1/155. Anterior interosseous nerve compression after supracondylar fracture of the humerus: a metaanalysis.OBJECT: The authors conducted a metaanalysis of reports of anterior interosseous nerve syndrome, a rare nerve compression neuropathy that affects only the motor branch of the median nerve. This syndrome is characterized by paralysis of the flexor pollicis longus, the flexor digitorum profundus to the index finger, and the pronator quadratus, with weakness on flexion of the interphalangeal joint of the thumb and the distal interphalangeal joint of the index finger without sensory loss. methods: The authors reviewed reports of 34 cases of anterior interosseous nerve syndrome combined with supracondylar fractures of the humerus in children. They have added a new case identified in a 7-year-old boy in whom a diagnosis was made from the clinical findings and whose treatment and outcome are analyzed. The ages of patients reported in the literature ranged from 4 to 10 years. Ten patients (29%) were treated with closed reduction and application of a cast, whereas 25 patients (71%) were treated with open reduction and fixation of the fracture. CONCLUSIONS: All patients regained full flexion and strength after 4 to 17 weeks. The fractures that were surgically treated showed no entrapment of the anterior interosseous nerve.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
2/155. Focal myositis presenting with radial nerve palsy.Focal myositis is a rare inflammatory pseudotumor of skeletal muscle which usually has a benign course. We report a 56-year-old woman with a painful mass in the left arm with a radial nerve palsy. magnetic resonance imaging (MRI) of the left arm showed a mass in the triceps muscle that was suggestive of a soft-tissue sarcoma. electromyography showed a severe radial neuropathy involving both motor and sensory axons. An open biopsy showed focal myositis. Treatment with corticosteroids resulted in complete disappearance of the mass clinically and by MRI, without recurrence for more than 2 years. radial nerve function also recovered completely. As a treatable cause of focal neuropathy, focal myositis should be included in the differential diagnosis of a muscle mass.- - - - - - - - - - ranking = 2keywords = neuropathy (Clic here for more details about this article) |
3/155. Uncompacted myelin in hereditary neuropathy with liability to pressure palsies with the 17 p11.2 deletion.A 16-year-old girl with a typical features of hereditary neuropathy with liability to pressure palsies (HNPP) and deletion on chromosome 17p11.2 was described. In the mother who was asymptomatic the same genetic defect was found. In a sural nerve biopsy obtained from the girl myelin thickenings characteristic for this disease and de- and remyelination in nerve fibers were found. Special attention was paid to the occurrence of uncompacted myelin, which was present in diffuse and focal forms. It is concluded that high amount of uncompacted myelin is characteristic for HNPP and it is probably related to the under-expression of peripheral myelin protein 22.- - - - - - - - - - ranking = 5keywords = neuropathy (Clic here for more details about this article) |
4/155. Bilateral phrenic neuropathy as a presenting feature of multifocal motor neuropathy with conduction block.Diaphragmatic paralysis has previously been reported as a result of diverse pathologic processes involving the peripheral nervous system. We report the clinical history, physical findings, and antibody profile of an atypical case of multifocal motor neuropathy with conduction block initially presenting with respiratory failure secondary to bilateral phrenic neuropathy.- - - - - - - - - - ranking = 10keywords = neuropathy (Clic here for more details about this article) |
5/155. Fulminant case of hereditary neuropathy with liability to pressure palsy.Hereditary neuropathy with liability to pressure palsy (HNPP) is typified as isolated nerve palsies caused by trivial compression or trauma. It rarely presents in two extremities and even more infrequently affects all four limbs simultaneously. We present a patient who concurrently experienced right shoulder, left hand, and bilateral foot weakness mimicking several multifocal conditions. electromyography suggested HNPP and subsequent nerve biopsy and genetic testing were confirmatory. The case demonstrates that HNPP can present in a fulminant manner and should be included in the differential diagnosis of acute multiple mononeuropathies. The possible causes for such a rapid clinical course in our patient are discussed.- - - - - - - - - - ranking = 5keywords = neuropathy (Clic here for more details about this article) |
