity, and in most cases, facial muscles exhibited a denervation pattern. Distal motor nerve conduction velocity on limbs and F wave latencies were normal, whereas the sensory nerve action potentials were altered in all but one case when tested (seven out of eight cases). By means of blink reflex study performed in four patients, no significant pattern of brainstem dysfunction was discovered. The authors discuss the preeminent role of peripheral nerve lesions with regard to the ataxia and ophthalmoplegia.

Adolescent

[Baló's concentric sclerosis].

A clinico-pathological case of concentric sclerosis (Baló type) is reported. A 30-year-old man experienced dizziness, nausea and vomiting. Twelve days later he developed gait disturbances. Neurological examination showed broad based gait, brisk tendon reflexes, bilateral extensor plantar responses, right hemihypoesthesia, cerebellar dysmetria, and a left lateral gaze palsy. CSF examination showed, 520 mg p. 100 ml protein, 7500 red blood cells, 31 lymphocytes and 9 polymorphonuclear leukocytes/mm3, 18 p. 100 gammaglobulin. Three CT scans were performed and showed a round hypodensity in the parieto-occipital white matter with contrast enhancement on one occasion, and several other hypodensities in the contralateral parieto-occipital white matter and in both frontal lobes. 23 days after the onset of the disease, the patient became comatose. A cerebral biopsy was obtained from one of the frontal lesions. He died from aspiration bronchopneumonia 2 months after the first signs. Neuropathological examination showed numerous concentric zones of demyelination which involved the white matter of both hemispheres, brain stem, and cerebellum. On light microscopy sudanophilic myelin breakdown products were numerous in the bands of demyelinisation. Astrocytic proliferation was marked, with frequent Rosenthal fibers. Edema was noted in some lesions. Myelin-axonal dissociation was obvious, but some axonal swelling were observed. Electron microscopy demonstrated the integrity of oligodendrocytes and of blood vessels and confirmed the prominent alterations of the astrocytes. Fifteen similar cases of the literature have been reviewed. The present case seems to be the first one with CT scan examination and electron microscopic study of a brain biopsy. The nosological situation of Baló's disease among the inflammatory demyelinating diseases of the group of MS is discussed.

Adult

Parinaud's syndrome: electro-oculographic and anatomical analyses of six vascular cases with deductions about vertical gaze organization in the premotor structures.

Six cases of Parinaud's syndrome, with downward (Cases 1, 2), upward (Cases 3, 4) and both downward and upward gaze paralysis (cases 5, 6) are reported. Four cases (Cases 1, 2, 3, 5) were studied anatomically using serial sections of the brain and 3 cases (Cases, 1, 4, 6) analysed electro-oculographically. In all the cases there were rather small vascular lesions in the mesodiencephalic region, sparing the oculomotor nuclei. Since the rostral interstitial nuclei of the medial longitudinal fasciculus (riMLF), located above the oculomotor nuclei, contain the final relays producing all vertical saccades, it is suggested that the different aspects of Parinaud's syndrome may result from damage to their cells or to their excitatory efferent tracts, or even to their afferent pathways. Downgaze paralysis results from bilateral lesions involving the regions located just caudal, medial and dorsal to the upper poles of the red nuclei. The critical area is probably related to the mediocaudal part of the riMLF, the lateral portion of which appears to be spared. These anatomical data, combined with the clinical observation that most downward eye movements (except slow reflex movements) are affected in the case with such paralysis, lead us to propose that it is the riMLF efferent tracts mediating downgaze and projecting on to the oculomotor nuclei that are principally damaged by the lesions. Upgaze paralysis results from unilateral lesions in or near the posterior commissure. The clinical data allow us to propose that it is also the riMLF efferent tracts, mediating upgaze, that are damaged in such cases. consequently these tracts, probably originating from the dorsolateral part of the riMLF, would decussate through the posterior commissure before they reach the oculomotor nuclei. Combined downgaze and upgaze paralysis results from bilateral lesions involving the region related to the whole riMLF on both sides. The principal conclusion is that the riMLF efferent tracts mediating upward and downward gaze have clearly separate courses in the immediate premotor structures.

Adult

[The pontine reticular formation syndrome. Physiopathologic data on voluntary eye movement abnormalities].

A new clinical case of Pontine Reticular Formation (P.R.F.) syndrome is reported and analysed using electro-oculograms. The CAT scan showed a probably metastatic tumour, clearly limited and located in the ventral part of the left tegmentum in the lower pons. The oculomotor disorders were typical of the P.R.F. syndrome and similar to those previously reported in pathological studies. The left P.R.F. damage was clinically evidenced by the loss of all leftward saccades (voluntary saccades and quick phases of nystagmus), including also those situated in the right hemifield of movement. It was noticed that the voluntary return movement driving both eyes from the right lateral position to the midline was remarkably slow and twice slower for the left eye (10 degrees/s) than for the right one (20 degrees/s). The loss of pursuit movements in the left hemifield of movement suggested that the lower part of the left P.R.F. was affected by the lesion. The preservation of normal leftward oculocephalic movements for the right eye indicated that the left abducens nucleus was spared. Lastly, the complete abductive paralysis of the left eye including the oculocephalic movement implied that the left abducens motoneurons passing through the lower part of the P.R.F. were damaged. All these functional and structural correlates are consistent with the location of the lesion given by the CAT scan. They are reviewed in the light of recent experimental and clinico-pathological data. A detailed physiopathological interpretation accounting for the slow voluntary movements, observed in place of the leftward return saccades in the right hemifield of movement, is proposed. The slowness of the movements of both eyes results from lack of phasic excitation of the motoneurons of the agonist muscles and from lack of phasic inhibition of the motoneurons of the antagonist muscles, both mechanisms being suppressed by the lesion which damages the excitatory burst neurons of the left P.R.F. As the tonic cells of the left P.R.F. are also out of action, these slow movements could mainly be controlled by the tonic cells of the right P.R.F. For the left eye, as the root fibres of the left abducens nucleus are damaged, no excitation can occur on the agonist muscle (left lateral rectus) and there is only a voluntary tonic disfacilitation of the motoneurons of the antagonist muscle (left medial rectus). For the right eye, the same mechanism involving the antagonist muscle (right lateral rectus) would be combined with some tonic excitation of the motoneurons of the agonist muscle (right medial rectus), thus accounting for the twice faster velocity of this eye. This tonic excitation reaching the right medial rectus could be mediated either by one of the other afferent neurons of the left abducens nucleus encoding eye position--namely those arising from the vestibular nuclei or from both prepositus hypoglossi nuclei--or by the right ascending tract of Deiters projecting to the right medial rectus motoneurons.

Abducens Nerve