11/155. Cervical myelopathy due to a "tight dural canal in flexion" with a posterior epidural cavity.A 41-year-old man noticed weakness and atrophy in his right hand and forearm resembling the non-progressive juvenile muscular atrophy of unilateral upper extremity (Hirayama's disease). MRI showed an abnormal cavity in the posterior epidural space which appeared on neck flexion communicating with the subarachnoid space in addition to the flattening of the lower cervical spinal cord on neck flexion. When evaluating atypical cases of Hirayama's disease, the pathomechanism demonstrated in the present case should be taken into consideration.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
12/155. gyrate atrophy of the choroid and retina: clinical, ophthalmologic, and biochemical considerations.A case of gyrate atrophy of the choroid and retina associated with hyperornithinemia has been subjected to extensive clinical and biochemical investigation. The familial occurrence of the ocular disease and of abnormality of amino acid was unique to this 28-year-old male, being absent in parents and siblings. He presented with progressive visual loss, and was found to have cataracts and large areas of peripheral lacunar atrophy. Clinically there was no other abnormality. However, he was hyperuricemic and has an abnormal EEG. Despite otherwise normal biochemical indices of hepatic, renal, and muscle function; selective catheterization of an artery, the hepatic vein, the renal vein, and a deep forearm vein showed all of these circulatory beds to be producing ornithine according to arteriovenous difference measurements. cerebrospinal fluid and urine contained increased amounts of ornithine. Though electromyography was normal, the muscle biopsy was abnormal. Clinical tests including arginine loading, glucose tolerance testing, and other measurements of blood variables provided inferences as to the metabolic locus of the abnormality. The syndrome is a systemic multiorgan disorder in which the choroid and retina would appear to be target organs and the hyperornithinemia to be of, as yet, undetermined cause and pathogenic significance.- - - - - - - - - - ranking = 0.1240309375646keywords = loss (Clic here for more details about this article) |
13/155. Juvenile muscular atrophy of distal upper extremity associated with airway allergy: two cases successfully treated by plasma exchange.Juvenile muscular atrophy of the distal upper extremity (JMADUE) is postulated to be a type of flexion myelopathy. However, patients with typical clinical features of JMADUE but without evidence of flexion myelopathy have been reported. We recently reported a significant association of JMADUE with airway allergy. Here we report the successful treatment by plasma exchange (PE) of two patients with both airway allergy and JMADUE without evidence of flexion myelopathy. Patient 1 had a 5-year history of allergic rhinitis and high titers of mite antigen-specific IgE, and patient 2 had an 8-year history of pollinosis and high titers of cedar pollen-specific IgE. Both patients noted progressive distal muscle atrophy and weakness of the upper extremities: patient 1 for 3 months and patient 2 for 3 years. Neurologically, both showed asymmetric intrinsic hand muscle atrophy, oblique atrophy of the forearm muscles, and weakness and contraction fasciculation (minipolymyoclonus) of these muscles without any sensory impairment. Neither had any evidence of flexion myelopathy on magnetic resonance imaging (MRI) in a flexed position. Both were subjected to PE three times. Soon after the PEs, both showed improvement of distal muscle weakness of the upper extremities and marked reduction of contraction fasciculation of the forearm muscles. Patient 1 showed marked reduction in ongoing denervation potentials in the distal muscles on needle electromyography, while patient 2 showed marked improvement of F wave persistence of bilateral median and ulnar nerves. serum total and allergen-specific IgE decreased after PEs in both patients. PE was thus considered to be effective in these two patients having JMADUE without evidence of flexion myelopathy. These observations may suggest the involvement of an immune-mediated process in the neural damage of JMADUE without evidence of flexion myelopathy, especially in patients with atopic diathesis, and may support the notion that JMADUE is etiologically heterogeneous.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
14/155. Concomitant compression of median and ulnar nerves in a hemophiliac patient: a case report.A 15-year-old boy, with a diagnosis of hemophilia a, suffered bleeding into his left forearm 5 months before being admitted to our medical center. His neurological examination revealed a pronounced median neuropathy and a minor ulnar neuropathy on the left side. There was marked muscle atrophy on the thenar side and, to a lesser degree, on the hypothenar side and in the forearm. Electromyographic findings demonstrated an evident, nearly complete, sensorimotor axonal loss in the median nerve. magnetic resonance imaging studies showed atrophy in muscles of the left forearm and median nerve. The patient was diagnosed as having median nerve axonotmesis and ulnar nerve neuropraxia due to compartment syndrome. In hemophiliac patients, frequent single nerve compressions (often involving the femoral nerve) can be seen. However, concomitant median and ulnar nerve injuries with differing severity are rare.- - - - - - - - - - ranking = 0.1240309375646keywords = loss (Clic here for more details about this article) |
15/155. Bilateral suprascapular nerve entrapment.Bilateral suprascapular nerve entrapment syndrome is very rare. It presents with shoulder pain, weakness and atrophy of the supraspinatus and infraspinatus muscles. We present a twenty-year old man having a history of bilateral shoulder pain associated with weakness. Electromyographic studies revealed signs of a lesion that caused a neupraxic state of the left suprascapular nerve, moderate axonal loss of the right suprascapular nerve and denervation of the right suprascapular muscle. The patient was treated with physical and medical therapy. Due to worsening of the symptoms, a surgical operation was performed by the excision of the transverse scapular ligaments bilaterally. His pain, weakness and atrophy had diminished on examination six weeks later. Suprascapular nerve entrapment should be considered in patients with shoulder pain, particularly those with weakness and atrophy of the supraspinatus and infraspinatus muscles.- - - - - - - - - - ranking = 0.1240309375646keywords = loss (Clic here for more details about this article) |