6/155. Synovial osteochondromatosis at the elbow producing ulnar and median nerve palsy. Case report and review of the literature.The authors present the case of a 53-year-old woman suffering from synovial osteochondromatosis of her right elbow responsible for ulnar and median nerve entrapment neuropathy. This condition is characterised by the formation of multiple cartilaginous nodules in the metaplastic synovium of otherwise normal joints, bursae or tendon sheaths. Treatment consisted of partial synovectomy, removal of loose bodies and microscopic nerve release. Synovial osteochondromatosis complicated by nerve compression syndromes has been rarely reported, usually with ulnar tunnel syndrome at the elbow. The literature on this subject is reviewed.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
7/155. Hypertrophic perineurial dysplasia in multifocal and generalized peripheral neuropathies.Two cases are described, one with a multifocal cranial and limb neuropathy of adult onset associated with optic neuropathy, and the other with a diffuse demyelinating neuropathy characterized by congenital cataract, mental retardation and progressive lower limb paresis with an onset in childhood. Extensive investigation in both failed to establish the causation. No family history of similar disorder was obtained in either case. Nerve biopsy in both showed similar perineurial abnormalities, the endoneurium being compartmentalized by hypertrophic perineurial cells that exhibited dysplastic features. The appearances resemble those described in a previously reported case of multifocal neuropathy and probably represent an unusual but non-specific response to a peripheral neuropathy.- - - - - - - - - - ranking = 5keywords = neuropathy (Clic here for more details about this article) |
8/155. Hereditary neuropathy with liability to pressure palsies: a case report.Hereditary neuropathy with liability to pressure palsy is a rare autosomal dominant disorder characterized by multiple episodes of focal demyelinating neuropathies after minor trauma to peripheral nerves. It usually appears in early adulthood with recurrent attacks of pain, numbness, and muscular weakness along the distribution of the clinically affected nerve. Segmental demyelination and thickenings of the myelin sheath are the pathologic findings. Electrophysiologic studies show a nonuniform mild demyelinating neuropathy with prolonged distal latencies. Genetic tests are available to aid in diagnosis as molecular analysis has identified a deletion in the chromosome 17p11.2 in the majority of these patients. There is a paucity of information in the orthopaedic literature regarding hereditary neuropathy with liability to pressure palsy. A case report is presented of a patient with this disorder to promote awareness and recognition that this entity should be considered in patients with multiple nerve palsies.- - - - - - - - - - ranking = 7keywords = neuropathy (Clic here for more details about this article) |
9/155. A novel 3'-splice site mutation in peripheral myelin protein 22 causing hereditary neuropathy with liability to pressure palsies.Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant, demyelinating peripheral neuropathy. Clinical hallmarks are recurrent painless focal neuropathies mostly preceded by minor trauma or compression at entrapment sites of peripheral nerves. In the majority of the patients, HNPP is caused by a 1.5 Mb deletion on chromosome 17p11.2-p12 containing the peripheral myelin protein 22 (PMP22) gene. Point mutations within this gene are reported in only a few families. We report a novel mutation in the PMP22 gene in a Spanish family with HNPP. The mutation is a 3' splice-site mutation, preceding coding exon 3 (c.179-1 G>C), causing a mild HNPP phenotype.- - - - - - - - - - ranking = 6keywords = neuropathy (Clic here for more details about this article) |
10/155. Focal myopathy mimicking posterior interosseous nerve syndrome.A 25-year-old man developed weakness of extension of the right index, middle, and fourth fingers at the metacarpophalangeal joints, over 2 years. No sensory deficit was present. Nerve conduction studies, including the right radial nerve, were within normal limits. Needle electromyographic (EMG) examination showed myopathic changes that were limited to the right extensor digitorum communis and extensor indicis proprius muscles. An intravenous edrophonium chloride test had no effect on weakness and repetitive stimulation showed no significant decremental response. An EMG-guided open biopsy of the extensor digitorum communis muscle revealed severe myopathic changes. Evaluation for the cause of myopathic involvement was negative. After 13 months, clinical examination and electrophysiological studies showed no significant progression. This case exemplifies the fact that a focal myopathy may mimic an entrapment neuropathy.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
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