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)

Sixteen cases of juvenile, non-progressive muscular atrophy localized in the hand and forearm were seen at our neurology out-patient clinic for the past 8 years. The analyses of these 16 cases disclosed characteristic features as follows: 1) juvenile onset 2) male preponderance 3) unique distribution of muscular atrophy and weakness in the hand and forearm. 4) insidious onset, initial progressive period and subsequent non-progressive course 5) tendon reflexes of the arms are hypoactive in half of the cases 6) no pathological reflexes 7) cold paresis 8) no definite sensory disturbance 9) no cranial nerve involvement 10) neurogenic patterns on EMG According to these features, this clinical entity carrying good prognosis must be differentiated from several diseases associated with similar muscular atrophy of extremities, especially amyotrophic lateral sclerosis which is notorious as a fetal disease.
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PMID:[Juvenile, non-progressive muscular atrophy localized in the hand and forearm. Report of 16 cases (author's transl)]. 54 13

Three cases of tethered cord syndrome are presented with reference of reported literatures. This syndrome is usually associated with spina bifida occulta and cutaneous manifestation such as subcutaneous tumor, skin dimple, hairy patch, and neavus. Presented symptoms are various progressive neurological deficits involving weakness and/or sensory disturbance of lower limbs, bowel and/or bladder dysfunction, leg and/or low back pain, and orthopedic pathological conditions such as scoliosis and/or foot deformities. Properly performed myelography is the only method of preoperative confirmation of this syndrome. As the surgical release of the tethered spinal cord is effective for these various symptoms except for foot deformity, early investigation and early surgery are recommended.
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PMID:[Tethered cord syndrome--our three cases and the review of the literature (author's transl)]. 66 74

A patient with hypokalemic myopathy occurring in the context of chronic alcoholism was reported. A 56-year-old male patient, heavy drinker for 20 years, complained of marked weakness and acutely developing pains in his limbs. The principal clinical findings were weakness and tenderness of the proximal limbs and girdle muscle. He was unable to lift his head or any extremities from the bed. Deep tendon reflexes were diminished, but not absent. There was no sensory disturbance except for muscle tenderness. These clinical manifestations disappeared gradually by abstinence from drinking, and potassium administration therapy, and the patient recovered completely on the 26th day after onset. On the day after admission (8th day), serum potassium value was 2.2 mEq/L, and serum CPK activity was 4270 IU. The ECG pattern was consistent with a diagnosis of low potassium content in serum, and the EMG pattern was consistent with a diagnosis of myopathy. These electrophysiological findings had a tendency to recover from this pattern to normal range correspondingly with clinical improvement. The repeated muscle biopsies showed that vacuolation, hyaline degeneration and significant phagocyte infiltration were observed in the muscle on the 9th day after the onset of muscle weakness, and that these pathological findings disappeared almost completely three weeks later. The frequently repeated examinations of potassium content and CPK activity in sera showed that there was a close correlation between these biochemical abnormalities and clinical improvement. The pathogenesis of alcoholic myopathy and significance of CPK abnormality in chronic alcoholism were discussed.
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PMID:Hypokalemic myopathy due to chronic alcoholism. 102 45

A 68-year-old man was admitted because of sudden onset gait disturbance and bradykinesia. He experienced left putaminal bleeding following mild right hemiparesis and emotional incontinence without any difficulties in his daily life since three years before admission. On neurological examination the patient was alert and oriented. He showed forced crying. Myerson's sign was positive. There were no abnormal findings in ocular movements, pupillary reflexes and other cranial nerves. Muscle tone was increased with cog-wheel phenomenon in bilateral upper extremities. Coordination was preserved. He showed severe akinesia and small steppage gait with stooped posture. Freezing phenomenon was observed in initiation of gait and turning. The deep tendon reflexes were increased in the right side with bilateral pathological reflexes. There was no definite weakness and sensory disturbance in all extremities. Brain CT revealed a small high density lesion in the medial side of right cerebral peduncle and a lens-shaped low density lesion in the left putamen. On T1 and T2 weighted images of MRI, right peduncular lesion showed low signal. It extended to the substantia nigra which was partially destructed. His parkinsonism was rapidly improved and completely disappeared within following two weeks. High density lesion of right peduncle on CT also disappeared. We discussed the mechanisms of parkinsonism following unilateral mesencephalic hemorrhage in this patient.
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PMID:[A case of transient parkinsonism due to mesencephalic hemorrhage]. 129 Nov 68