16/155. magnetic resonance imaging of thoracic epidural venous dilation in Hirayama disease.Hirayama disease is a nonprogressive, asymmetric amyotrophy of the hands and forearms, possibly caused by compression of the lower cervical cord on neck flexion. The authors used phase-contrast MR angiography to study a patient with this disorder and observed abnormal spinal epidural venous dilation on neck flexion. In addition to mechanical compression of the lower cervical cord, venous congestion in the spinal canal may have a role in promoting anterior horn damage.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
17/155. myositis in a patient with large granular leukocyte leukemia.We describe the case of a 58-year-old patient with subacute progressive weakness in both legs accompanied by recurrent opportunistic infections. White cell count was normal, but immunophenotyping revealed an increased number of CD8( ) T cells and deficiency of natural killer cells, B cells, and CD4( ) T cells in the peripheral blood. Large granular leukocyte (LGL) leukemia was diagnosed based on a clonal T-cell receptor rearrangement. Muscle biopsy demonstrated severe myositis with extensive CD8( ) T-cell infiltrates. Since no evidence of microbial muscle infection was found, autoimmune myositis was diagnosed. Immunosuppressive treatment resulted in clinical improvement and normalization of creatine kinase (CK) serum levels. The immunological phenotype of the patient and the positive response to treatment adds further to the concept that CD8( ) T cells mediate disease in autoimmune myositis.- - - - - - - - - - ranking = 0.35954948382763keywords = leg (Clic here for more details about this article) |
18/155. Muscle MRI findings of X-linked spinal and bulbar muscular atrophy.We report here muscle MRI findings of the lower limb in X-linked spinal and bulbar muscular atrophy (SBMA). T1-weighted imaging of muscle MRI disclosed that the thigh muscles, including the semimembranosus, biceps femoris longus and the vastus lateralis muscles, showed high intensity signals with atrophy. Contrarily, the sartorius, gracilis and rectus femoris muscles were comparably preserved. Not only the thigh muscles, but also the calf muscles including the gastrocnemius medialis and lateralis, and soleus muscles showed high intensity signals. In amyotrophic lateral sclerosis (ALS), the leg muscles are generally atrophic, but the selective pattern of fatty degeneration, seen in SBMA was not observed. Muscle MRI is a useful method of estimating the distribution and severity of SBMA in affected muscles.- - - - - - - - - - ranking = 0.35954948382763keywords = leg (Clic here for more details about this article) |
19/155. Lumbar myofascial flap for pseudomeningocele repair.OBJECT: Initial management for lumbar pseudomeningoceles entails the closed external drainage of cerebrospinal fluid (CSF) with or without blood patch application. The presence of longstanding pseudomeningoceles and those associated with nonmicroscopic dural tears can be more problematic. Additionally the failure of nonoperative measures may necessitate surgery. Ideally the procedure should involve repairing the dural defect, removing the encapsulated cavity of the pseudomeningocele, and obliterating the extraspinal dead space to minimize the recurrence of the problem. methods: The authors describe a technique performed in 12 patients with large (> 5-cm-diameter) pseudomeningoceles referred for management following the failure of less aggressive measures. diagnosis was based on symptoms of lumbar wound swelling, postural headaches, back and leg pain, and was confirmed by imaging studies. In all patients subarachnoid CSF drainage and initial operative attempts to obliterate the pseudomeningocele had failed. They were treated between July 1990 and July 1998. The cause of the pseudomeningoceles was lumbar discectomy (four patients), lumbar decompression (one patient), lumbar decompression and placement of instrumentation (five patients), and intradural procedures (two patients). Their mean age was 47.9 years (range 20-67 years), and they presented at a mean of 5.5 months postoperatively (range 3 weeks-37 months). In all cases there was a satisfactory repair of the pseudomeningocele, dead space obliteration, and long-term symptomatic resolution. CONCLUSIONS: Lumbar myofascial advancement for this problem is a useful technique in cases of symptomatic pseudomeningoceles. This technique requires the medial advancement of the musculofascial units of the paravertebral muscles for a layered closure over the exposed spinal canal with obliteration of the pseudomeningocele.- - - - - - - - - - ranking = 0.35954948382763keywords = leg (Clic here for more details about this article) |
20/155. Monomelic amyotrophy associated with the 7472insC mutation in the mtDNA tRNASer(UCN) gene.We describe a 49-year-old male patient who experienced progressive amyotrophy with no sensorial abnormality in the left arm since 45 years of age. The neuromuscular syndrome was identical to that known as Hirayama disease, a rare form of focal lower motor neuron disease affecting the C7-C8-T1 metamers of the spinal cord. Asymmetric neurosensorial hearing loss was present since age 35 in the patient, and was also documented in an elder sister and in the mother. A muscle biopsy showed cytochrome c oxidase (COX) negative fibers but no ragged-red fibers, and mild reduction of COX was confirmed biochemically. The patient was found to have high levels of a known pathogenic mutation of mtDNA, the 7472insC in the gene encoding the tRNA(Ser(UCN)). Investigation on several family members showed a correlation between mutation load and clinical severity. This is the second report documenting the association of lower motor neurone involvement with a specific mtDNA.- - - - - - - - - - ranking = 0.1240309375646keywords = loss (Clic here for more details about this article) |
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