Cases reported "Chronic Disease"

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1/391. Chronic motor axonal neuropathy: pathological evidence of inflammatory polyradiculoneuropathy.

    Chronic immune and inflammatory motor neuropathies may resemble motor neuron disease, and the distinction may be particularly difficult if conduction block or GM1 antibodies are absent. The pathology of this axonal type of chronic motor neuropathy has not been characterized except in a few cases associated with paraproteinemia. We describe the clinical, electrophysiological, and pathological findings in a patient with a chronic motor axonal neuropathy, normal immunoelectrophoresis, and no GM1 antibodies. At autopsy the spinal cord was normal with the exception of chromatolytic motor neurons. All the ventral roots were greatly thinned. Of 10 mixed nerves and numerous spinal roots sampled, five showed areas of perineurial, perivascular lymphocytic infiltration. There was severe axonal loss in the motor roots that was not as evident in mixed nerves, and the sensory nerves and roots were virtually unaffected. Our findings suggest that a chronic motor axonal neuropathy without paraproteinemia or GM1 antibodies may, in some cases, result from an inflammatory process.
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keywords = nerve, block
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2/391. Chronic axonal sensory and autonomic polyneuropathy without motor involvement: a new 'chronic inflammatory neuropathy?'.

    We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow-up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the 'chronic inflammatory neuropathies' are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area.
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ranking = 0.53927882403541
keywords = nerve
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3/391. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.

    The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.
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ranking = 1.6178364721062
keywords = nerve
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4/391. The fourth-compartment syndrome: its anatomical basis and clinical cases.

    We propose a new term, the "fourth-compartment syndrome" to describe chronic dorsal wrist pain of the fourth compartment. Five main causes responsible for this syndrome are thought to be as follows: 1. Ganglion involvement, including an occult ganglion; 2. Extensor digitorum brevis manus muscle; 3. Abnormal extensor indicis muscle; 4. Tenosynovialitis; 5. Anomaly or deformity of carpal bones. Should the above mentioned conditions occur in the fourth compartment, pressure within the fourth compartment increases, ultimately compressing the posterior interosseous nerve directly or indirectly. Anatomical studies of the fourth compartment of the wrist and the posterior interosseous nerve are presented and the fourth-compartment syndrome is summarized with twelve clinical cases (six cases of occult ganglions, two cases of extensor digitorum brevis manus, two cases of tenosynovialitis, one case of abnormal extensor indicis muscle, and one case of carpal bossing).
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ranking = 0.53927882403541
keywords = nerve
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5/391. retinitis pigmentosa, nanophthalmos, and optic disc drusen: a case report.

    OBJECTIVE: Although the associations of retinitis pigmentosa (RP) with nanophthalmos/microphthalmos and RP with optic disc drusen have previously been recognized, the concurrence of all three features, as far as the authors are aware, has not previously been reported. DESIGN: Case report. RESULTS: The authors report a sporadic case of nanophthalmos, RP, and optic nerve drusen with the additional complication of chronic angle closure glaucoma. CONCLUSIONS: Visual loss may be secondary to the complications of nanophthalmos, RP, or optic nerve drusen. Chronic angle closure may be caused by choroidal effusion with serous retinal detachment, which may, in turn, cause a pseudo-RP picture. It is therefore important to recognize the possible association of true RP with nanophthalmos as a cause for visual deterioration.
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ranking = 0.53927882403541
keywords = nerve
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6/391. sciatic neuropathy secondary to a uterine fibroid: a case report.

    Lesions of the sciatic nerve outside the pelvis have been well described. Lesions within the pelvis, however, are far less common. We report the case of a 55-yr-old woman with a history of chronic low back pain who presented with progressive right buttock and posterolateral right lower limb pain associated with right foot numbness and tingling. She denied any associated low back or left lower limb pain. The patient was initially treated for a probable right lumbosacral radiculopathy, without improvement. A subsequent magnetic resonance image of the lumbosacral spine revealed multilevel disc degeneration at L3-4 through L5-S1, without disc herniation or canal stenosis. A magnetic resonance image of the pelvis revealed a markedly enlarged uterus, with a large pedunculated myoma impinging on the right sciatic foramen. The patient underwent a subtotal abdominal hysterectomy, with resolution of her right lower limb pain. This case illustrates the importance of considering intrapelvic causes of sciatic neuropathy. To our knowledge, this is the first reported case of sciatic neuropathy secondary to a uterine fibroid.
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ranking = 0.26963941201771
keywords = nerve
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7/391. Spontaneous CSF otorrhea caused by abnormal development of the facial nerve canal.

