Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We presented a rare care who had right frontal lobe infarction, with left side pseudoataxia, and the mechanism, causing pseudoataxia, was considered. The patient, a 51 year-old, righ-handed male, was admitted on August 9, 1980, complaining of left-side pseudoataxia. About p.m. 7:00, July 29, 1980, he suddenly noticed numbness of the left foot, and he found himself difficulty in standing in the next morning. He had a mild paresis and tactile-tactile of the left side including the face, which was rapidly improved. However, there was pseudoataxia of the left extremities, which had not been improved. On physical examination, dysarthria, aphasia, finger agnosia, difficulty in right left orientation or muscle weakness was not recognized, and there was no sensory disturbance except for slight impairment of stereognosis, two point discrimination and vibratory sense. Demonstrable impairment of tactiletactile from was observed in the left hand. Notable dysmetria, terminal tremor and dysdiadochokinesia were seen in the left limbs, which were remarkably worsened with eyes closed. However, tapping and line-drawing tests were normal. Babinski-Weil's test disclosed typical compass gait. There was marked swaying in Romberg position. Tandem gait was impossible with a tendency to decline the left. Deep reflexies were normal except for mildly hyperactive radial reflex in the left. Carotid and vertebral angiographies revealed neither evidence of vascular occlusion nor displacement of vessels CT scan demonstrated a low density area, which included the right inferior and middle frontal gyri, the head of the right caudate nucleus and a part of anterior crus of right internal capsule. There was enlargement of anterior horn of the right lateral ventricle. Caloric test, electronystagmography, eye tracking test or optokinetic nystagmus test disclosed no abnormalities. Vibration induced falling, which is the postural reaction to muscle vibration during standing (Ekuland, G., 1972), was not recognized when the left Achiles' tendon was stimulated. Pseudoataxia of this patient differed from the typical cerebellar or vestibular ataxia. From a review of the literatures concerning frontal pseudoataxia, almost all cases had no distinct cerebellar signs, and showed positive Romberg's sign. The impairment of tactile-tactile form and postural reaction to vibratory stimulation to the left leg, appeared in this case, could be hardly explained by the lesion of parietal lobe or deconnection syndrome. Sensory perception of parietal lobe and pyramidal motor system were thought to be almost normal in this case. Therefore, these findings should be due to impairment of integration center between sensory and motor systems. The pseudoataxia in frontal lesion seems to occur as the results of involvement of this center, in which caudate nucleus maybe has important role, but not as the results of disturbances in the front-ponto-cerebellar or front vestibular pathway.
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PMID:[Frontal pseudoataxia, discussion on its mechanism (author's transl)]. 732 87

Two Japanese male siblings, aged 68 and 59 years, affected by late-onset progressive ataxia distinguished by extensive sensory and mild autonomic disturbances are described. They had global thermoanalgesia, positive Romberg signs, sensorineural deafness, canal paresis and ageusia. Their autonomic disturbances consisted of absence of overflow tears with usual stimuli, dysphagia, blood pressure and vasomotor instability, diarrhoea/constipation, and urinary frequency. Sensory nerve action potentials were completely absent, whereas motor conduction velocity was slightly reduced only in the lower extremities. Sural nerve biopsy on the younger brother demonstrated a marked loss of myelinated fibres and a reduction in the number of unmyelinated axons. Tongue histology revealed absence of fungiform papillae and taste buds. Autonomic function tests showed widespread but mild sympathetic and parasympathetic failures. Neuro-imaging studies revealed atrophy of the spinal cord, cerebellum, brainstem and corpus callosum, and enlargement of the lateral, third and fourth ventricles. These siblings represent a previously unrecognized variant of late-onset hereditary spinocerebellar degeneration with global thermoanalgesia and absence of fungiform papillae on the tongue.
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PMID:Late-onset hereditary ataxia with global thermoanalgesia and absence of fungiform papillae on the tongue in a Japanese family. 867 78

Forced duction and generation testing of a patient with Parry-Romberg syndrome (progressive hemifacial atrophy) confirmed that his incomitant hypotropia and esotropia were restrictive and not due to nerve paresis. This suggests that an orbital inflammatory process (causing extraocular muscle fibrosis) is part of this rare and poorly understood syndrome.
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PMID:Restrictive strabismus in Parry-Romberg syndrome. 1727 38

A 68-year-old male who suffered from dementia, progressing for four months without Parkinson's symptoms, was admitted to the Department of Neurology because of vertigo, slight left hand paresis and positive Romberg test. During hospitalization the patient's status deteriorated. The intracerebral lobar haemorrhage, subarachnoid haemorrhage and ischaemic lesions observed on CT scans suggested the clinical diagnosis of CAA. He died after 53 days due to pneumonia. On macroscopic examination, the brain showed general cortical atrophy and ventricular dilatation. Frontal lobar haemorrhage and focal subarachnoid haemorrhage were seen on the brain autopsy. Microscopic observation demonstrated neuronal loss and microspongiosis in the hippocampus, severe neuronal loss and depigmentation in the substantia nigra pars compacta and locus coeruleus. Lewy bodies were visible in the substantia nigra and amyloid angiopathy, predominantly severe CAA according to the Vonsattel scale, in the meningeal and cortical vessels. In the presented case, the microscopic findings were typical for DLB with concomitant severe CAA. In progressive dementia, neurological deterioration, presence of lobar hemorrhagic infarcts and ischaemic lesions suggest CAA coexistent with DLB and/or AD.
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PMID:Complications of severe cerebral amyloid angiopathy in the course of dementia with Lewy bodies. A case report. 2122 12

The authors describe four patients with Parry-Romberg syndrome (PRS) who had abnormal eye movements, ptosis, and facial hemiatrophy. There were delays in diagnosis in all cases, often requiring multiple imaging studies, prior to diagnosis of PRS. These cases demonstrate the variable ophthalmic manifestations of PRS, which can lead to difficulty in diagnosis. Case 1 was a 23-year-old woman with lateral rectus paresis who presented with horizontal diplopia and developed ptosis and facial hemiatrophy. Case 2 was a 28-year-old woman with medial rectus weakness adjusted to diplopia by head turn and gaze preference. Case 3 was a 68-year-old woman who presented with dry eye symptoms and was noted to prefer right gaze and have left hemifacial atrophy. Case 4 was a 68-year-old woman who presented with ptosis, enophthalmos, and restriction of up gaze. The ocular motor defect in all of these cases is best explained by muscle fibrosis rather than nerve paresis.
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PMID:Ocular motor dysfunction in Parry-Romberg syndrome: four cases. 2214 72

The authors describe a patient with Parry-Romberg syndrome (PRS) who had acquired monocular elevation deficit (MED), ptosis, and facial hemiatrophy. A 12-year-old female patient manifested progressive ptosis and hypotropia companied with progressive hemifacial atrophy was conducted to the ophthalmology clinic for diagnosis and treatment. Forced duction and generation testing confirmed that her incomitant hypotropia was caused by paralytic muscles and not due to restriction, so it was diagnosed as MED. Knapp procedure was conducted to correct her eye positions. Six months later, the frontal muscle suspension surgery was performed to treat her ptosis. Postoperatively, the patient had orthotropia without residual left hypotropia in the primary position and her left upper eyelid function improved significantly. The ocular motor defect, in this case, is best explained by nerve paresis rather than muscle fibrosis.
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PMID:Acquired monocular elevation deficit in a case of Parry-Romberg syndrome. 3162 56