Cases reported "Muscle Weakness"

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1/159. Cervicomedullary astrocytoma simulating a neuromuscular disorder.

    A 12-year-old male developed progressive proximal upper extremity weakness over a 3- to 4-year period. The clinical findings of proximal upper extremity weakness and atrophy, prominent scapular winging, and no sensory deficits or upper motor neuron signs suggested a neuromuscular disorder. electromyography was consistent with a chronic denervating disorder involving the upper cervical anterior horn cells or their axons. A cervical magnetic resonance image revealed a large intramedullary mass extending from the inferior aspect of the fourth ventricle down to the level of T2. A biopsy of the lesion was consistent with a low-grade astrocytoma.
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2/159. Spinal arachnoid cyst with weakness in the limbs and abdominal pain.

    A 7-year-old male admitted with neck rigidity, severe pain in the abdomen, and progressive weakness in the lower limbs was diagnosed as having a spinal intramedullary arachnoid cyst. There was a dramatic and immediate recovery after fenestration of the cyst.
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3/159. An uncommon mechanism of brachial plexus injury. A case report.

    PURPOSE: To report a case of brachial plexus injury occurring on the contralateral side in a patient undergoing surgery for acoustic neuroma through translabrynthine approach. CLINICAL FEATURES: A 51-yr-old woman underwent surgery for acoustic neuroma through translabrynthine approach in the left retroauricular area. She had a short neck with a BMI of 32. Under anesthesia, she was placed in supine position with Sugita pins for head fixation. The head was turned 45 degrees to the right side and the neck was slightly flexed for access to the left retroauricular area, with both arms tucked by the side of the body. Postoperatively, she developed weakness in the right upper extremity comparable with palsy of the upper trunk of the brachial plexus. hematoma at the right internal jugular vein cannulation site was ruled out by CAT scan and MRI. The only remarkable finding was considerable swelling of the right sternocleidomastoid and scalene muscle group, with some retropharyngeal edema. An EMG confirmed neuropraxia of the upper trunk of brachial plexus. She made a complete recovery of sensory and motor power in the affected limb over the next three months with conservative treatment and physiotherapy. CONCLUSIONS: brachial plexus injury is still seen during anesthesia despite the awareness about its etiology. Malpositioning of the neck during prolonged surgery could lead to compression of scalene muscles and venous drainage impedance. The resultant swelling in the structures surrounding the brachial plexus may result in a severe compression.
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4/159. Autosomal dominant distal myopathy not linked to the known distal myopathy loci.

    The distal myopathies are clinically, pathologically and genetically heterogenous. Thus far, seven types of distal myopathy have been linked to four chromosome loci. We recently examined four affected members from three generations of an autosomal dominant distal myopathy kindred. A muscle biopsy was performed on the index case. Muscle histopathology showed non-specific myopathic findings including increased variation in fiber size and increased internalized nuclei. No abnormal inclusions or vacuoles were present. Microsatellite markers for the four distal myopathy loci on chromosomes 2, 9 and 14 were studied on affected and several unaffected family members. Affected patients developed distal weakness in anterior foreleg muscles followed by progressive distal upper and proximal lower extremity involvement. Chromosome 2, 9 and 14 regional markers were informative and demonstrated recombinations with affected individuals in the pedigree. The resulting LOD scores obtained from the multipoint analyses gave no evidence of positive linkage to any of the regions and positively excluded (lod score less than -2) all, or virtually all, of the candidate regions examined. This autosomal dominant distal myopathy family does not show evidence of linkage to any of the known distal myopathy loci, suggesting the existence of at least one more distal myopathy locus. Furthermore, the clinical and pathological features appear distinct from other previously described but genetically-undetermined autosomal dominant distal myopathies.
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5/159. An unusual manifestation of diabetes mellitus.

    MEDICAL history: Type 2 diabetes mellitus for five years; unexplained 35-lb weight loss three years ago; Bell's palsy on right side many years ago. MEDICATIONS: glipizide, 10 mg/day. family history: Father died of leukemia at age 65; mother has kidney stones; no diabetes or neuromuscular disease. SOCIAL history: insurance salesman; heterosexual, promiscuous, uses condoms; smokes (25 pack years); does not drink. physical examination: Well-nourished, well developed, not in acute distress; had difficulty rising from a sitting position because of right lower extremity weakness. blood pressure, 154/74; pulse, 88; temperature, 36.6 degrees C; respiratory rate, 16. head, eyes, ears, nose, and throat: normal. neck: normal. heart: S4. Lungs: clear. abdomen: mildly obese. extremities: no cyanosis, clubbing, or edema; atrophy and weakness of right thigh and both calves; wide-based gait; able to walk on toes but not heels. Neurologic responses: cranial nerves intact; deep tendon reflexes, 1 symmetrically; plantar reflexes, flexor bilaterally. skin: macular rash in sun-exposed areas. LABORATORY FINDINGS: Hemoglobin, 13.2 gm/dL; mean corpuscular volume, 80 micron 3; white blood cell count, 7,200/mm3 (normal differential); platelet count, 137,000/mm3. serum: electrolytes, normal; blood urea nitrogen, 18 mg/dL; creatinine, 0.8 mg/dL; glucose, 308 mg/dL; total protein, albumin, liver enzymes, and creatine kinase, normal. urine: 1 glucose. Venereal disease test: nonreactive; hiv test: negative. DIFFERENTIAL diagnosis: dermatomyositis; heavy-metal poisoning; diabetic amyotrophy. HOSPITAL COURSE: The patient was given 50 mg/day of oral amitriptyline to alleviate the painful paresthesias and was switched to 20 U/day of subcutaneously injected neutral protamine Hagedorn (NPH) insulin to normalize the blood glucose level. Histologic studies of skin and muscle showed sun damage and neuropathic changes, respectively. There was no evidence of vasculitis. Screening for heavy-metal toxins produced negative results.
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6/159. Distal neuralgic amyotrophy.

