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1/42. Subacute sensory neuropathy associated with Epstein-Barr virus.

    A 35-year-old man experienced severe sensory loss, pseudoathetosis, and areflexia during recovery from a severe viral illness. Sensory nerve action potentials were absent, motor conduction velocities were mildly slowed, and blink reflexes were normal. magnetic resonance imaging (MRI) revealed abnormal signal within the central and dorsal aspects of the thoracic cord. Acute and convalescent Epstein-Barr virus (EBV) titers suggested EBV as the etiology. Subacute sensory neuropathy, with peripheral and central nervous system involvement, is a rare complication of EBV infection.
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ranking = 1
keywords = neuropathy, peripheral, nerve, nervous system
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2/42. iliac artery aneurysm causing isolated superior gluteal nerve lesion.

    Lesions of the superior gluteal nerve (SGN) lead to weakness of hip abduction, manifesting itself as a gait abnormality, with contralateral tilting of the pelvis with each step. Causes are numerous and may occur at different anatomical locations before the nerve enters the suprapiriform foramen, in the foramen itself, or after the nerve has exited the foramen. This case report describes an SGN lesion by a large iliac artery aneurysm in a patient presenting with a gait disorder.
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ranking = 0.26406590526607
keywords = nerve
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3/42. Interfacing the body's own sensing receptors into neural prosthesis devices.

    Functional electric stimulation (FES) is today available as a tool in muscle activation used in picking up objects, in standing and walking, in controlling bladder emptying, and for breathing. Despite substantial progress over nearly three decades of development, many challenges remain to provide a more efficient functionality of FES systems. The most important of these is an improved control of the activated muscles. Instead of artificial sensors for feedback, new developments in electrodes to do long-term and reliable recordings from peripheral nerves emphasize the use of the body's own sensors. These are already installed and optimised through millions of years of natural evolution. This paper presents recent results on a system using electrical stimulation of motor nerves to produce movement and using the natural sensors as feedback signals to control the stimulation that can replicate some of the functions of the spinal cord and its communication with the brain. We have used the nerve signal recorded from cutaneous nerves in two different human applications: (1) to replace the external heel switch of a system for correction of spastic drop foot by peroneal stimulation, and (2) to provide an FES system for restoration of hand grasp with sensory feedback from the fingertip. For the bladder function, the sacral root stimulator is a useful control tool in emptying the bladder. To decide when to stimulate, we are at present carrying out experiments on pigs and cats using cuff electrodes on the pelvic nerve and sacral roots to record the neural information from bladder afferents. This information can potentially be used to inhibit unwanted bladder contractions and to trigger the FES system and thereby bladder emptying. Future research will show whether cuffs and other types of electrodes can be used to reliably extract signals from the large number of other receptors in the body to improve and expand on the use of natural sensors in clinical FES systems.
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ranking = 0.32711215532994
keywords = peripheral nerve, peripheral, nerve
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4/42. A case of peroneal neuropathy-induced footdrop. Correlated and compensatory lower-extremity function.

    This article reports on the case of a man with peroneal neuropathy-induced footdrop who was seen at the authors' institution 3 years after open reduction and internal fixation of a proximal fibular fracture and a distal, spiral, oblique tibial fracture of the right leg. A comprehensive gait analysis was conducted. A significant footdrop in gait resulted in a "reverse check mark" center-of-pressure pattern, an increased transverse-plane rotation of the foot, and excessive knee and hip flexion in the sagittal plane. These objective findings documented significant dysfunction within the involved lower extremity; in addition, aberrant biomechanics were observed in structures other than the site of initial injury within both limbs.
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ranking = 0.88515925672165
keywords = neuropathy
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5/42. Primary pelvic hydatid cyst: an unusual cause of sciatica and foot drop.

