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Biomedical subjects

F Gray

Publications and source records attributed to F Gray.

At least 253 records · Page 14Linked to original sources
PubMed1981

Rigid spine syndrome: histological examinations of male and female cases.

Histological findings in the limb-muscle of two new cases of Rigid Spine Syndrome are described. The first male case exhibited a disproportion in fiber type with predominance of the type II fibers with a slight increase of large fibers. Some of the type I fibers were slightly atrophic. No other consistent abnormalities could be seen, excepted thickness of vessel basal lamina. The second female case had a relatively distinct muscular pattern. Necrotizing phenomenon were mild without fibrosis. Type I predominance were obvious. Atrophic and hypertrophic fibers could be seen in both types populations, with a relatively higher rate in type II. These two additional cases and those of the literature showed varied non specific histology, without any histo-chemical nor ultrastructural characteristic pattern.

Adenosine Triphosphatases↗
PubMed1981

Pallido-luyso-nigral atrophy and amyotrophic lateral sclerosis.

Clinical and neuropathological studies of a case of pallido-luyso-nigral atrophy and amyotrophic lateral sclerosis (ALS) in a young woman with a strong likelihood of a similar familial past medical history have been presented. Microscopic examination revealed neuronal loss and gliosis of globus pallidus, corpus luysii and substantia nigra. Pallor of the pyramidal tracts and neuronal loss in hypoglossal nuclei and anterior horns with gliosis were present. The rarity of the association of a pallido-luyso-nigral atrophy and an ALS, the occurrence of an ALS at such a young age and the fact that her grandmother died of Parkinson disease at age 30 suggest that this association may represent more than a coincidental occurrence.

Adult↗
PubMed1981

Bilateral infarction of the anterior cingulate gyri and of the fornices. Report of a case.

A 70-year-old woman had complex behavioural changes of sudden onset. The symptoms consisted of indifference, docility and inappropriate urination, but predominantly in a lack of attention. She was unable to maintain the attention necessary to perform a goal-directed activity and she was distracted by any stimulus, such as a sound, an object, or a word, which might induce behaviour irrelevant to the preconceived activity. She also exhibited confabulatory-amnestic syndrome. Neuropathological examination of the brain revealed infarcts in th territories of both anterior cerebral arteries. The rostral part of the anterior cingulate gyrus (Acg), small areas of the adjacent medial prefrontal cortex, and the underlying white matter were destroyed bilaterally. Infarction involved the deep territory of the left anterior cerebral artery, with a bilateral lesion of the fornices. This cingulate damage was more restricted than the Acg lesions reported in some cases of akinetic-mutism, which extended more caudally, but was presumably larger than the lesions created in psychosurgery. The impairment of attention was analyzed according to the possible roles of the cingulate and of the fornix lesions as causing a dysfunction between the frontal lobes and the hippocampal formations.

Aged↗
PubMed1981

[Angio-immunoblastic lymphadenopathy and neurological manifestations (author's transl)].

Cases of associated angio-immunoblastic lymphadenopathy (AIL) and peripheral neuropathies have been rarely reported in the literature. Three such cases are described, in two men and one woman aged 79, 59, and 45 years respectively. Diagnosis of AIL was confirmed by lymph node biopsy in the two latter patients during their lifetime, and in the first case from examination of cervical nodes at autopsy. All three patients presented neurological disorders, of the polyradiculoneuritis type in the first case, multiple neuritis of the lower limbs followed by radiculalgia in the second, and myalgia and neuralgia with neurogenic signs in the EMG in the third case. No evidence of a toxic, metabolic, or infections aetiology was found, histological examination of nerve and muscle specimens demonstrated localised AIL lesions in one case, and discrete lymphoplasmocytic infiltration of the peripheral nervous system in the other two patients.

Aged↗
PubMed1981

[Neuropathy in angioimmunoblastic lymphadenopathy (author's transl)].

