Cases reported "Muscular Atrophy"

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1/7. Acute isolated suprascapular nerve palsy limited to the infraspinatus muscle: a case report.

    Suprascapular nerve palsy resulting in isolated weakness of the infraspinatus muscle is subtle at its onset and must be differentiated from cervical radiculopathy or bone and joint diseases of the shoulder, especially in the presence of pain. The literature suggests an association of cumulative trauma with ganglionic cyst formation and entrapment neuropathy of the suprascapular nerve in the spinoglenoid notch. Here we present clinical, electrodiagnostic, and radiologic features in a young patient who presented with a 2-week history of isolated, painless weakness of the right shoulder. His electromyography showed acute denervation of the right infraspinatus muscle. magnetic resonance imaging revealed a ganglionic cyst in the spinoglenoid fossa and edema in the infraspinatus muscle consistent with denervation. The patient recovered after removal of the cyst.
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2/7. Neuronal intranuclear hyaline inclusion disease showing motor-sensory and autonomic neuropathy.

    BACKGROUND: Neuronal intranuclear hyaline inclusion disease (NIHID), a rare neurodegenerative disease in which eosinophilic intranuclear inclusions develop mainly in neurons, has not yet been described to present as hereditary motor-sensory and autonomic neuropathy. methods: patients in two NIHID families showing peripheral neuropathy were evaluated clinically, electrophysiologically, and histopathologically. RESULTS: In both families, patients had severe muscle atrophy and weakness in limbs, limb girdle, and face; sensory impairment in the distal limbs; dysphagia, episodic intestinal pseudoobstruction with vomiting attacks; and urinary and fecal incontinence. No patients developed symptoms suggesting CNS involvement. Electrophysiologic study showed the reduced motor and sensory nerve conduction velocities and amplitudes, and also extensive denervation potentials. In sural nerve specimens, numbers of myelinated and unmyelinated fibers were decreased. In two autopsy cases, eosinophilic intranuclear inclusions were widespread, particularly in sympathetic and myenteric ganglion neurons, dorsal root ganglion neurons, and spinal motor neurons. These neurons also were decreased in number. CONCLUSION: patients with neuronal intranuclear hyaline inclusion disease (NIHID) can manifest symptoms limited to those of peripheral neuropathy. NIHID therefore is part of the differential diagnosis of hereditary motor-sensory neuropathy associated with autonomic symptoms. Intranuclear hyaline inclusions in schwann cells and in the myenteric plexus may permit antemortem diagnosis of NIHID.
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3/7. Hereditary motor and sensory neuropathy type II. Clinicopathological study of a family.

    A family with hereditary motor and sensory neuropathy (HMSN) type II is described in which 10 affected and 17 unaffected members in three generations were examined. The peak age of onset was in the second decade. In the youngest generation, the proportion of affected to unaffected individuals at risk significantly differed from the expected 50%. There was slight slowing of conduction velocities in 36% of nerves; however, only 3 out of 10 affected members had entirely normal conduction studies. The amplitude of the sensory potentials of median and peroneal nerves was almost uniformly reduced. In all affected patients electromyography of anterior tibial muscles showed signs of neurogenic involvement. Histological study of two sural nerves and a sciatic nerve and its branches revealed loss of myelinated fibres with a proximal-to-distal gradient in this fibre loss, clusters of small regenerating fibres, and atrophic axons. Postmortem study of the proband showed loss of anterior horn and dorsal root ganglion neurons in the lumbar and sacral segments and degeneration of the fasciculus gracilis. Morphometric evaluation of L5 ventral and dorsal roots revealed a normal number of myelinated fibres, diameter histograms being shifted to the left because of a significant loss of large myelinated fibres and regeneration. These anatomical findings are consistent with the hypothesis that HMSN type II represents a primary neuronopathy affecting motor and sensory neurons.
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4/7. Painless infraspinatus atrophy due to suprascapular nerve entrapment.

    A case of painless suprascapular nerve entrapment, resulting in isolated atrophy and weakness of the infraspinatus, is described. electromyography was used to confirm the clinical diagnosis. The etiology of this neuropathy ranges from trauma to overexertion of the upper extremity. Consequent entrapment most often occurs at the suprascapular or spinoglenoid notch. Local ganglionic cysts have been found compressing the suprascapular nerve. Conservative management, including "benign neglect," physical therapy, and local injections, is often effective. Surgical decompression may be indicated in refractory or posttraumatic cases.
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5/7. Juvenile spinal muscular atrophy: a new hexosaminidase deficiency phenotype.

    A 24-year-old Ashkenazi Jewish man was evaluated for a nine-year history of progressive leg weakness with fasciculations. electromyography, nerve conduction velocities, muscle biopsy, and serum creatine kinase were consistent with anterior horn cell disease. On rectal biopsy, ganglion cells were filled with membranous cytoplasmic bodies and an unusual submucosal layer of periodic acid-Schiff positive histiocytes filled with granules was seen. hexosaminidase a in serum and leukocytes was severely decreased in the patient and partially decreased in parents and a brother. A paternal relative had classic infantile tay-sachs disease. Juvenile spinal muscular atrophy in this patient, closely resembling the Kugelberg-Welander phenotype, resulted from an alpha-locus hexosaminidase deficiency disorder, possibly a genetic compound of HEX alpha 2 and a milder hexosaminidase alpha-locus allele. Other cases of hexosaminidase deficiency have included anterior horn cell disease as part of a more complex disorder, but this is the first case, to our knowledge, of a hexosaminidase deficiency disorder presenting as spinal muscular atrophy.
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6/7. Sensory ganglioneuropathy in infantile spinal muscular atrophy. light and electronmicroscopic findings in two cases.

    light and electronmicroscopic findings in two cases. Neuropediatrics 12: 215-31 (1981). Two cases of infantile spinal muscular atrophy (Werdnig-Hoffmann disease) are described in unrelated children deceased at 11 months (acute clinical onset at 6 months) and 2 years (onset at birth). Severe respiratory difficulties, hypotonia, muscular weakness and depressed tendon reflexes were the main clinical features. Bulbar palsy, bilateral ptosis, pale optic discs and atactic movements of the hands were observed in the child deceased at 11 months. Besides severe loss of anterior horn cells and neurogenic muscle atrophy there was evidence of an extensive sensory involvement in both cases. Shrinkage, vacuolation as well as chromatolytic changes of dorsal root ganglion cells, together with the evidence of a primary axonal damage in sural nerve biopsies were interpreted in terms of ganglioneuropathy of the primary sensory neurons. An invasion of fibrous astrocytes into dorsal roots constituted another striking anomaly in one case as well as a pronounced degeneration of cranial nerves V and VIII in the other case, a finding not hitherto reported in Werdnig-Hoffmann disease.
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7/7. MRI of suprascapular neuropathy in a weight lifter.

    Suprascapular neuropathy results from abnormal compression of the suprascapular nerve, typically at the suprascapular or spinoglenoid notch. This may be produced by either mass effect such as ganglion cyst or by certain repetitive shoulder motions producing wide scapular excursion (e.g., hyperabduction), which causes traction upon the nerve. Certain sports activities such as weight lifting predispose to this type of neuropathy. The clinical presentation is frequently not specific and the patient may be sent for MR evaluation to rule out rotator cuff tear or other more common shoulder abnormalities. This entity should be suspected if MR images demonstrate selective atrophy of the spinatus muscles with a structurally intact rotator cuff.
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