Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a rare case showing Weber's syndrome associated with supranuclear vertical gaze palsy caused by the ipsilateral lesion of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), which is regarded as the supranuclear control center of vertical gaze. To date, no literature concerning Weber's syndrome associated with the ipsilateral riMLF lesion was documented. The patient was a 53-year-old female, who suddenly developed unconsciousness and left-sided weakness. Neurological findings on admission revealed right third nerve palsy, severe supranuclear vertical gaze palsy, almost complete convergence palsy, left hemiparesis and hyperreflexia with positive Babinski's sign on the left side. There was no sign of pupillary disturbance or no abnormality of the horizontal movements of the left eye. The vestibulo-ocular reflex of the left eye was preserved. There was no sensory disturbance. Cranial MRI had the advantages in demonstrating unilateral ischemic lesions at the cerebral peduncle and the thalamomesencephalic junction involving the unilateral riMLF on the right side. Recent reports have demonstrated that supranuclear vertical gaze palsy is caused by the unilateral riMLF lesion. We confirm that the unilateral riMLF lesion causes supranuclear vertical gaze palsy in our case and that cranial MRI has the advantages in demonstrating the specific lesion.
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PMID:[A case report of Weber's syndrome associated with supranuclear vertical gaze palsy caused by the ipsilateral thalamomesencephalic lesion]. 834 98

A 48-year-old male was admitted to our hospital because of fever, headache and vomiting. At admission, the level of consciousness was depressed (drowsy) with slight confusion. Extremely miotic pupils, nuchal stiffness, ataxia and myoclonic movements of both upper limbs were observed. The eye movements were almost normal and there was no definite limb weakness or sensory impairment. A few days after admission, his level of consciousness further decreased, and opsoclonus, ataxic breathing and intestinal paralysis appeared. The body temperature fluctuated remarkably ranging from 33.0 degrees C to 39.0 degrees C. The cerebrospinal fluid (CSF) examination revealed lymphocytic dominant pleocytosis, increase of protein and decrease of glucose. Enzyme-linked immunosorbent assay (ELISA) showed increased antibody (IgG) to herpes simplex virus (HSV) in both serum and CSF. The antibody in CSF further elevated at the later examination. Magnetic resonance imaging (MRI) demonstrated high signal intensity areas mainly in the cerebellum and sporadically in the supratentorial subcortical white matter on T2-weighted images. Administration of Gadolinium-DTPA also revealed an additional lesion in the pons. From these findings, he was diagnosed as herpetic encephalitis involving the brainstem and the cerebellum, and acyclovir was administered. Although his initial symptoms and signs started to recover three weeks after admission, he newly developed complete flaccid paraplegia, dysuria and sensory disturbance with the spinal cord level of the 4th thoracic segment. The oligoclonal IgG bands were detected in the cerebrospinal fluid of the convalescent stage.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Herpetic simplex encephalitis followed by myelopathy]. 836 51

The thoracic outlet syndromes encompass the diverse clinical entities affecting the branchial plexus or subclavian artery including cervical ribs or bands. Thoracic outlet syndrome are often difficult to diagnose on existing clinical and electrophysiological criteria and new diagnostic methods are necessary. This study reports our experience with magnetic resonance imaging (MRI) of the brachial plexus in 20 patients with suspected thoracic outlet syndrome. The distribution of pain and sensory disturbance varied widely, weakness and wasting usually affected C8/T1 innervated muscles, and electrophysiology showed combinations of reduced sensory nerve action potentials from the fourth and fifth digits, and prolonged F-responses or tendon reflex latencies. The MRI study was interpreted blind. Deviation of the brachial plexus was recorded in 19 out of the 24 symptomatic sides (sensitivity 79%). Absence of distortion was correctly identified in 14 out of 16 asymptomatic sides (specificity 87.5%). The false positive rate was 9.5%. Magnetic resonance imaging demonstrated all seven cervical ribs visible on plain cervical spine radiographs. Magnetic resonance imaging also showed a band-like structure extending from the C7 transverse process in 25 out of 33 sides; similar structures were detected in three out of 18 sides in control subjects. These MRI bands often underlay the brachial plexus distortion observed in our patients. We also observed instances of plexus distortion by post-traumatic callus of the first rib, and by a hypertrophied serratus anterior muscle. If they did not demonstrate a cervical rib, plain cervical spine radiographs had no value in predicting brachial plexus distortion. We believe MRI to be of potential value in the diagnosis of thoracic outlet syndrome by: (i) demonstrating deviation or distortion of nerves or blood vessels; (ii) suggesting the presence of radiographically invisible bands; (iii) disclosing other causes of thoracic outlet syndrome apart from ribs or bands.
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PMID:Thoracic outlet syndromes and magnetic resonance imaging. 760 97