Four cases of angioimmunoblastic lymphadenopathy associated to peripheral neuropathy are described. The neuropathy was mixed, sensory and motor, more or less extensive, always asymetrical. In two cases, the clinical symptomatology and the clinical course were very peculiar, characterized by sensory disorders of a precise topography, circumscribed and sometimes suspended and by a relapsing and remitting course. In the third case, the neurological signs were acute and rapidly extensive with mandatory respiratory assistance. In this case, death occurred after a few weeks and the exact diagnosis was only attained at post-mortem examination. In the fourth case the neuropathy was very painful but the course was slow. In all four cases marked and extensive pain was present prior to the neurological disorders. Electrophysiological abnormalities were a constant feature with a marked slowing down of nerve conduction velocity. CSF was normal at the beginning in one case but was otherwise markedly pathological with an increased number of cells due to a large number of lymphocytes ranging from 6 to 40 cells while protein ranged from 60 to 160 mg per 100 ml. Nerve and muscle biopsies were non specific, i.e. neurogenous muscular atrophy and demyelination, except in case n. 4 where specific angioimmunoblastic lymphadenopathy infiltrates were present both in nerve and muscle. In cases 1 and 3 a non specific lymphohistiocytic infiltrate was present in spinal roots and meninges. Corticotherapy was used and efficient in two cases. These data are compared with a review of the literature. Since 1976, 7 cases of angioimmunoblastic lymphadenopathy associated to peripheral neuropathy have been reported. Clinical, electrophysiological and biological features are similar. Only one case underwent a post mortem examination of the central nervous system: a non specific lymphocytic infiltration in the spinal roots and meninges was mentioned. The role of the dysproteinemia associated with the AIL in the occurrence of the neuropathies is discussed. It remains a possible factor only. The role of a massive specific localization of the pathological process is well established in only one case (case 4). Such localization in lymphomas are not unusual. In 2 of our cases as well as in 2 from the literature the pathological findings are non specific: mild lymphoplasmocytic infiltrates of spinal roots and meninges. Those lesions are similar to neuropathies associated to non metastasizing neoplasms or malignant hemopathies.

Aged↗
PubMed1981

[Creutzfeldt-Jakob disease in the squirrel monkeys].

Four different strains of Creutzfeldt-Jakob disease virus (2 primary and 2 passaged in primates or mice) were inoculated intra-cerebrally into squirrel monkeys implanted with continuously-recording indwelling electrodes. Simultaneous EEC and videotape recordings were made on unrestrained animals. In addition EEG recordings were made of evoked visual potentials on restrained animals. EEG abnormalities appeared in every animal before the first clinical signs (6 to 20 months after inoculation) and included generalized slowing, epileptiform patterns and occasional episodes of pseudo-periodic activity. Abnormal evoked visual potentials and disturbances of consciousness were also noted. All viral strains produced similar disorders and the death of inoculated animals. The relative frequency of epilepsy seen in the CJD-inoculated squirrel monkey contrasts with its irregular occurrence in most other monkey species, and its total absence in the spider monkey. This could be related to the lesser complexity of neo-cortical evolution in the squirrel monkey and a less pronounced development of inhibitory CNS mechanisms under the general control of GABA-ergic neurons.

Animals↗
PubMed1981

[Echodactyly syndrome, ectodermic dysplasia, facial cleft, and EEC syndrome : report on 12 cases (author's transl)].

Twelve cases of a rare syndrome are reported. Findings included a facial cleft, adactyly of the 2nd and 3rd fingers and 2nd and 3rd toes, and ectodermic dysplasia involving anodontia, hypotrichosis, and albinoid type pigmentation of the skin and the exoskeleton, with clear eyes and chronic conjunctivitis. An interesting finding was that the anodontia affected the permanent teeth only, initially involving the incisors and the second premolars (nine cases out of twelve). Inversely, the deciduous teeth were unaffected, except for the upper first molars in two cases. The canines, usually constantly present, were absent in two out of three cases.