    In two patients with surgically proved CSF fistula through the facial nerve canal, MR and CT examinations showed smooth enlargement of the geniculate fossa with CSF signal. In the clinical setting of CSF otorrhea or rhinorrhea, the presence of an enlarged labyrinthine facial nerve canal and enlarged geniculate fossa on CT scans and CSF intensity on MR images strongly suggests a CSF fistula through the facial nerve canal.
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ranking = 1.8874758841239
keywords = nerve
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8/391. optic atrophy and chronic acquired polyneuropathy.

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, multifocal disorder usually defined as limited to the peripheral nervous system. Multifocal motor neuropathy, an acquired demyelinating neuropathy with conduction block affecting motor neurons only, may be a pathogenically distinct syndrome or a predominantly motor variant of chronic inflammatory demyelinating polyneuropathy. central nervous system demyelination including optic neuropathy has been reported uncommonly previously in these entities. We report two cases and review the literature on the possible association of optic neuropathy and chronic acquired polyneuropathy.
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ranking = 0.19108176394688
keywords = block
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9/391. Gustatory otalgia and wet ear syndrome: a possible cross-innervation after ear surgery.

    HYPOTHESIS: The chorda tympani and Arnold's nerves have close approximation to each other and their cross-innervation is possible after ear surgery. STUDY DESIGN: A retrospective study was performed with a temporal bone pathology case and two clinical cases as representatives of such a possibility. patients had severe otalgia and wet ear during gustatory stimulation. methods: A temporal bone pathology case was studied under a light microscope. earache and/or wet ear were provoked during gustatory stimulation. Wet ear was tested with iodine-starch reaction after the subject tasted lemon juice. RESULTS: The temporal bone specimen has clusters of regenerated fibers in the tympanic cavity in the area of the chorda tympani and Arnold's nerves, suggesting a possibility of mixing. There are regenerated fibers in the iter chordae anterius, showing successful bridging of the chorda tympani nerves across a long gap. Detachment of the skin over the operated mastoid bowl obscured signs in one clinical case. Another clinical case of gustatory wet ear showed objective evidence of cross-innervation with iodine-starch reaction. CONCLUSION: The detachment procedure and iodine-starch reaction were the proofs that the signs were related to regenerated fibers. This is the first report of gustatory otalgia and wet ear after ear surgery.
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ranking = 0.80891823605312
keywords = nerve
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10/391. Progressive catatonia.

    We present the case of a young man with a diagnosis of a childhood-onset pervasive developmental disorder who developed a progressive neurologic deterioration with persistent catatonia and right hemiparesis. On his initial evaluation approximately three years after the onset of mutism, he manifested right hemiparesis and catalepsy. Two years later, although catalepsy had subsided, motor function had deteriorated so that he could not use his hands to feed or dress himself. Oral-facialbuccal dyskinesia manifested by blepharospasm and grimacing were present constantly during waking hours. Quantitative electroencephalography demonstrated markedly decreased amplitude, a finding associated with catatonia. Left sural nerve biopsy indicated large axon cylinder degeneration. Left deltoid biopsy demonstrated perimysial fibrosis and type II fiber predominance. Although magnetic resonance imaging of the head without contrast was normal, positron emission tomography indicated hypometabolism of the right cerebral and the right cerebellar hemispheres. The patient continues to deteriorate despite a course of 25 electroconvulsive treatments. He continues to manifest criteria for catatonia including motoric immobility, mutism, and peculiarities of voluntary movement such as prominent grimacing. We suspect an inherited neurodegenerative disorder. Since catatonia is a treatable condition frequently associated with medical and neurological diseases, examination for the features of catatonia must be included in the assessment of patients with progressive brain degeneration. This report is an attempt to clarify the traits of a serious variant of progressive brain degeneration.
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ranking = 0.26963941201771
keywords = nerve
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