Carpal tunnel syndrome is well known to be associated with hypothyroidism, but other mononeuropathies have been rarely reported. We report a 65-year-old male who showed right deep peroneal nerve palsy caused by hypothyroidism. The patient was admitted to our hospital because of general fatigue and right drop foot. On admission, bilateral pretibial pitting edema was observed, predominant on the right side. There was no muscle contraction in the right anterior tibial muscle and extensor hallucis longus in addition to slight weakness of the proximal muscles; whereas, muscle atrophy of the anterior tibial muscle was not noted. There was no sensory disturbance. On an electrophysiological examination, there was no muscle action potentials by the stimulation of the right deep peroneal nerve. Other nerves showed normal results both in the conduction velocity and in the compound action potential. Two months after the administration of levothyroxine sodium (0.025 mg/day), the right deep peroneal nerve palsy was completely recovered, associated with disappearance of pretibial edema. It is not yet determined which of axonopathy or demyelination is dominantly responsible for neuropathy associated with hypothyroidism. These results suggested a conduction block in deep peroneal nerve associated with focal edema. It is necessary to consider hypothyroidism as well as trauma, diabetes mellitus, and vasculitis when investigating mononeuropathy of deep peroneal nerve.
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PMID:[Deep peroneal nerve palsy associated with hypothyroidism]. 839 65

The patient was a 55-year-old man. He was well until eleven months earlier, when he noticed the onset of numbness in his right fingers. Over the next three months, the numbness spread up the right arm and involved the right leg, left arm and left leg in succession. Six months before admission, sensory impairment of the trunk appeared progressively. Mild diffuse muscle weakness occurred gradually. Swelling of the right lateral cervical lymph node was found just before admission. On neurological examination, sensory disturbance involving all modalities of sensation was recognized in the extremities and trunk. The sensory findings were asymmetric and predominant at the lower extremities and distal parts. A positive Romberg's sign and poor coordination was recognized. All the deep tendon reflexes were absent without pathological reflexes. Motor weakness, which was less severe than sensory findings, was present. There was no sphincter disturbance nor orthostatic hypotension. Routine laboratory findings were within normal limits. The protein level of cerebrospinal fluid was slightly increased. The electrophysiological studies and histological evaluation of the biopsied peroneal muscle and sural nerve showed degeneration and reduction of large myelinated fibers of sensory nerves with minimal motor nerve changes. Histological study also revealed the increase of regenerating fibers. Squamous cell carcinoma of epipharynx with metastasis of neck lymph nodes was found. Radiotherapy without chemotherapy resulted in a complete tumor remission and probably suppressed the progression of neurological disturbances. After two years from the onset, diffuse high intensity area was noted in the posterior spinal column on T2 weighted MRI.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of carcinomatous neuropathy with degeneration of posterior spinal column on MRI]. 839 18

A 76-yr-old male patient with carcinomatous neuropathy associated with hepatic cell carcinoma, whose initial symptom was deep sensory disturbance followed by muscle weakness is described. The onset was subacute, followed by slow progression. Sural nerve biopsy, as well as electrophysiological examinations, revealed severe axonal degeneration without any evidence of demyelination. The autopsy findings were similar to findings described in the literature on carcinomatous neuropathy. Although carcinomatous neuropathy is usually associated with lung cancer, this report describes an association with hepatic cell carcinoma. The patient also had motor nerve involvement with positive serum anti-GM1 ganglioside antibody which decreased after immunosuppressant therapy in parallel with recovery of muscle weakness. The anti-GM1 ganglioside antibody may be involved in the pathogenesis of motor disturbance in the present case.
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PMID:Carcinomatous neuropathy associated with hepatic cell carcinoma: an autopsy case report. 840 Aug 64

We report a 19-year-old girl with multiple sclerosis (MS) who had chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). At the age of 9 (1981), she was diagnosed as having Devic disease, including optic neuritis and transverse myelitis. For two years after the onset of the disease, she suffered from three relapses of MS and was treated with prednisone. At the age of 11, she noticed muscle weakness and sensory disturbance of four limbs. On examination, all tendon reflexes were absent, CSF protein was elevated, and motor nerve conduction velocity of the ulnar nerves was markedly slowed. Sural nerve biopsy showed decreased density of myelinated fibers and de-re-myelinated fibers with onion-bulb formations. Between 11 and 19 years, she had a number of episodes of the central nervous system (CNS) and peripheral nervous system (PNS) deficits with partial or complete recovery by oral administration of prednisone. The clinical course and laboratory data are consistent with MS associated with CIDP. Such our case of MS with CIDP in childhood is very rare. Patients with MS and CIDP may represent a subpopulation who had a common pathogenetic factor for both CNS and PNS demyelination.
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PMID:[A case of multiple sclerosis associated with chronic inflammatory demyelinating polyradiculoneuropathy]. 841 99

