1/23. Chronic inflammatory demyelinating polyneuropathy with multiple hypertrophic nerves in intracranial, and intra- and extra-spinal segments.Hypertrophic nerves have occasionally been seen in chronic inflammatory demyelinating polyneuropathy (CIDP), but most are in the cauda equina. We report a case with CIDP in whom magnetic resonance imaging (MRI) with gadolinium diethylene triamine penta-acetic acid (Gd-DTPA) enhancement demonstrated hypertrophy of various peripheral nerves including multiple cranial nerves. Interestingly, none showed neurological signs corresponding to the lesions, except for clinical signs consistent with CIDP. MRI can be useful for the detection of silent, but abnormal nerve involvement in CIDP.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
2/23. Hypertrophic inflammatory neuropathy involving bilateral brachial plexus.BACKGROUND: The present case is an example of hypertrophic inflammatory neuropathy (HIN). This entity is a rare tumor-like, chronic inflammatory, focal or multifocal, mainly demyelinating neuropathy of unknown origin, most frequently involving the brachial plexus. CASE DESCRIPTION: The authors describe a 67-year-old man presenting with a nodular mass in his right supraclavicular fossa. A nodular mass grossly resembling a schwannoma originating from a single nerve fascicle was surgically removed from the right C6 spinal nerve. Histologically, endoneurial edema, fibrosis, focal chronic inflammation, and extensive "onion bulb" formation were seen. Electron microscopy studies and immunohistochemistry proved that the onion bulb-forming cells were schwannian in nature and that the whorls of onion bulbs surrounded a generally demyelinated axon. Three months following surgery the patient developed acute painless paralysis of his right biceps brachii muscle that rapidly reversed; after that he remained neurologically asymptomatic. MRI revealed multiple fusiform mass lesions involving the brachial plexus bilaterally. Electrophysiologic studies demonstrated a bilateral, asymmetrical, mainly demyelinating neuropathy involving the brachial plexus; they failed to reveal any abnormality suggestive of generalized neuropathy. CONCLUSION: HIN is different from other focal tumor-like neuropathies and in particular from localized hypertrophic neuropathy (LHN).- - - - - - - - - - ranking = 0.0092173970831383keywords = demyelinating (Clic here for more details about this article) |
3/23. Chronic demyelinating hypertrophic brachial plexus neuropathy.A patient with unilateral, painless, chronic progressive upper limb sensorimotor deficit showed electrophysiological evidence of a focal demyelinating neuropathy with almost complete conduction block across the brachial plexus. magnetic resonance imaging disclosed marked brachial plexus hypertrophy. Intravenous immunoglobulin led to fast and complete recovery, maintained by intermittent perfusions. Hypertrophic brachial plexus neuropathy can be a presentation of focal chronic inflammatory demyelinating polyradiculoneuropathy. Objective and quantitative assessment of hand function is useful to evaluate treatment results and to optimize treatment regimens.- - - - - - - - - - ranking = 0.75687228731582keywords = chronic inflammatory demyelinating, polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
4/23. Sonographic detection of diffuse peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy.Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune disease characterized by recurrent demyelination and remyelination with resultant thickening of the peripheral nerves. We report a case in which sonography was instrumental in demonstrating diffuse peripheral nerve hypertrophy. On sonography, both brachial plexuses were found to be diffusely hypertrophic and hypoechoic. Similar findings were noted for the median, sciatic, and femoral nerves. The brachial plexus findings were confirmed by MRI.- - - - - - - - - - ranking = 3.2860722238541keywords = chronic inflammatory demyelinating, polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
5/23. 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 = 0.0046086985415692keywords = demyelinating (Clic here for more details about this article) |
6/23. Hypertrophic nerve roots in a case of Roussy-Levy syndrome.Hypertrophic radiculopathy is a rare feature of neuropathies. Single cases of enlarged nerve roots have been described in hereditary motor sensory neuropathies (HMSN) and chronic inflammatory demyelinating diseases (CIDP). This is the first description of hypertrophied nerve roots in a patient with Roussy-Levy syndrome. MRI did not show contrast enhancement of the enlarged nerve roots or nodular lesions.- - - - - - - - - - ranking = 0.50645739934863keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/23. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with hypertrophic spinal radiculopathy mimicking neurofibromatosis.This report illustrates a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) masquerading as neurofibromatosis due to multifocal enlargements of spinal nerve roots. The patient initially complained of intermittent numbness of the hands and leg weakness at age 62. Nerve conduction velocities were reported to be abnormally slow, suggesting a diagnosis of demyelinating neuropathy. A complaint of progressive lower back pain 4 years later prompted a lumbar CT myelogram, which demonstrated bilateral nerve root enlargements. A biopsy of an enlarged lumbar root obtained during decompressive laminectomy was interpreted as consistent with a plexiform neurofibroma. He suffered recurrent paraparesis, at times with a sensory level indicating spinal cord compression, which responded to corticosteroid therapy. An autopsy 15 years after the onset of symptoms revealed hypertrophic radiculopathy and peripheral neuropathy due to CIDP with no evidence of neurofibromatosis. This case illustrates how the hypertrophic neuropathy accompanying CIDP can be mistaken for neurofibromatosis.- - - - - - - - - - ranking = 2.1414656748383keywords = chronic inflammatory demyelinating, polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
8/23. Hypertrophic neuropathy of the cauda equina: case report.OBJECTIVE AND IMPORTANCE: Hypertrophic neuropathy of the cauda equina (HNCE) is a rare form of peripheral neuropathy. The diagnosis is complicated by an insidious clinical presentation and complex radiographic images. We present a case of HNCE caused by chronic inflammatory demyelinating polyneuropathy with symptomatic improvement after decompressive lumbar laminectomy and dural expansion. CLINICAL PRESENTATION: A 54-year-old woman with a history of back pain since she was in her 20s presented with low back and radicular pain that had increased during a period of 6 months, bilateral lower-extremity weakness, and sensory loss in the right thigh. magnetic resonance imaging of the lumbosacral spine revealed multiple, poorly enhancing mass lesions and apparent intrathecal nerve root thickening from L1 to L5. INTERVENTION: An L1-L5 decompressive laminectomy, performed with continuous somatosensory evoked potential and electromyographic monitoring, revealed multiple segmentally enlarged nerve roots. One nerve root that did not respond to high levels of stimulation was identified. This root was resected and submitted for pathological analysis. The dura was expanded with an 11-cm-long dural patch. The pathological examination revealed hypertrophic neuropathy, with extensive S-100-positive "onion bulb" formation. The patient's symptoms improved postoperatively. CONCLUSION: HNCE is a rare disorder that can cause radicular pain and lower-extremity weakness, sensory loss, and hyporeflexia. One possible cause is demyelinating polyneuropathy. Although medical management is typically effective in the treatment of demyelinating polyneuropathy, it has little effect on compressive symptoms caused by intradural nerve root enlargement. As this case demonstrates, surgical management of symptomatic radiculopathy by lumbar laminectomy is a reasonable and effective approach to the treatment of HNCE.- - - - - - - - - - ranking = 0.51567479643176keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
9/23. Spinal and cranial hypertrophic neuropathy in multiple sclerosis.Two patients with multiple sclerosis developed symptomatic chronic inflammatory demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. sural nerve biopsy in one showed only moderate demyelination, axonal loss, and onion-bulb formation, illustrating dichotomy between severe proximal and milder distal nerve involvement. patients with coexistent central and peripheral demyelination usually are symptomatic from dysfunction at one site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in this situation must be explored further.- - - - - - - - - - ranking = 0.50645739934863keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
10/23. Chronic inflammatory demyelinating polyradiculoneuropathy: two cases with cervical spinal cord compression.Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a peripheral nerve disorder probably due to an immunological disturb. It evolves either in a steadily progressive or in a relapsing and fluctuating course. Weakness is mainly in the lower limbs proximally and distally. The electromyography is demyelinating. The cerebral spinal fluid protein is most of times elevated. Sometimes enlarged nerves are found. There are few cases described with spinal cord compression due to hypertrophic spinal nerve roots. Two patients (females, 66 and 67 years old) with diagnosis of a long standing CIDP are described. In the first one, the evolution was characterized by remission and relapsing course. The second patient had a chronic and progressive course. These patients presented after a long evolution a cervical spinal cord compression syndrome due to hypertrophic cervical roots. Neurologists must be aware of the possibility of development of spinal cord compression by enlarged spinal roots in patients with a long standing CIDP.- - - - - - - - - - ranking = 1.7583939256525keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
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