Migrant sensory neuritis, which was first proposed by Wartenberg, is very uncommon and only a few case reports have ever been published. We described one case of migrant sensory neuritis and discussed the pathogenesis of this disease. A 44-year-old man noticed numbness in the lateral aspect of the dorsum of the left foot in February 1985. Physically, there was hypoesthesia in the region of left sural nerve with positive Tinel's sign. During the next 4 years, pain, tingling sensation or hypoesthesia appeared in the regions of the right superficial radial nerve, right axillary nerve, left intercostal nerve, left lateral plantar nerve, digital nerve of the right second digit, left saphenous nerve, right superficial peroneal nerve, left superficial radial nerve, bilateral ulnar nerves and bilateral median antebrachial cutaneous nerves one after another in a migrating fashion. Tinel's sign was also positive at the right superficial radial nerve. In some occasions, decreased deep tendon reflexes were observed, but there had been no muscle weakness. Some nerves showed complete recovery, but others showed persistent involvement. Some nerves were affected repeatedly. Laboratory examination failed to clarify underlying disease except for mild liver dysfunction. Electrophysiological study showed reduced amplitude of the sensory nerve action potential (SNAP) of right sural nerve, left ulnar nerve, right superficial radial nerve and digital branch of right median nerve with preserved sensory nerve conduction velocity. SNAP of left sural nerve was absent. These findings mean the cause of the sensory disturbance is axonal degeneration rather than segmental demyelination. There were no abnormalities in motor nerve conduction study.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Migrant sensory neuritis--electrophysiological and pathological study]. 129 55

A 56-year-old woman noticed non-progressive weakness in the proximal part of the right upper extremity from her childhood. At the age of 37 years, she was diagnosed as "spinal muscular atrophy" by an orthopedic surgeon. At the age of 56, neurological examinations revealed muscular atrophy and weakness confined to the right deltoid, biceps brachii and brachioradialis together with minor sensory disturbance in the lateral side of the right shoulder and forearm without pyramidal sign in the lower extremities. The neurological features of this case differed from those of juvenile type of distal and segmental muscular atrophy of upper extremities in distribution of muscular atrophy, and simulated those of cervical spondylotic amyotrophy. Myelography demonstrated root avulsion of the right C5 and C6 roots. CT myelography revealed traumatic meningocele. Therefore a clinical diagnosis of cervical root avulsion resulting from unrecognized birth injury was made. The reason of motor dominant pictures of this case may be vulnerability of the anterior nerve roots to traction injury.
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PMID:[Cervical root avulsion presenting proximal segmental muscular atrophy of unilateral upper extremity]. 130 31

An 82-year-old woman without previous medical problem noticed vague back pain on December 31, 1989, and was admitted to a hospital because she developed a fever, a rapidly progressive weakness followed by anesthesia of the lower extremities and sphincter disturbance. On myelography and myelo-CT, the spinal cord appeared to be displaced by an extramedullary mass which partially blocked the subdural space at the level of T-9 to L-1. When transferred to our hospital on January 8, 1990, she was febrile and complaining of headache with meningeal signs. Percussion tenderness was present at T-8 to L-1 spinal spinous process. Neurological examination revealed that the patient had mild consciousness clouding, total paraplegia in the legs, sensory disturbance of a partial degree at L-1 to L-3 and totally below L-3, brisk but equal tendon reflexes in the upper extremities, areflexia in the legs with positive bilateral Babinski signs and sphincter disturbance. Otherwise she was neurologically unremarkable. Acute inflammatory reactions were prominent among the laboratory findings on admission. A lumbar tap yielded purulent fluid with more than 170,000 cells/mm3, 5,000 mg/dl of protein, 44 mg/dl of glucose and culture of the fluid isolated Escherichia coli. T1-weighted sagittal MRI disclosed an ill defined mass which showed the same or locally higher with gadopentetate dimeglumine (Gd-DTPA) signal intensity as soft tissue, compressing the spinal cord anteriorly from T-7 to L-3. The lesion was noticed to have a more extensive rostral-caudal extent than was inferred from myelography and myelo-CT.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[An autopsy case of spinal subdural abscess in the aged--comparative study with neuroradiological findings]. 135 3

