11/594. Complete rupture of the distal semimembranosus tendon with secondary hamstring muscles atrophy: MR findings in two cases. Complete rupture of the hamstring muscles is a rare injury. The proximal musculo-tendinous junction is the most frequent site of rupture. We present two cases of complete rupture of the distal semimembranosus tendon, which clinically presented as soft-tissue masses. MR imaging permitted the correct diagnosis. There has been only one other such case reported. ( info) |
12/594. 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. ( info) |
13/594. 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. ( info) |
14/594. Myoadenylate deaminase deficiency with progressive muscle weakness and atrophy caused by new missense mutations in AMPD1 gene: case report in a Japanese patient. A 46-year-old woman with exertional myalgia developed slowly progressive weakness in her lower extremities. She had slight muscle weakness in her facial and upper extremities, and severe muscle weakness and atrophy in lower extremities more marked in the proximal portions. serum creatine kinase was slightly elevated. After ischemic forearm exercise test, blood ammonia had no elevation although lactate level increased normally. The computed tomography revealed that a characteristic distribution of skeletal muscle involvement with proximal and flexor muscles more severely affected than distal and extensor in the lower extremities. In addition, the left sternocleidomastoid muscle showed marked atrophy with an asymptomatic weakness of over 20 years duration suggesting abnormal development. Needle EMG examination showed a large number of easily recruited, short-duration, low-amplitude motor unit potentials in all extremities. Muscle biopsy showed absence of adenosine monophosphate deaminase activity with normal cytochrome c oxidase and phosphorylase activity. With the muscle enzyme activity assay, adenosine monophosphate deaminase activity was found to be lower than 0.2% of the controls. The dna analysis revealed that she was compound heterozygote involving two missense mutations (R388W and R425H) in exon 9 and exon 10 of AMPD1 gene. This is the first report of primary myoadenylate deaminase deficiency with progressive weakness and atrophy caused by novel compound heterozygous mutations of AMPD1 gene, and suggests that adenosine monophosphate deaminase is closely related not only to energy metabolism but also to the development of skeletal muscle. ( info) |
15/594. Gluteal neuralgia - unusual presentation in an adult with intrasacral meningocele: a case report and review of literature. A nineteen year old man with intrasacral meningocele is reported, who presented with long standing episodic gluteal pain and progressive muscle wasting. magnetic resonance imaging established the diagnosis. Surgical excision relieved the pain but muscle wasting persisted. Pertinent literature is reviewed. ( info) |
16/594. rhabdomyolysis triggered by an asthmatic attack in a patient with McArdle disease. We describe a patient with McArdle disease who developed rhabdomyolysis triggered by a bronchial asthmatic attack. A 64-year-old man had chronic pulmonary emphysema with asthma, and an asthmatic attack led to severe rhabdomyolysis that required continuous hemodiafiltration. After 2 years, a physical examination revealed atrophy of the extremities compared with previous examinations, especially of the intercostal muscles. During that time, he suffered two severe bronchial asthmatic attacks. His serum level of creatinine kinase remained between 4,000 and 7,000 IU/l when he did not suffer from asthmatic attacks and rhabdomyolysis had abated. Therefore, we suspected that his recent muscle atrophy was caused by asthmatic attacks, and discussed the possibility of his respiratory muscle weakness due to McArdle disease in relation to his severe bronchial asthmatic attacks as well as chronic obstructive pulmonary disease. ( info) |
17/594. Radiological diagnosis of abductor denervation after hip surgery. A case of total hip arthroplasty through a direct lateral approach is described. The patient had a markedly positive Trendelenburg test at follow-up. Radiographs showed features consistent with the denervation of the gluteus medius. This was confirmed on CT scan. The standard post-operative radiograph following a total hip replacement may suggest denervation of the gluteus medius. ( info) |
18/594. Intravenous immunoglobulin therapy for diabetic amyotrophy. A 49-year-old woman with diabetes mellitus developed progressive weakness and atrophy of both thighs rendering her wheelchair-bound within two months. The neurological findings and electrophysiological test results were compatible with diabetic amyotrophy (DA). Immediately after intravenous immunoglobulin (IVIg) therapy (20 g x 3 days), she became able to walk with a cane. After the next course of the therapy, she could walk without assistance. This dramatic effect of IVIg therapy together with the recent observation of vasculitic neuropathy in DA indicates an inflammatory process in this condition, and gives support to this treatment for DA. ( info) |
19/594. Autoimmune hypopituitarism in patients with coeliac disease: symptoms confusingly similar. Coeliac disease does not always respond properly to a gluten-free diet, and treatment may be complicated by an underlying autoimmune endocrine disorder. We report three cases of hypopituitarism in patients with coeliac disease who seemed to have incomplete dietary response. The first patient had diabetes and suffered from hypoglygaemic events; the second had muscular atrophy of unknown origin while the third had growth failure. None had a pituitary mass, suggesting that hypopituitarism was of autoimmune origin. overall condition improved only after replacement therapy for the underlying hormone deficiency; this association should thus be recognized. ( info) |
20/594. Congenital contractural arachnodactyly with neurogenic muscular atrophy: case report. We report the case of a 3-(1/2)-year-old girl with hypotonia, multiple joint contractures, hip luxation, arachnodactyly, adducted thumbs, dolichostenomelia, and abnormal external ears suggesting the diagnosis of congenital contractural arachnodactyly (CCA). The serum muscle enzymes were normal and the needle electromyography showed active and chronic denervation. The muscle biopsy demonstrated active and chronic denervation compatible with spinal muscular atrophy. Analysis of exons 7 and 8 of survival motor neuron gene through polymerase chain reaction did not show deletions. Neurogenic muscular atrophy is a new abnormality associated with CCA, suggesting that CCA is clinically heterogeneous. ( info) |