Abnormalities, Multiple↗
PubMed1981

[Necrotic aspects of multiple sclerosis and Schilder's disease (author's transl)].

Two anatomo-clinical cases of a necrotic form of demyelinating disease are reported. The disease occurred in two women, had a late onset (patient were about 50 years old) and had a relapsing-remitting course during more than 10 years. The CSF displayed a high protein level over 125 mg/100 ml whereas the gamma-globulin level was normal. The anatomical study found symmetrical cavitations involving both hemispheres and optic tracts with clear-cut limits. Axons and myelin were both destroyed, only the vascular network being partially spared. At the lesion's border-line mononuclear cell infiltrates as well as some phagocytes with sudanophilic inclusions were found. The scarcity of the compound granular corpuscules suggest an old pathological process. A narrow zone of myelin-axonal dissociation was also observed. Astrocytic proliferations was unimportant. Blood vessels were normal. In one case plaques of multiple sclerosis were found in the spinal cord. Those two cases are unusual forms of a diffuse disseminated sclerosis: multiple sclerosis and Schilder's disease are considered as two anatomo-clinical variants of the same pathological process. The observed necrotic lesions are different from the acute necrotic forms of multiple sclerosis as the latter have rapidly developed. The long lasting course of the disease, over 10 years, allowed a complete resolution of the lesions explaining the cavitations. The late onset of the disease and the CSF high protein level are pointed out. The significant of the high protein level and normal gammaglobulin level in the CSF is discussed.

Brain↗
PubMed1981

[Asymptomatic multiple sclerosis - 3 cases (author's transl)].

Multiple Sclerosis (MS) cases found at autopsy in patients who had died from other diseases and in whom no sign or symptom could be related to MS are called "asymptomatic". Three cases are reported. The first patient was a 62 year old man who presented with a slowly progressive disturbance of gait, incontinence and deterioration of intellectual function. A falx meningioma was surgically removed. The patient died 3 years later with an acute respiratory illness. Examination of the brain disclosed evidence of the operation and numerous old plaques disseminated through the cerebral hemispheres (centrum semi-ovale, periventricular regions, internal thalamus and junction between cortex and white matter) and in the brain stem. The second case, a 77 year old woman with diabetes mellitus and hypertension, presented with cortical blindness and disturbances of memory of acute onset. She died one year later. Examination of the brain showed multiple infarcts involving the territories of both posterior cerebral arteries and the left middle cerebral artery. Numerous old plaques were seen in the periventricular regions, in the corpus callosum and in the left middle cerebellar peduncle. The third case, a 60 year old woman with mitral and aortic stenosis, presented with cortical deafness and transient right hemiparesis. She died 5 years later. Brain examination showed infarcts involving both middle cerebral artery territories. There was also many old plaques in the periventricular areas, thalamus, internal capsule, centrum semi-ovale, brain stem and right nucleus dentatus. In the 3 cases, the optic tracts were normal. The spinal cord, examined only in the first case, was also normal. The asymptomatic character of these MS cases can be explained first by the location of the plaques and the lack of spinal cord and optic tract involvement. It could also be due to the small size of the plaques and to axonal preservation. Such features are rare since our 3 observations have been selected from a pathological collection of 125 MS cases and 9,300 general neuropathological records. Six other cases have been previously reported by other authors.

Aged↗
PubMed1980

[Necrotic myelopathies and neoplastic pathologie. Three clinico-pathological cases (author's transl)].

Three clinico-pathological cases of necrotic myelopathies with a distant malignancy are presented. Two cases had a lymphosarcoma and one case a prostatic carcinoma. They were compared to 13 well studied other cases collected in the literature. These myelopathies were related to solid visceral tumours in 8 cases and to lymphomas in 5 cases. The disease could be individualized on clinical grounds (flaccid paraplegia with bladder and bowell incontinence and sensory loss without clear-cut upper boundary developing over a few weeks with normal CSF and fast impairement of general condition), and, on pathological features. It is characterized by one or several spinal cord necrosis areas, often asymetrical, involving mostly white matter, without any vascular topography. Axons are involved as well as myelin sheats. There is mild inflammation and no specific vascular alteration. There is no metastases in the cord, meninges, vertebral column or nerve root. No vascular occlusion is found. The mechanism of the disease is unknown. The frequent occurence of lymphomas could suggest the presence of immunopathological factors.

