Biomedical subjects
Publications and source records attributed to J de Recondo.
The case of a 70 year-old patient with familial dementia is reported. A cerebral biopsy and neurotransmitters dosages in CSF and brain tissue ruled out a primary dementia as Pick's or Alzheimer's disease. Our case may be related to Neumann's cases of subcortical gliosis.
Conduction in the proximal segment of the sciatic nerve was assessed by recording the latencies of M and H responses from soleus muscle, in the distal segment by comparing the latencies of M waves from the abductor hallucis muscle, obtained by stimulating the tibial nerve in the popliteal fossa and at the ankle. Data from 30 patients with amyotrophic lateral sclerosis (ALS) were compared with an age-matched group of healthy subjects. We observed an increase in the difference between the latencies of M and H responses from soleus, and a lengthening of the distal latency of the M wave from abductor hallucis. The motor conduction velocity of the tibial nerve was preserved. The functional significance of the data are discussed. It is suggested that they indicate a slowing of conduction in the proximal segment of the sciatic nerve and that they can be correlated with pathological findings in proximal nerves in ALS and in the experimental anterior horn cell disorder.
A 67 year-old right-handed man presented with an aphasia of acute onset associated to paralysis of vertical eye movements, mild cerebellar right dysmetria and right hemiparesis without hemianopia. CT scan showed two low density areas in the left thalamus and left occipital lobe. Neuropsychological examination revealed a non fluent aphasia with normal repetition, semantic paraphasias, perseverations and good comprehension of verbal and written commands. There was no alexia. Writing was impaired only by motor disturbances. Three weeks after the onset, tetraparesis and impairment of consciousness followed by a locked in syndrome supervened. Death occurred four months after onset. Neuropathological examination showed a left paramedian thalamic infarct involving the ventrolateral, dorso-medial and intralaminar nuclei, sparing the pulvinar. There was an occipital infarct sparing the calcarine scissura and multiple infarctions in the pons and the cerebellum. Broca's and Wernicke's areas were spared. We suggest that the involvement of medial nuclei could be partly responsible of language disturbances in thalamic aphasia.
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Initial reaction to the discovery of a carotid bruit in an adult is that a local atheromatous lesion exists. This sign may arise, however, when more distal lesions are present, such as an angioma provoking an increased rate of blood flow. All patients presenting with neurological signs of a lesion in one hemisphere should, therefore, in the absence of contra-indications, be investigated by arteriography.
A 19-year-old man presented with an apparently non-familial neurological disorder that had progressed from the age of 6 years. Dystonia of the trunk and limbs with extrapyramidal rigidity, dysarthria, a pyramidal syndrome with spasticity of the lower limbs, bilateral optic atrophy, and nystagmiform ocular movements were present. CT scan demonstrated symmetrical putaminal lesions. The different aetiologies of bilateral striatal lesions are considered, the final diagnosis being a juvenile form of Leigh's disease.
Three cases of cerebral vascularitis are reported. This affection does not occur frequently and is often unrecognized. It can be isolated or associated with disease such as sarcoidosis, Behcet's disease, or collagen disorders (lupus erythematosus, mixed connective tissue diseases). Diagnosis is made from the evolution with transient remission, the inflammatory nature of the CSF, and the presence of segmentary narrowing on arteriography. In case of doubt an exploratory operation with biopsy is necessary. The most likely etiology is an auto-immune disturbance, and cortico-therapy has greatly improved the prognosis.
Visuomotor ataxia is a disorder of movement performed under visual control. It can occur in the absence of disturbance of ocular fixation and in the absence of spatial agnosia. This disorder may extend over the whole visual field or it may be localized to one visual half-field, right or left. It may involve both hands or one hand only, so that visuomotor ataxia may be divided into: (1) Unilateral visuomotor ataxia, localized to a single field. In this case it may affect both hands or a single hand. It is direct when the hand is ataxic in the ipsilateral visual field and it is crossed when the hand is ataxic in the contralateral visual field; (2) Bilateral visuomotor ataxia, involving the whole visual field. Each hand may be ataxic only in the contralateral visual field, that is, bilateral crossed visuomotor ataxia; or in the ipsilateral field when it is called bilateral direct visuomotor ataxia. The observed clinical variations which are described here imply the existence of both direct and crossed visuomotor connections, the latter probably crossing the corpus callosum in the splenium.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The authors describe five cases of adrenomyeloneuropathy, an adult form of adrenoleukodystrophy. Three of the cases were related. The clinical picture comprises progressive spasmodic paraplegia, sphincter disorders, peripheral neuropathy, sometimes hollow foot and posterior columns disorders. In one case psychic disorders were observed. Biochemical analyses confirmed or revealed adrenal insufficiency in four cases and gonadal insufficiency in three cases. A detailed genealogical study was made in two patients belonging to the same family. Histocompatibility tests revealed the presence of A2 and B15 antigens in three cases. Diagnosis was confirmed in three cases by plasma determination of long-chain fatty acids, with an increase in the level of C26 acids and the C26/C22 ratio; biopsy of the peripheral nerve in one patient revealed inclusions characteristic of Schwann's cells. Symptomatological links between ALD and familial spasmodic paraplegia, the mode of genetic transmission of the disease, the contribution of neuromuscular biopsy to the diagnosis and the therapeutic prospects of a diet low in long-chain fatty acids are discussed.
Visuomotor ataxia causes difficulty in the prehension of objects under visual control which can occur without paralysis of ocular fixation and spatial agnosia. This can involve the total visual field or the right or left half-fields, and can affect both hands or one only. 1) Unilateral visuomotor ataxia is localized to the two right or left homonymous half-fields and can affect both hands or one only. It is direct when the ataxic hand is on the same side as the affected visual half-field, and crossed when the ataxic hand is contralateral to the affected visual half-field. 2) Bilateral visuomotor ataxia involves the total visual field. Each hand may be ataxic only for the contralateral visual field with a crossed bilateral visuomotor ataxia, or in the homolateral field with a direct bilateral visuomotor ataxia. The anatomical and clinical observations reported imply the existence of visuomotor connections, both direct and crossed, the latter crossing the median line through the corpus callosum.