Cases reported "Muscular Atrophy"

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11/347. Cervical spondylotic amyotrophy with intramedullary cavity formation.

    STUDY DESIGN: A case report. OBJECTIVE: To show that an intramedullary lesion was the cause of cervical spondylotic amyotrophy. SUMMARY OF BACKGROUND DATA: Cervical spondylotic amyotrophy is the clinical syndrome characterized by muscle wasting and weakness in the upper extremities without a remarkable sensory loss or spastic tetraparesis. It is still unclear whether the ventral roots or the anterior horn are selectively damaged. methods: magnetic resonance imaging and delayed computed tomographic myelography were performed on a patient who showed severe wasting of the left triceps muscle without any sensory disturbance or long tract sign. RESULTS: On sagittal magnetic resonance images, a linear area was noted within the spinal cord at C6 and C7 spinal segments, which showed low signal intensity on T1-weighted image and high signal intensity on T2-weighted image. On axial T1-weighted image intramedullary low signal intensity area was observed, which was located in the left anterior horn. On axial T2-weighted image the area showed high signal intensity. A delayed (6 hours) computed tomographic scan after intrathecal injection of metrizamide revealed intramedullary enhancement in the area corresponding to the left anterior horn, which would represent cavitation or cystic necrosis. CONCLUSIONS: This is the first case report of cervical spondylotic amyotrophy, in which intramedullary lesion was confirmed only at the affected side of the spinal cord.
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12/347. An unusual form of spinal muscular atrophy with mental retardation occurring in an inbred population.

    Three sibs are described suffering from hereditary non-progressive spinal muscular atrophy with non-progressive mental retardation. One of them had in addition signs of pyramidal tract involvement. Muscular weakness was more pronounced proximally than distally and the neck muscles were severely involved. Th.ey all had small skulls and several associated congenital malformations were observed including syndactyly of the left hand in 1 patient. The patients belong to a small inbred community in the netherlands. Erythropoietic protoporphyria was also present in the family but segregated independently. This combination of "congenital" mental retardation with "congenital" non-progressive spinal muscular atrophy is believed to represent a new syndrome, caused by a rare recessive gene.
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13/347. nail-patella syndrome associated with respiratory chain disorder.

    nail-patella syndrome (NPS) has not been described to be associated with a respiratory chain disorder (RCD) before. In a 42-year-old man with the typical phenotype of an NPS, weakness and wasting of the shoulder girdle muscles, muscle cramps, fatigability, hyperhidrosis, chest pain and creatine kinase elevation were observed. echocardiography revealed left ventricular hypertrabeculation. Needle electromyography was myopathic, lactate stress testing was abnormal, muscle biopsy showed typical features of an RCD and mtDNA analysis revealed the A3243G MELAS mutation. In conclusion, this case demonstrates that NPS may be randomly associated with RCD. NPS patients should undergo detailed cardiological and neurological investigations, in order not to overlook a double trouble partially mimicking NPS.
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14/347. A case of Dejerine-Sottas disease with schizophrenic symptoms. A clinical and pathological study.

    A case of hypertrophic interstitial neuritis with a disturbance of intelligence and schizophrenic symptoms was reported. The patient, a 41-year-old male, showed monologia at the age of 14 and auditory hallucination at the age of 21. He was diagnosed as schizophrenia. Torsion of the head to the right side and muscular atrophy were also noticed at 21 years. Atrophy of the muscles progressed gradually, particularly in the distal parts of the upper and lower extremeties. Psychiatric examination revealed schizophrenic symptoms such as auditory hallucination, thought disorder, lack of spontaneity, autism, apathy, etc. There was also a disturbance of intelligence (imbecility). In neurological examination, the torsion of the head to the right side, slight exaggeration of the peripheral reflexes, muscular atrophy in the distal parts of the extremeties were noted. The bilateral ulnar nerves were markedly hypertrophied. The biopsy of the right ulnar nerve revealed a remarkable thickening of the endoneuria and "onion bulb" formations. A large amount of the interstitial accumulation of PAS positive substance, which showed metachromasia in cresyl violet staining, was also noted. Schwann cell processes were proved electron-microscopically to surround the myelin sheaths and some of them meandered and extended and into the metachromatic substance. The basement membrane of the sheath cell was hypertrophic in some portions and elongated into interstitium. The nucleus of the sheath cell was hyperchromic and showed marked atrophy. Histological examination of the biopsy specimen obtained from the gastrocnemius muscle suggested neurogenic muscular atrophy. The relationship among the hypertrophic interstitial neuritis, schizophrenic symptoms, disturbance of intelligence and torsion of the head was also discussed.
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15/347. autopsy case of autosomal recessive hereditary spastic paraplegia with reference to the muscular pathology.