Multiple spinal meningiomas are relatively rare and account for from only 2 to 3.5% of all spinal meningiomas. Two cases of multiple meningiomas of the thoracic spinal cord were reported. Case 1. A 73-year-old woman was admitted with a 5 month history of progressive motor weakness and sensory disturbance in the legs bilaterally. Neurological findings on admission revealed paraparesis, hypesthesia and hypalgesia under the Th10 level, hyperreflexia of both legs, and urinary incontinence. Myelography and MRI revealed two intradural extramedullary lesions at the Th7 and Th10 level. Both tumors were removed completely. Histopathological examination showed psammomatous meningiomas. Case 2. A 52-year-old man was admitted with a 2 month history of progressive motor weakness and numbness of both legs. Neurological findings on admission revealed paraparesis, hypesthesia and hypalgesia under the Th10 level, hyperreflexia of both legs, and genitourinary incontinence. Lumbar myelography showed complete block at the Th9 level. MRI showed stenosis of the spinal canal at the Th8/9 level, and a deviation of the spinal cord. MRI with Gd-DTPA showed that the spinal cord was compressed by intradural extramedullary tumors. However, myelography and MRI could not detect the multiplicity of tumors. CT myelography demonstrated three separate tumors from the Th7 to the Th9. Three tumors were totally removed. Histopathologically, they were meningotheliomatous meningiomas. These cases were considered as being multiple meningiomas of the spine. Both patients showed no manifestations of von Recklinghausen's disease. The cause of the multiplicity in these cases was uncertain. Multiple spinal tumor is very difficult to diagnose because of unusual clinical symptoms.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Multiple meningiomas of thoracic spinal cord: report of two cases]. 842 95

The aim of this study is to clarify the clinicopathological characteristics of the multisystem degeneration seen in two male siblings with familial amyotrophic lateral sclerosis (FALS). A similar neurological disorder affected their elder sister and paternal uncle, but not their parents. The older brother (case 1) developed muscular weakness at 50 years of age and the younger brother (case 2), at 42 years of age. The duration of illness was 19 months in case 1 and 31 months in case 2. The clinical picture was the common (suspended) form in case 1 and the pseudopolyneuritic form in case 2. Pyramidal tract sign was obscure in both cases and cerebellar sign, sensory disturbance, sphincter disturbance and oculomotor palsy were not observed in either case. Neuropathological examination revealed similar findings in the two cases: 1) marked loss of lower motor neurons in the spinal anterior horn and motor nuclei of the lower brain stem in both cases, with neuronal loss of Onuf's nuclei in case 2; 2) very mild involvement in Clarke's nuclei, the dorsal and ventral spinocerebellar tracts and the middle root zone of the posterior column; 3) relatively well preserved Betz cells in the upper motor cortex with the appearance of a few macrophages, and mild changes in the pyramidal tract of the spinal cord; and 4) mild degenerative changes in the pallidoluysian system and the dentatorubral system. The most characteristic pathological findings common to both cases were the extremely mild involvement of the middle root zone of the posterior column, Clarke's nuclei and spinocerebellar tracts. The pattern of lower motor neuron system degeneration paralleled the development of clinical features. Genetic studies demonstrated no mutations in exons 1, 2 and 4 of Cu/Zn-binding superoxide dismutase gene. We emphasized the existence of mild involvement of middle root zone of posterior column, Clarke's nuclei and spinocerebellar tract in FALS with multisystemic degeneration.
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PMID:[Two siblings of familial amyotrophic lateral sclerosis with multisystemic degeneration characterized by mild involvement of the middle root zone of the posterior column, Clarke's nuclei and spinocerebellar tract]. 852 32

A 74-year-old man developed proximal muscular weakness and wasting of the left upper extremity without sensory disturbance or myelopathic symptoms. The muscle atrophy had not progressed for a few years. Radiological examination of the spine showed cervical disc herniation. These findings and electrophysiological studies excluded motor neuron diseases, permitting the diagnosis of dissociated motor loss syndrome. Interestingly, delayed computerized tomographic myelography disclosed cavities in the anterior horns of the spinal cord, which coincided with the clinical symptoms. Previous radiological and pathological examinations showed formation of such cavities within the spinal cord resulting from chronic compression, which was followed by ischemic change. In this context, the present case supports ischemia as a cause of dissociated motor loss syndrome.
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PMID:Dissociated motor loss syndrome with cavities in the anterior horns. 856 84


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