1/22. 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.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/22. 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.- - - - - - - - - - ranking = 0.88301902552493keywords = brain (Clic here for more details about this article) |
3/22. Brainstem conundrum: the Chiari I malformation.PURPOSE: To describe the Chairi I Malformation in relation to the anatomy of the brain and spinal cord, the common manifestations of the condition, diagnostic considerations, and management for the primary care provider. DATA SOURCES: Extensive review of the world-wide scientific literature on the condition, supplemented with actual case studies. CONCLUSIONS: The adult Chairi I Malformation is an insidious congenital brainstem anomaly that consists of caudal displacement of the cerebellar tonsils, brainstem and fourth ventricle into the upper cervical space, resulting in overcrowding of the posterior fossa. IMPLICATIONS FOR PRACTICE: Due to the vague, and often ambiguous presenting symptoms of Chiari I Malformation, many patients are misdiagnosed with conditions such as multiple sclerosis, fibromyalgia, chronic fatigue syndrome, or psychiatric disorders. patients frequently experience symptoms months to years prior to accurate diagnosis and often incur irreversible neurologic deficits.- - - - - - - - - - ranking = 2.6490570765748keywords = brain (Clic here for more details about this article) |
4/22. Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report.STUDY DESIGN: A case report of craniocervical spine lesions including basilar impression, atlantoaxial dislocation, and syringomyelia, with osteogenesis imperfecta is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the surgical management of craniocervical spine lesion with osteogenesis imperfecta. SUMMARY OF BACKGROUND DATA: osteogenesis imperfecta is known to have various spine lesions as complications. However, few reports have described craniocervical lesions associated with osteogenesis imperfecta. methods: A 14-year-old girl with osteogenesis imperfecta, Silence classification IVB, experienced difficulty walking, with marked motor disturbance and muscle weakness in the extremities. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities, and positive Babinski reflex and ankle clonus were observed bilaterally. Basilar impression, atlantoaxial dislocation, and syringomyelia were shown by plain radiography, tomography, three-dimensional computed tomography, and magnetic resonance imaging. RESULTS: In the reported patient, posterior fossa decompression and atlantoaxial posterior fusion could not be performed because the foramen magnum and upper cervical spine invaginated to the base of the skull. Therefore, occipitocervical spine fusion using titanium loop and wires was performed at the reduced position of the atlantoaxial dislocation, resulting in improvement of neurologic deficits. CONCLUSIONS: For patients with atlantoaxial dislocation, syringomyelia, and basilar impression without clinical symptoms or signs of brain stem compression, occipitocervical spine fusion alone at the reduction of the atlantoaxial dislocation may be indicated because these procedures improve neurologic deficits and prevent postoperative development of basilar impression and enlargement of syringomyelia.- - - - - - - - - - ranking = 0.88301902552493keywords = brain (Clic here for more details about this article) |
5/22. 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.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/22. Unusual presentation of GM2 gangliosidosis mimicking a brain stem tumor in a 3-year-old girl.We report a case of GM2 gangliosidosis revealed by MR imaging of an isolated brain stem abnormality in a 3-year-old girl referred for gait difficulties related to ataxia and pyramidal signs. Brain MR imaging displayed a brain stem lesion with high signal intensity on fluid-attenuated inversion recovery and T2-weighted images, suggesting either a tumor or an inflammatory process. Stereotactic biopsy findings showed the presence of swollen neurons with storage material in lysosomes. Enzyme study revealed deficiency of hexosaminidase a, variant B1. gangliosidoses should be considered in the differential diagnosis of isolated infiltrating brain stem lesions in childhood.- - - - - - - - - - ranking = 6.1811331786745keywords = brain (Clic here for more details about this article) |