    An autopsied case of autosomal recessive hereditary spastic paraplegia with severe neurogenic muscular atrophy is described herein. This patient, a 16-year-old woman, presented with gait disturbance. She developed progressive spastic paralysis of the upper and lower limbs and mental deterioration. She became bedridden at approximately 40years of age. dysarthria worsened at 45 years of age. She died of pneumonia at 50 years of age. Her younger sister has shown similar clinical symptoms and became bedridden at 37 years of age. Their parents were second cousins. autopsy revealed a severely atrophic brain, weighing 720 g. The cerebral cortex was thin, and the white matter was extremely reduced in volume. Microscopically, neuronal loss and variable astrogliosis with diffuse spongy changes were evident at the cerebral cortex, thalamic nuclei, basal ganglia and hippocampus. The remaining neurons were atrophied with heavy deposition of lipofuscin. In the spinal cord, the pyramidal tracts as well as the dorsal spinocerebellar tracts were degenerated. In addition, marked loss of the anterior horn cells was seen. Severe neuronal loss of the nucleus gracilis was also detected. In contrast, only mild degeneration of the ventral spinocerebellar tracts and fasciulus cuneatus in the spinal cord were observed. In the frozen sections of skeletal muscle, severe neurogenic atrophy and fatty infiltration were evident. In addition, several rimmed vacuoles were observed in the atrophic fibers, and cytochrome coxidase-deficient fibers were present in part. Reduced nicotinamide adenine dinucleotide (NADH)-tetrazolium reductase reaction revealed abnormal accumulation of mitochondria around the center of the non-atrophic muscle fibers. It is suggested that an analysis of mitochondrial function of Japanese autosomal recessive hereditary spastic hemiplegia may provide additional information to clarify the pathogenesis.
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16/347. carpal tunnel syndrome and the Riche-Cannieu anastomosis: electrophysiologic findings.

    Two cases of carpal tunnel syndrome with Riche-Cannieu anomalies are reported. Despite complete absence of a median nerve evoked compound muscle action potential from the thenar eminence, these patients had significant preservation of function and minimal muscle atrophy. Activation of the ulnar nerve at both the wrist and elbow generated easily obtainable compound muscle action potentials from the thenar eminence with initial negative onset. This observed preservation of function and electrophysiologic responses are best explained by the presence of a Riche-Cannieu anastomosis innervating the thenar eminence through branches from ulnar nerve. To our knowledge there has not been a report of similar cases in patients with profound carpal tunnel syndrome and a Riche-Cannieu anomaly. We review the clinical findings, the electrodiagnostic data, and the impact of a Riche-Cannieu anastomosis on advanced carpal tunnel syndrome.
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17/347. Isolated brachialis wasting: an unusual presentation of neuralgic amyotrophy.

    Although neuralgic amyotrophy can selectively affect discrete components of the brachial plexus including individual peripheral nerves, involvement of an individual nerve fascicle is rare. Discrete fascicular musculocutaneous neuropathy as a manifestation of neuralgic amyotrophy has not previously been reported to our knowledge. We report two cases of otherwise typical neuralgic amyotrophy with isolated brachialis muscle wasting. Abnormal spontaneous activity, motor unit remodeling, or both, was observed only in the brachialis muscle. Lateral antebrachial cutaneous nerve conduction studies were normal. These cases serve to broaden the spectrum of the clinical presentation of neuralgic amyotrophy.
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18/347. Progressive neural muscular atrophy in a case of phenylketonuria.

    Clinical, neurophysiological and nerve-biopsy findings are described in a 13-1/2-year-old boy with classical phenylketonuria who developed progressive muscular atrophy. Clinical examination revealed atrophy of the calf muscles and pes varus. Tendon jerks were brisk in the upper extremities but were absent in the right leg and weak in the left leg. Nerve conduction velocities of the median and peroneal nerve were strongly reduced. light- and electronmicroscopic investigation of sural nerve biopsy revealed axonal dystrophy and 'onion-bulb' formation of the schwann cells. It is assumed that the combination of phenylketonuria and progressive muscular atrophy in this patient is an accidental occurrence.
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19/347. Myotonic pupils in charcot-marie-tooth disease. Successful relief of symptoms with 0.025% pilocarpine.

    Twenty-seven members of a family with dominantly inherited charcot-marie-tooth disease (CMTD) were examined. Fifteen members had CMTD and 13 of these had varying amounts of myotonic pupillary abnormalities similar in some ways to Adie tonic pupil syndrome. Those with graver neurologic disease showed greater pupillary abnormalities. Ten of the 15 patients had pupillary constriction with methacholine chloride (Mecholyl) and some of these had extensive iris atrophy. Several affected patients received symptomatic relief from 0.025% pilocarpine. Seven other patients with CMTD who were not related to our initial family were checked for myotonic pupils; two had findings similar to our initial family. Pupillary abnormalities in certain patients with CMTD appear secondary to a parasympathetic denervation of the iris sphincter and ciliary muscle, as shown by a positive methacholine test, and probably represent part of the autonomic nervous system dysfunction associated with the polyneuropathy in CMTD.
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20/347. bone resorption of the mandible in progressive systemic sclerosis.

    Five of 16 patients with progressive systemic sclerosis were found to have bone resorption at the angle of the mandible. This finding appears to be closely related to the tightness of the skin of the face, atrophy of the masseter and pterygoid muscles, small size of the oral orifice, and a significantly high frequency in blacks. The mandible must therefore be added to the list of those bones that can be resorbed in progressive systemic sclerosis.
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