    STUDY DESIGN: A case report of primary pelvic hydatid cyst causing sciatica and foot drop. OBJECTIVE: To document the occurrence of primary pelvic hydatid cyst as one of the hidden causes of lower limb weakness and foot drop, and to recommend inclusion of the pelvic cavity when assessing sciatica and foot drop. SUMMARY OF BACKGROUND DATA: It is common to see foot drop caused by peripheral lesions around the knee or disc herniation in the lumbar spine, but if these sites were excluded, the pelvic cavity must be examined for hidden disease that may explain the cause of foot drop and sciatica. methods: The authors involved in the care and management of this patient were interviewed and all medical records, radiologic investigations, and related literature were reviewed. RESULTS: After exclusion of spinal and peripheral causes of foot drop, computed tomography of the pelvis showed a well-localized cystic swelling in the right side of the pelvis over the lumbosacral plexus roots. Surgical excision of the cyst resulted in partial recovery of the foot drop at 3 years of follow-up. CONCLUSION: Primary pelvic hydatid cyst rarely causes pressure on the lumbosacral plexus. This was a case of hydatid cyst in the pelvis causing sciatica and foot drop, and it indicates the pelvis as a hidden source of sciatica and foot drop. After surgical excision followed by 4 months' mebendazole therapy, there was no evidence of recurrence on long-term follow-up.
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ranking = 0.11661332785786
keywords = peripheral
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6/42. myasthenia gravis presenting as a unilateral abducens nerve palsy.

    A 48-year-old man with no significant medical history presented to the emergency department of a large, urban, tertiary-care hospital complaining of double vision and unsteady gait. physical examination was remarkable for an isolated, unilateral sixth nerve palsy. After comprehensive testing, a final diagnosis of myasthenia gravis was made. The patient's unsteady gait was a manifestation of sensory distortion from the diplopia and corrected when the patient closed his eyes. The presentation of myasthenia gravis as an isolated sixth nerve palsy is unprecedented in the emergency medicine literature.
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ranking = 0.22634220451378
keywords = nerve
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7/42. The giant axonal neuropathy--clinical and hisotological aspects, differential diagnosis and a new case.

    The giant axonal neuropathy (GAN) is morphologically characterized by axonal swellings and accumulations of neurofilaments in giant axons and other cell types. Curly hair is not a constant finding. The clinical course is progressive and mostly starts in early childhood. We report the case of a boy aged 6 years at the time of sural nerve and muscle biopsy. Suralis nerve showed a reduced numerical density of myelinated fibres with a consecutive endoneural fibrosis. Morphometric investigation revealed a pronounced reduction of fibres measuring 8-12 microm in diameter. Giant axons were seen in relatively low number and were not very large with a maximum diameter of 18 microm. They had a relatively thin myelin sheet proved also by the high G ratio in the histogram. Many onion bulb formations of schwann cells were present. There are only few reports of giant axons with such low maximum diameter in cases with GAN, the lowest maximum diameters being reported in case reports on Japanese children. Up to now, this is the first report of a non-Japanese patient with a low maximum diameter of giant axons of less than 20 microm in peripheral nerve biopsy. Ultrastructurally, typical accumulations of neurofilaments and osmiophilic aggregates were found in giant axons. Other diagnoses with occurrence of giant axons could be excluded in view of the absence of specific findings. Sporadic or familial cases with giant axons are discussed. Sceletal muscle biopsy (M. quadriceps femoris) showed neurogenic affection with presence of small angulated atrophic muscle fibres.
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ranking = 1.136824010547
keywords = neuropathy, peripheral nerve, peripheral, nerve
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8/42. Severe sensory ataxia and demyelinating polyneuropathy with IgM anti-GM2 and GalNAc-GD1A antibodies.

