Cases reported "Muscular Atrophy"

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1/180. Anaesthetic implications of rigid spine syndrome.

    The perioperative management of a 14-year-old girl, suffering from the muscular disorder rigid spine syndrome, is presented. The anaesthetic implications with regard to possible difficult intubation, cardiac involvement, malignant hyperthermia, neuromuscular blocking agents, and postoperative recovery are discussed.
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keywords = block
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2/180. Cervical cord tethering mimicking focal muscular atrophy.

    spinal cord tethering rarely occurs in the cervical region. In adults, it usually results from previous operations. However, congenital origin is always diagnosed and treated early in the infant period. We report a 12-year-old boy with cervical spinal dysraphism which was erroneously diagnosed as focal muscular atrophy, a benign form of motor neuron disease. The patient was brought to our hospital because of rapid deterioration of symptoms. Careful evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C(4) to C(6) levels and fusion of the laminae of C(4) and C(5). spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and the ventral nerve roots were markedly stretched, especially over the left side. Surgical intervention was undertaken and the patient's muscle power improved after untethering. The purpose of this report is to acquaint the reader with a surgically treatable condition that may appear to be benign focal amyotrophy. skin lesion at the nuchal area should be carefully looked for.
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ranking = 158.34203572249
keywords = nerve
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3/180. Hereditary neuropathy and vocal cord paralysis in a man with childhood diphtheria.

    We present the case of a 37-year-old Afghani man with a history of childhood diphtheria, who was diagnosed with bilateral vocal cord paralysis at age 15 years. At about this time he developed progressive muscular wasting and distally predominant weakness, and subsequently developed respiratory insufficiency, necessitating nocturnal ventilatory support. His examination suggested a distal symmetric sensorimotor neuropathy, and his brother was similarly affected, although to a lesser degree. electromyography (EMG) and nerve conduction studies revealed this process to be purely axonal. A diagnosis of possible hereditary motor and sensory neuropathy (HMSN) type IIc, hereditary axonal polyneuropathy with vocal cord paralysis, is proposed, although the question of early diphtheritic involvement of the vocal cords and peripheral nerves is also considered.
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ranking = 316.68407144498
keywords = nerve
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4/180. motor neuron disease with predominantly upper extremity involvement: a clinicopathological study.

    We report two autopsy cases of motor neuron disease (MND) patients with an unusual type of muscular atrophy predominantly affecting the shoulder girdle and the upper extremities with proximal dominance. Both patients are considered to be clinically categorized into the El Escorial suspected form of amyotrophic lateral sclerosis (ALS). At autopsy, they showed marked loss of spinal anterior horn cells accompanied by astrogliosis positively immunostained with anti-glial fibrillary acidic protein antibody at the cervical level. At the lumbosacral level, anterior horn neurons were relatively well preserved and Bunina bodies, ubiquitin-positive skein-like inclusions and Lewy body-like inclusions were observed in the remaining neurons. In one patient, brain stem motor neurons (nerves V, VII, XII) and motor cortex, including Betz cells, were also affected and the corticospinal tracts were degenerated at the level of the thoracic and lumbar spinal cord. Pathological findings of this patient are consistent with those of ALS. In the other patient, the motor cortex, brain stem motor nuclei and the corticospinal tracts were well preserved, which is pathologically compatible with progressive spinal muscular atrophy. These patients with such a peculiar pattern of progressive muscular atrophy should be placed in a subgroup of ALS.
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ranking = 158.34203572249
keywords = nerve
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5/180. Acute quadriplegic myopathy in a 17-month-old boy.

    Acute quadriplegic myopathy is a rare condition associated with the use of nondepolarizing muscle-blocking agents and corticosteroids in the course of severe systemic illness. A 17-month-old boy underwent liver transplantation for fulminant hepatitis. He was intubated for 24 days and treated with vecuronium bromide and high-dose methylprednisolone. The child was weaned from the ventilator and presented extreme weakness in the upper limbs and total paralysis of the lower limbs. serum creatine kinase level was normal and electromyography showed myopathic abnormalities. Muscle biopsy showed severe type-1 fiber atrophy and selective loss of myosin thick filaments was seen on electron microscopy. Scattered regenerating fetal myosin-positive fibers were present, mu calpain was absent, while m calpain was diffusely expressed. Physical therapy was immediately started and the child recovered even though corticosteroids were not discontinued. The pathogenesis of acute quadriplegic myopathy is still unknown. We suggest that it could be due to abnormal protein turnover in the muscle. Several independent factors, such as corticosteroid treatment, immobilization, or cytokines, could take part in a cascade of events that leads to an excessive yet selective degradation of proteins involving myosin thick filaments and possibly components of sarcolemma, causing muscle inexcitability.
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6/180. ulnar nerve injuries of the hand producing intrinsic muscle denervation on magnetic resonance imaging.

    Muscle and nerve injuries in the hand may be difficult to detect and diagnose clinically. Two cases are reported in which magnetic resonance imaging showed ulnar nerve injury and intrinsic hand muscle denervation. The clinical, anatomical and radiological features of injury to the deep motor branch of the ulnar nerve and associated muscle denervation are discussed and illustrated.
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ranking = 1108.3942500574
keywords = nerve
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7/180. Isolated hypoglossal nerve palsy due to an anomalous vertebral artery course: report of two cases.

    The authors report two patients with isolated unilateral tongue atrophy. magnetic resonance imaging (MRI) of the brain stem and angio-MRI demonstrated a dolichovertebral artery with an abnormal course compressing the medulla oblongata at the emergence of the hypoglossal rootlets. The semeiological observation of a sectorial and not uniform distribution of atrophy in the half-affected tongue is discussed in relation to the lesional site.
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ranking = 633.36814288997
keywords = nerve
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8/180. Postinjection vastus lateralis atrophy: 2 case reports.

    We report two cases of postsurgical intramuscular meperidine injection with injury to the femoral nerve and subsequent vastus lateralis atrophy. The first case is a patient who had arthroscopic anterior cruciate ligament reconstruction; the second, a patient with a C6-C7 anterior fusion. photographs, radiography, and electrodiagnostic studies clearly depict the nature of the injuries, and their etiology is discussed. These case reports describe a unique neuropathic injection injury that, to our knowledge, has never before been described in the literature.
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ranking = 158.34203572249
keywords = nerve
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9/180. 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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ranking = 316.68407144498
keywords = nerve
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10/180. 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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ranking = 475.02610716747
keywords = nerve
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