Adenocarcinoma↗
PubMed1980

[Acute spongiform leucoencephalopathy with selective intramyelinic involvement of U fibers associated with an ovarian carcinoma. Syndrome of disconnection of U fibers (author's transl)].

Case report of clinical, pathological and ultrastructural features in an acute spongiform leucoencephalopathy with selective involvement of U fibers. A 52 years old woman exhibited an acute encephalopathy of 2 months duration, with dementia and multifocal impairment of cortical functions. The cerebral cortex was normal. This acute dementia resulted from a diffuse intercortical disconnection. Spongy degeneration was only found in U fibers. No other changes were noted especially in basal ganglia, optics tracts, and spinal cord. The white matter status spongious was related to an intramyelinic oedema. Such intramyelinic oedema is known only in Van Bogaert and Bertrand and Canavan disease, which is quite different, and in toxic encephalopathies, especially those induced by the hexachlorophene and triethyltin. In the present case no drugs or toxins were found. An ovarian carcinoma was found at post-mortem examination.

Acute Disease↗
PubMed1980

[Paralysis of digestive tract with lesions of myenteric plexuses. A new paraneoplastic syndrome (author's transl)].

A 67 years old man was admitted on July 1979 for nausea, dysphagia and rectal pain. At age 64 he had undergone radiotherapy on the lower lip for an epidermoid carcinoma. He remained then healthy. His medical history was negative with the exception of chronic bronchitis. He had never been exposed to toxic agents or drugs and had never left Europe. A few days after admission he suffered acute intestinal obstruction but at laparostomy no etiology was found. At the same time the patient complained of pain in all four limbs and he was found to have diffuse wasting of muscles, areflexia and distal sensory loss. No sign of dysautonomia was present. Physical examination was negative with the exception of a cervical lymphadenopathy. The lymph node biopsy showed an undifferentiated metastatic carcinoma. Negative investigations included: blood cells count; serum ionogram and immunoelectrophoresis; thyroid function tests; serological test for Chagas' disease. The following abnormalities were found: ESR: 55-105; CSF protein: 145 mg/100 ml and 1 cell mm3; whole blood folic acid: 1,7 mg/ml; Hbs antigen was present in blood; EMG showed evidence of denervation but motor conduction velocities were normal. By September the patient's weakness had increased and complete intestinal obstruction persisted. At oesophageal, gastric and duodenal fibroscopy no contraction was visible, and biopsies were negative. The patient died of peritonitis on October 5th, 1979. At necropsy peritonitis secondary to multiple perforation of the large bowel was found. No recurrence of the lip carcinoma or metastase or evidence of a primary carcinoma was found. Light microscopy showed no evidence of amyloidosis or scleroderma. Examination of the alimentary tract showed abnormalities restricted to the myenteric plexuses which varied from one level to another. In the small bowel there was hyperplasia of the smooth muscle and the myenteric plexuses were enlarged by marked proliferation of Schwann cells. Severe neuronal loss and nodules of Nageotte were also noted. Schwann cells proliferation was less marked in the stomach and large bowel. Lympho-histiocytic infiltration strictly confined to the region of the myenteric plexuses was present in oesophagus, stomach, large bowel and rectum. Mild chronic inflammatory lesions were also found in anterior and posterior spinal roots and semi-lunar ganglia. The striking feature of this case is the association of an undifferentiated carcinoma and a polyradiculoneuritis with a complete alimentary tract palsy of rapid onset, secondary to lesions restricted to the myenteric plexuses. The low folate level was insufficient to explain the neuropathy. Investigations showed no evidence of the usual causes of intestinal pseudo-obstruction: muscular, dysautonomic, toxic, plexic (idiopathic, familial, inflammatory), Chagas' disease). The clinical course, the pathological pictures of the alimentary tract and spinal roots and the association with a carcinoma suggest that our case may represent a paraneoplastic syndrome...