A 50-year-old woman was admitted to our hospital because of abnormal involuntary movement of upper abdomen. Three months before admission, she had suffered from left lateral chest pain without skin lesions for one week. The neurological examination on admission revealed myoclonus of upper abdomen, and hyperalgesia and thermohyperesthesia from T4 to T9. There was no weakness, the tendon reflexes were symmetrical and the plantar responses were flexor. The surface EMG disclosed the symmetrical, synchronous contractions of m. rectus abdominis and m. obliques externus abdominis. This spinal myoclonus reduced during sleep. The EEG, CT and MRI showed no abnormalities. Serum varicella-zoster virus (VZV) titers increased significantly on follow-up examinations. Clonazepam, 1.5 mg daily was effective in this patient. The myoclonus spontaneously disappeared without clonazepam in six weeks after onset, and at the same time the sensory disturbance also improved. From the neurological findings and clinical course, we consider this spinal myoclonus was probably elicited by involvement of the inhibitory interneurons of the dorsal horns, due to immune response to latent VZV infection but not to direct neuronal destruction by VZV. Spinal myoclonus should be recognized as the spectrum of neurological disease associated with VZV even in the absence of skin lesions.
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PMID:[Varicella-zoster virus-associated spinal myoclonus without skin lesions]. 139 36

This 60-year-old man had been well until four years prior to admission, when he developed slowly progressive weakness of the lower extremities. On admission he was found to have a spastic paraparesis, sensory disturbance below the level of T10 and mild sphincter dysfunction. Conventional myelography and CT myelography demonstrated an epidural mass located from T5 to T8 vertebral level. MRI revealed that the epidural mass was fusiform and markedly enhanced by the contrast medium. Laminectomy was performed and a fibrous tumor red in color was subtotally removed. Histological findings were consistent with those of angiolipoma. Angiolipoma is a rare tumor in the central nervous system and is regarded as a clinical entity different from a spinal lipoma. Occurrence of angiolipoma in the central nervous system has been reported in 40 cases in the world literature. Thirty nine cases of them occurred in the spinal canal, especially in the dorsal epidural space of the thoracic region. The fusiform shape demonstrated in this case might also be characteristic of angiolipoma in this region.
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PMID:[Spinal extradural angiolipoma: a case report]. 140 44

This paper presented a case of a right-handed male who showed a right hemiplegia without aphasia and apraxia. He lost the ability to write with the left hand. A 56-year-old right-handed man, who had a daughter of left-handedness, was sent to our hospital with a homonymous hemianopsia, facial weakness, spastic hemiparesis and sensory disturbance in the right side. CT scan revealed an infarction in the territory of the left middle cerebral artery. On a month after the onset, he was alert and oriented. His speech was normal and verbal comprehension was intact. Although he neglected the right side of the page, he could read and comprehend it correctly. In contrast with his normal abilities to speak, comprehend, and read, difficulties in writing were prominent. Spontaneous writing with the left hand was extremely poor, and he even had difficulty writing his own name. His dictation was also poor, but his writing improved with copying letters. Agraphia had seen even after USN was recovered. Analysis of this case suggested the presence of the dominance for speech, comprehension, and praxis in the intact right hemisphere, and writing center in the damaged left hemisphere.
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PMID:["Left unilateral agraphia with right hemiparesis" after occlusion of the left middle cerebral artery]. 141 44


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