Cases reported "Muscle Weakness"

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1/8. giant cell arteritis of the cervical radicular vessels presenting with diaphragmatic weakness.

    The clinical and histopathological details of a patient who succumbed to giant cell arteritis (GCA) of the cervical radicular vessels are described. The initial clinical presentation, with diaphragmatic weakness, has not previously been reported. Normal inflammatory indices and the unusual presentation prevented diagnosis during life, but GCA should be considered in the differential diagnosis of any unexplained neuropathic or radiculopathic syndrome, as corticosteroid therapy may lead to recovery. This is the first account of the pathological findings in cervical radiculopathy associated with GCA.
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2/8. Amyloid myopathy presenting with distal atrophic weakness.

    Amyloid myopathy is a rare complication of primary amyloidosis usually presenting with proximal muscle weakness. We report a woman with multiple myeloma in whom marked atrophy and weakness of finger flexor muscles were the first manifestations of systemic amyloidosis. Muscle biopsy revealed amyloid angiopathy with deposits of lambda light chains in vessel walls. The recognition of amyloid myopathy is important because clinical symptoms may respond to chemotherapy.
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3/8. Amyloid myopathy: characteristic features of a still underdiagnosed disease.

    A 62-year-old man with progressive proximal weakness underwent extensive evaluation including muscle biopsy without a clear diagnosis being established. A repeat muscle biopsy including congo red-stained sections revealed infiltration of blood-vessel walls and endomysium with amyloid protein, as well as an unusual pattern of pathologic changes to muscle fibers. From a review of 79 cases of amyloid myopathy reported in the English-language literature, the characteristic features of this disorder are described. congo red-stained sections of muscle biopsy viewed under fluorescent or polarized optics, and serum or urine protein immunoelectrophoresis, play an important role in the evaluation of myopathy. Amyloid myopathy should be a consideration in adults with progressive neuromuscular weakness of uncertain cause.
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4/8. Lumbar hemorrhagic synovial cysts: diagnosis, pathogenesis, and treatment. Report of 3 cases.

    BACKGROUND: To define the etiologic, clinical, histological, and surgical features of lumbar hemorrhagic synovial cysts (LHSCs). Three personal cases are reported together with a review of the pertinent literature. methods: We identified 3 cases of LHSC treated in our departments and 20 cases culled from the literature. RESULTS: A total of 23 cases of LHSC were selected. All the patients underwent surgical treatment because of untreatable radicular pain and/or neurological deficits. The amount of bleeding, either massive or minor but repeated, influenced the timing of surgery. In our cases, the histological examinations showed an inflammatory reaction within the cyst and the consequent formation of neoangiogenic vessels. CONCLUSIONS: Hemorrhagic synovial cyst of the spine is rare and its most common localization is lumbar. Bleeding within the cyst leads to an increase of its volume, accompanied by neurological deficits and/or painful symptoms that are violent and generally intractable. In this event, surgical excision is the treatment of choice and, in some cases, emergency surgery is necessary. Hemorrhages are probably caused by the rupture of fragile neoangiogenic vessels.
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5/8. delayed diagnosis of spinal dural arteriovenous fistula in the absence of pathological vessels on MRI.

    The authors report on a 69-year-old man presenting with progressive leg weakness and gait ataxia over two years. A central intramedullary cord lesion ranging from T8-12 on MR imaging was misdiagnosed as a low-grade glioma and a biopsy was attempted followed by temporary clinical deterioration. Selective spinal angiography revealed a spinal dural arteriovenous (AV) fistula on the left L3 nerve root sheath despite the absence of pathological vessels on MR imaging. The fistula was successfully treated by microsurgical interruption of the arterialized intradural vein. The present case should remind us to include selective spinal angiography in our diagnostic work-up in patients predisposed for spinal dural AV fistula by male sex, advanced age and clinical presentation of slowly progressive sensorimotor symptoms with myelopathy on MR imaging, even in the absence of any pathological vascular structures.
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6/8. Amyloid myopathy presenting with rhabdomyolysis: evidence of complement activation.

    At age of 57 years, a man experienced an episode of rhabdomyolysis. On that occasion muscle biopsy was not performed, however monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. Further he developed a moderate proximal muscle weakness with CK level persistently elevated (1000-1200U/l). When he came to our observation, at age 67, a muscle biopsy revealed an amyloid myopathy and multiple myeloma was at the same time disclosed. Terminal complement complex C5b9 (membrane attack complex) deposits were found in the vessel walls and muscle fibers surface depicted by amyloid. Our case suggests to keep in mind the possibility that amyloid myopathy may begin as an isolate episode of rhabdomyolysis. The detection of complement complex C5b9 suggests that complement cascade is implicated in the muscular damage of amyloid myopathy.
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7/8. radiation myelopathy: a clinicopathological study with special reference to correlation between MRI findings and neuropathology.

    We describe magnetic resonance imaging (MRI) and neuropathological findings in a patient with chronic progressive radiation myelopathy (CPRM). An 81-year-old man with esophageal cancer underwent radiotherapy. Four years later he developed a progressive neurological deficit below the irradiated level of the spinal cord. Neurological examination revealed spastic paraplegia. MRI findings showed an area of high signal intensity on T2-weighted images of the thoracic spinal cord. On the basis of clinical and MRI findings, we diagnosed his condition as CPRM. MRI performed thirteen months after onset of neurological signs revealed mild atrophy of the spinal cord detected on T1-weighted images and an area of high signal intensity within the spinal cord detected on T2-weighted images. Neuropathological examination revealed findings consistent with radiation myelopathy. We speculate that the area of high signal intensity within the spinal cord detected on T2-weighted images might be a result of proliferation of small vessels, which was discovered upon autopsy.
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8/8. Intravascular malignant lymphomatosis with neurologic presentation: factors facilitating antemortem diagnosis.

    Intravascular malignant lymphomatosis (IML) is a rare disorder of small and medium size vessels that frequently goes undiagnosed until the time of autopsy. The clinical courses of two such patients were examined to determine factors that would facilitate antemortem diagnosis. Both patients had mental status changes, pyramidal tract signs, and peripheral neuropathy. Despite postmortem evidence of widespread lymphocytic invasion of vessels throughout the body including peripheral and central nervous systems, neuroimaging studies, cerebrospinal fluid analysis, peripheral blood studies, and bone marrow biopsy failed to reveal diagnostic evidence of the underlying neoplastic process. Although markedly abnormal, nerve conduction studies were nonspecific. Familiarity with IML and its consideration in the differential diagnosis when central and peripheral nervous system dysfunction occur concurrently may guide the physician to tissue biopsy facilitating antemortem diagnosis and institution of appropriate therapy.
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