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1/29. 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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2/29. 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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3/29. 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 = 5
keywords = nerve
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4/29. Usefulness of gait analysis combined with motor point block in a stroke patient.

    This clinical note describes a typical case of dynamic varus deformity of the rear foot in a stroke patient. An overactive Tibialis Posterior muscle seemed mainly responsible for the varus deformity. However, this hypothesis was not confirmed by a motor point block of this muscle. It appeared that the Tibialis Posterior and Extensor Hallucis Longus muscles were both involved in the varus deformity. A double motor point block of both the Tibialis Posterior and Extensor Hallucis Longus muscles was performed. Kinematic and kinetic data showed improvement. This case report illustrates the usefulness of gait analysis combined with motor point block in the diagnosis and management of gait disturbance.
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5/29. 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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6/29. 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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7/29. 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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8/29. gait rehabilitation in a patient affected with charcot-marie-tooth disease associated with pyramidal and cerebellar features and blindness.

    Charcot-Marie-Tooth (CMT) disease, an inherited neuropathy characterized by length-dependent degeneration of the motor and sensory nerve fibers with consequent distal muscle atrophy and sensory reduction, can be associated with symptoms and signs of involvement of the central nervous system and/or cranial nerves. We present a patient with relatively severe CMT, cerebellar ataxia, pyramidal involvement, and blindness due to Leber's hereditary optic neuropathy. The patient presented with poor standing and gait, with consequent severe disability. Factors responsible for the patient's functional impairment (plantarflexor failure, footdrop, foot rotation, knee flexor contracture, poor proprioception, cerebellar dysfunction, spastic paraparesis, blindness) were identified and addressed by a rehabilitation management, which included, as a main intervention, ankle stabilization by drop-foot boots instead of ankle-foot orthoses. Improved balance and independent ambulation resulted from rehabilitation.
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ranking = 2
keywords = nerve
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9/29. Restoration of locomotion in paraplegics with aid of autologous bypass grafts for direct neurotisation of muscles by upper motor neurons--the future: surgery of the spinal cord?

    OBJECTIVES: paraplegia means a lifelong sentence of sensory loss, paralysis and dependence. Complete spinal cord lesions cannot heal up to now despite intensive experimental research, remarkable efforts and recent achievements in bio-technology and re-engineering. Traumatic paraplegia due to spinal cord injury (SCI) is a quite frequent condition and related to the socio-economical situation of the population. It is experienced disproportionately by young people. The rise in gunshot wounds is dramatic. SCI has appeared refractory to treatment. patients AND methods: Since 1980 G.A.B. had tried surgical repair of the spinal cord (SC) after experimental bisection in rats, and since 1993 research was done on monkeys (macaca fascicularis) to be closer to human physiology. The sciatic nerve was removed and used as an autologous graft from the lateral bundle of the spinal cord (tractus corticospinalis ventro lateralis) to the three muscles of both legs being known to be most important for locomotion: M. gluteus maximus, M. gluteus medius and M. quadriceps femoris. The first fruitful transplantation in a human being was performed in July 2000. RESULTS: The results in rats were promising and fulfilled the requirements of the American Task Force of the National Institute of Neurological and Communicative Disorders and stroke of the US. The results in monkeys confirmed the paradigm so that we performed the first operation in a young lady suffering for four months from complete SC lesion T9 after approval by the ethical committee. First voluntary movements of the connected muscles after 17 months. 27 months after op she was able to walk up to 60 steps with the help of a walker and to climb steps in the water. Improvement is still continuing. DISCUSSION: SCI has appeared refractory to any kind of treatment. Compensatory strategies are still experimental in human beings. Autologous nerve grafts from the spinal cord tissue (the lateral spinal bundle) connected to peripheral muscle nerves seem promising in paraplegics. But the physiology is still unclear when the glutamatergic upper motor neuron connected to motor end-plates (cholinergic) does work like in our patient. CONCLUSION: Further studies in primates and paraplegic patients are necessary to clarify the bypass grafting of the SC to muscle groups distal to the complete SCI to restore locomotion.
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ranking = 3
keywords = nerve
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10/29. Hereditary neuropathy with liability to pressure palsies (HNPP) in a toddler presenting with toe-walking, pain and stiffness.

    The typical clinical presentation of hereditary neuropathy with liability to pressure palsies is an adult-onset recurrent, painless monoparesis. Electrophysiological abnormalities--decreased nerve conduction velocities and delayed distal latencies--can be detected even in asymptomatic patients. We describe a toddler, who presented with asymmetric toe walking, painful cramps and stiffness in the legs. He had calf hypertrophy, brisk tendon reflexes and bilateral Babinski signs and the electrophysiological examination was normal. The unlikely diagnosis of hereditary neuropathy with liability to pressure palsies was reached 5 years later, when the boy started to complain of episodic numbness and weakness in the upper extremities. His father, paternal aunt and grandmother had similar symptoms, but they had never been investigated. The typical 1.5 Mb deletion on chromosome 17p11.2-12 was found in our patient and his affected relatives.
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