Aged↗
PubMed1980

[Ramsay-Hunt's syndrome: a case report with pathological examination (author's transl)].

A patient developed dyssynergia cerebellaris myoclonica, following an epileptic attack at the age of 19 years, with the progressive onset of involuntary movements and a cerebellar syndrome. He was second of a family of six children in which the same affection was present in the older sister while the four others were not affected. The involuntary movements corresponded to krebs type III intentino myoclonus, to massive myoclonic movements, and Krebs type I fibrillary myoclonia. The E.E.G. changes were typical, particularly the constant reinforcement of epileptic potential by photic stimulation. Completely disabled at 35 years, the patient was hospitalized at age 51 and kept under observation for 18 months. The per os administration of 5-hydroxytryptophane (5-H.T.P.), 150 mg daily, without a decarboxylase inhibitor, resulted in a spectacular reduction of the myoclonic movements and a marked improvement in autonomy. The patient died at 53 years of age from deglutition disorders related to oropharyngeal myoclonus. Pathological examination revealed no abnormalities in the brain, particularly in the dentate nuclei and the superior cerebellar penduncles. The only lesions observed were in the spinal cord: atrophy and demyelinization of Goll's columns and of the posterior lumbar and dorsal roots, and slight atrophy of the lateral funiculi. This case raises the problem of the relationship between myoclonus and lesions of the dentate nucleus, observed in 13 out of 16 cases of anatomically confirmed D.C.M., and lesions of its efferent pathways, present in 8 cases out of 16. The case reported here is the only one with an isolated spinal cord lesion, apart from that of Bradshaw in which no lesion was found but the cord had not been examined. The improvement obtained with the precursor of serotonin, 5-H.T.P. in the Lance and Adams syndrome (in which the intention myoclonus is semiologically very close to that of D.C.M., whatever their respective lesions), suggests that pathogenic studies should be carried out along biochemical lines. Though all authors do not agree, many accept a disturbance in serotoninergic pathways in the genesis of intentional myoclonus. The improvement in clinical signs and symptoms obtained in the present case with low doses of 5-H.T.P. brings additional evidence in favor of this hypothesis.

Cerebellar Ataxia↗
PubMed1979

Moyamoya disease and intracerebral hematoma. Clinical pathological report.

This report concerns a hypertensive woman who suffered a stroke at the age of 51 and recovered from left hemiparesis after 3 to 4 months. During the subsequent 24 years she had four seizures which involved the left arm and face and became generalized, ending with left hemiparesis, from which she recovered after 4 to 5 days. Carotid angiography was performed in 1967, 1973 and 1974 and the characteristic picture of moyamoya disease was demonstrated. She died at the age of 77 with extensive vascular disease. The literature concerning 215 cases of moyamoya disease, in which there were 14 intracerebral hematomas, is reviewed and discussed.

Adult↗
PubMed1979

[Progressive dialytic encephalopathy. Role of the aluminium and neurological study. One case (author's transl)].

Report a typical case of dialytic dementia in a patient treated with aluminium gels. The course was fatal in fifteen months duration. Before interruption of aluminium gel intake, the aluminium blood level measured by atomic absorption spectrography was at 1300 microgram/l (normal less than 40 microgram/l). Cerebral aluminium was studied by the method of Le Gendre and Alfrey. On the three studied specimens of gray matter including, the parieto rolandic cortex, the thalamus, the cerebellar cortex, the mean aluminium concentration was seven times higher than the witness. The optic and electronic microscopy study showed important accumulation of lipofuscin. No neurofibrillary degeneration was observed. In contrast to the intensity of the clinical signs and the fatal course the cerebral lesions were slight.

Aluminum↗