    Several polyneuropathy syndromes have been described with polyclonal serum immunoglobulin g (IgG) or immunoglobulin m (IgM) binding to gangliosides GM2 and GalNAc-GD1a that contain the terminal trisaccharide moiety GalNAc(beta1-4)Gal(alpha2-3)NeuAc. We describe the clinical and electrodiagnostic features in two patients with serum IgM monoclonal anti-GM2 and anti-GalNAc-GD1a antibodies. These patients had slowly progressive, panmodal sensory loss with severe sensory ataxia. Electrodiagnostic testing showed demyelinating features. Prominent improvement in gait ataxia occurred after treatment with human immune globulin but not after other immunomodulating therapies. Enzyme-linked immunoabsorbent assay and thin-layer chromatography demonstrate that the patient's serum monoclonal IgM bound to gangliosides GM2 and GalNac-GD1a but not to gangliosides without the GalNAc(beta1-4)Gal(alpha2-3)NeuAc moiety. This neuropathy differs from previously reported neuropathy syndromes associated with polyclonal GM2 and GalNAc-GD1a antibodies and from other chronic demyelinating polyneuropathies. We conclude that a distinct syndrome of chronic demyelinating neuropathy with sensory ataxia, unresponsive to corticosteroids, is associated with monoclonal IgM binding to gangliosides with a terminal GalNAc(beta1-4)Gal(alpha2-3)NeuAc trisaccharide moiety. diagnosis of this syndrome is important to direct appropriate treatment.
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ranking = 1.4162548107546
keywords = neuropathy
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9/42. Multimodality management of a giant cell tumor arising in the proximal sacrum: case report.

    STUDY DESIGN: Descriptive. OBJECTIVE: To outline a novel multimodality approach for a difficult surgical resection of a giant cell tumor in the cephalad portion of the sacrum. SUMMARY OF BACKGROUND DATA: giant cell tumors of the sacrum are rare primary bone tumors. Recent reports have demonstrated diminished giant cell tumor recurrence with cryosurgery by using a "direct pour" technique with liquid nitrogen. Although successful in decreasing tumor recurrence, this technique is accompanied by a 4%-8% rate of skin necrosis and high rates of pathologic fracture. The authors describe resection and a novel, controlled method of argon-based cryotherapy (followed by a unique pelvic reconstruction) for a large, difficult giant cell tumor of the sacrum. methods: A 29-year-old woman presented with complaints of right foot drop and decreased sensation of the right buttock, posterior thigh, posterior calf, and lateral aspect of the right foot. Radiographic evaluation revealed a mass in the right sacrum; histologic examination of CT-guided biopsy revealed a giant cell tumor. A combined anterior abdominal and posterior sacral approach was performed, the tumor was resected, and the margin of the cavity was treated with controlled argon-based cryotherapy. The combination of thermocouples, electromyographic monitoring, and rapid freeze-thaw cycles allowed a controlled ablation of the tumor margin while ensuring that surrounding structures, such as the rectal wall, sacral nerves, and gluteal muscles, were not damaged. Posterior spinal fusion L4 to sacrum, posterior spinal instrumentation L4 to pelvis, and allograft reconstruction of the right sacrum were performed. RESULTS: The patient recovered well without skin necrosis or pathologic fracture. Urinary and fecal continence were preserved. At the 20-month follow-up the patient has no evidence of local tumor recurrence and is fully ambulatory without a brace or narcotic medication. CONCLUSION: A novel multimodality approach, consisting of resection, controlled cryosurgery, and a unique lumbopelvic reconstruction, was safe and successful in managing a challenging proximal sacral giant cell tumor. Twenty months after surgery the patient has excellent bowel and bladder control, no tumor recurrence, and functional ambulation without a brace or pain.
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ranking = 0.037723700752296
keywords = nerve
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10/42. X-linked charcot-marie-tooth disease caused by a novel point mutation in the connexin-32 gene.

    We report the clinical and electrophysiological findings of a patient with X-linked charcot-marie-tooth disease and a novel point mutation in the connexin-32 gene. A 31-year-old man presented with a 5 year history of progressive imbalance and distal weakness in his legs. Electrophysiological studies confirmed an asymmetric, predominantly axonal sensorimotor neuropathy with some demyelinating features. genetic testing revealed a G/A transition (Ala40Thr) in a conserved transmembrane region of the connexin-32 gene.
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ranking = 0.17703185134433
keywords = neuropathy
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