7/22. Improved ambulation and speech production in an adolescent post-traumatic brain injury through a therapeutic intervention to increase postural control.PURPOSE: This case study examined the effectiveness of a programme designed to improve anticipatory postural control in an adolescent over years 2 and 3 post-traumatic brain injury (TBI). It was hypothesized that her difficulty in walking and talking simultaneously was caused by excessive co-activation of extremity, trunk, and oral musculature during upright activities. methods: The participant was treated weekly by physical and speech therapy. Treatment focussed on improving anticipatory postural control during gross motor activities in conjunction with oral-motor function. RESULTS: Initially, the participant walked using a walker at a speed of 23 cm s(-1). Two years later, she could walk without a device at 53 cm s(-1). Initial laryngoscopic examination showed minimal movement of the velum or pharyngeal walls; full movement was present after treatment. The measure of intelligibility improved from no single word intelligible utterances to 85% intelligible utterances after 2 years. DISCUSSION: The results suggest that less compensatory rigidification of oral musculature was needed to maintain an upright position against gravity as postural control improved. CONCLUSION: An adolescent 1-year post-TBI was followed as she underwent additional rehabilitation focussed on improving anticipatory postural control. The functional goal of simultaneously talking while walking was achieved through this intervention.- - - - - - - - - - ranking = 4.4150951276247keywords = brain (Clic here for more details about this article) |
8/22. Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a high-flow pial AVF: case report.BACKGROUND: Intracranial varices are rare and most are associated with vein of Galen arteriovenous malformations (AVM) or fistulas (AVF). DESCRIPTION: A 43-year-old left-handed man presented with right hemihypesthesia and spastic gait. Neuroradiological examination revealed a spinal AVF and a giant intracerebral varix associated with a high-flow pial AVF. He had recurrent episodes of nasal bleeding, which were also confirmed in his mother's medical history, and telangiectases in the tip of his tongue and fingers. He was diagnosed with Rendu-Osler-Weber disease. After resection of the spinal AVF that produced his symptoms, we surgically exposed and obliterated the giant varix and AVF under intra- and postoperative hypotension and mild barbiturate therapy. The arteriovenous shunt was completely obliterated without hyperperfusion of the surrounding brain. CONCLUSION: This is an extremely rare case of Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a high-flow pial AVF that did not involve the vein of Galen.- - - - - - - - - - ranking = 0.88301902552493keywords = brain (Clic here for more details about this article) |
9/22. Foot drop due to cranial gunshot wound.OBJECTIVE: We present a case of foot drop from hemorrhagic contusion after cranial gunshot, which has never been reported. methods: A 21-year-old man was admitted with inability of dorsiflexion 1 day after a tangential gunshot wound of the scalp. The scalp skin was cut by the rifle bullet. He had foot drop and his neurological examination was normal except for weakness at dorsiflexion of the right foot. Pathological reflexes and sensation failure were not detected. T1- and T2-weighted magnetic resonance images showed hyperintense contusion at the right superior frontal gyrus and mild subdural hemorrhage. peripheral nervous system examination was electrophysiologically normal. Motor-evoked potentials showed the location of the lesion at the motor cortex because no electrical record was obtained from the right anterior tibial and extensor digitorum brevis muscles, and there was a normal record on the left. Six months later, the patient's neurological examination was uneventful. CONCLUSION: When a cranial gunshot wound injury victim presents with foot drop, the central causes should be included in the differential diagnosis list.- - - - - - - - - - ranking = 0.29662211674904keywords = nervous system (Clic here for more details about this article) |
10/22. Young onset limb spasticity with PSP-like brain and spinal cord NFT-tau pathology.A 30-year-old white man presented with a sporadic form of gradually progressive spastic gait and, later, supranuclear vertical and horizontal gaze palsy, mild cognitive impairment, loss of postural reflexes, and falls. dna analysis revealed H1/H1 haplotype without tau gene (exons 9 to 13) mutation. Eight years later, postmortem revealed a tauopathy similar to progressive supranuclear palsy. Unusual aspects were early age at onset, neurofibrillary tangle, and tau involvement of the cord.- - - - - - - - - - ranking = 3.5320761020997keywords = brain (Clic here for more details about this article) |
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