    Neuralgic amyotrophy consists of severe pain around the shoulder and arm followed by weakness in one or several muscles of the same area. We describe four patients with distal neuralgic amyotrophy in whom acute, severe, and transient pain around the shoulder or arm was followed by weakness of the forearm and hand muscles only. Minor sensory symptoms were present in only one patient. The presence of structural lesions causing the extent of the forearm and hand motor deficit was excluded by ancillary examinations. Electrophysiological studies showed a motor axonopathy and minimal sensory axonopathy. A follow-up of 2 years or longer showed either spontaneous improvement or residual motor deficit. Unfamiliarity with a clinically distal localization of neuralgic amyotrophy may result in misdiagnosis of lower cervical (poly)radiculopathy in view of the distal localization of the motor deficit and the high prevalence of coincidental abnormalities of the lower cervical spine on plain radiography, computed tomography, or magnetic resonance imaging.
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7/159. Mitochondrial 3243 A-->G mutation (MELAS mutation) associated with painful muscle stiffness.

    The mitochondrial mutation A-->G at nucleotide position 3243 is associated with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) and other mitochondrial encephalomyopathies. We found this mutation in a 61-year-old patient who developed at the age of 54 a myopathy with painful muscle stiffness as the predominant symptom. Additionally hypacusis, a mild hemisensory syndrome and impaired glucose tolerance were present. Muscle histopathology showed few ragged red fibers. The mutation was detected heteroplasmatically in dna from muscle and blood. So far painful muscle stiffness has not been a known phenotype of the 3243 mutation.
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8/159. hypereosinophilic syndrome: progression of peripheral neuropathy despite controlled eosinophil levels.

    Idiopathic hypereosinophilic syndrome (HES) is a disorder of the hematopoietic system, characterized by persistent elevation in the total eosinophil count (> 1500/microliter) for over 6 months, associated with organ damage and no detectable underlying cause. Treatment is centered on the reduction of total circulating eosinophils, which generally leads to remission of symptoms. We report a 68-year-old female patient with HES and peripheral neuropathy, presenting with cutaneous lesions, mental changes, cardiac and pulmonary symptoms, followed by right foot drop and eventually paraparesis, which caused an inability to ambulate. Weakness progressed to include the upper extremities despite adequate control of eosinophilia by steroids. Worsening of the peripheral neuropathy can occur despite lowering of the eosinophil levels.
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9/159. Ipsilateral facial weakness in upper medullary infarction-supranuclear or infranuclear origin?

    We describe two patients with upper medullary infarctions showing ipsilateral facial weakness and relative sparing of the upper facial muscles. Electrophysiological follow-up using transcranial magnetic stimulation of the motor cortex in combination with stimulation of the peripheral facial nerve disclosed a supranuclear (corticofacial) tract lesion in one patient and a partial nuclear/infranuclear intra-axial facial nerve lesion in another.
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10/159. Adhesive capsulitis of the glenohumeral joint with an unusual neuropathic presentation: a case report.

    A 37-yr-old woman presented with a 7-mo history of unilateral shoulder girdle stiffness, pain, and weakness and had already been diagnosed with frozen shoulder. physical examination revealed scapular winging and suspicious focal paralysis of shoulder girdle muscles. Subsequently, electrodiagnostic studies reported denervation of deltoid, infraspinatus, serratus anterior, and lower cervical paraspinal muscles, in addition to a prolonged long thoracic nerve latency. The history, physical examination, and cervical magnetic resonance imaging scan seemed most consistent with neuralgic amyotrophy, although the electrodiagnostic examination could be interpreted as cervical radiculopathy. Some of the difficulties in identifying neuralgic amyotrophy and distinguishing it from cervical radiculopathy are discussed herein. Historically, frozen shoulder has seemed to develop as a complication of the neuropathic process. Both neuralgic amyotrophy and frozen shoulder have a poorly understood pathogenesis, and their combined presence is presumed to be rare. Because of difficulties inherent in the physical examination of frozen shoulder, a coexistent neuropathic process may go undetected.
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