1/13. Cerebral arteriovenous malformations and movement disorders.A series of six patients with movement disorders associated with cerebral arteriovenous malformations (AVM) is reported. The AVMs were classified according to the Spetzler-Martin classification as grade V (one patient), grade IV (four patients), and as grade III (one patient). One patient had action-induced hemidystonia caused by a contralateral frontoparietal AVM which compressed the putamen and was supplied partially by enlarged lenticulostriate arteries. Two patients presented with unilateral cortical tremor associated with contralateral high-frontal cortical/subcortical AVMs sparing the basal ganglia. Another patient developed hemidystonia and hemichorea-hemiballism after bleeding of a contralateral temporooccipital AVM and subsequent ischemia. Two patients had focal dystonia after thalamic and basal ganglia hemorrhage from AVMs. Five patients were operated on. The movement disorder was abolished in one patient postoperatively. Different mechanisms were identified that are relevant for the development of AVM-related movement disorders: mass effect, diaschisis, local parenchymal altered cerebral blood flow, and hemorrhagic or ischemic structural lesions.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/13. 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 = 1.2380087340092keywords = nervous system (Clic here for more details about this article) |
3/13. 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 = 0.8keywords = malformation (Clic here for more details about this article) |
4/13. 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 = 1.2380087340092keywords = nervous system (Clic here for more details about this article) |
5/13. 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 = 0.2keywords = malformation (Clic here for more details about this article) |
6/13. A case of Arnold-Chiari syndrome with flaccid paralysis and huge syringomyelia.STUDY DESIGN: A case report. SETTING: Department of Orthopaedic Surgery, Shiga University of Medical science, japan. PATIENT: A 13-year-old woman presented progressive weakness in the lower extremities, with predominance on the right. Magnetic resonance (MR) imaging revealed a huge syrinx. The patient also showed scoliosis, cleft palate, hearing impairment, excessive sweating, hairiness, dural ectasia, and malformation of the skull. METHOD AND OBJECTIVES: We treated a very rare case of Arnold-Chiari syndrome, which presented with flaccid paralysis. methods of differential diagnosis and suitable treatment are discussed. RESULTS AND CONCLUSION: Both the syrinx and muscle strength were quickly improved following placement of a syringo-peritoneal (S-P) shunt, after which the patient recovered the ability to walk. However, transient hypesthesia in the right hand occurred after the operation. The syrinx around the conus was thought to play a crucial role in the etiology of the patient case, which showed unique symptoms.- - - - - - - - - - ranking = 0.2keywords = malformation (Clic here for more details about this article) |
7/13. 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 = 1.2380087340092keywords = nervous system (Clic here for more details about this article) |
8/13. central nervous system superficial siderosis following spinal surgery.Superficial siderosis of the central nervous system is a rare progressive disease associated with hemosiderin deposition on the leptomeninges of the neuraxis. In addition to tumors and vascular lesions, dural sleeve pseudomeningoceles caused by brachial plexus avulsion injury may be the bleeding source in this disease. The authors describe a patient who underwent anterior cervical spine surgery for spinal cord compression due to the ossification of posterior longitudinal ligament. The operation was complicated by a dural tear and subsequent psedomeningocele formation. Nine years later, this patient developed superficial siderosis. The possible mechanisms involved in the development of superficial siderosis in this patient will be discussed.- - - - - - - - - - ranking = 6.1900436700459keywords = nervous system (Clic here for more details about this article) |
9/13. Acute exertional compartment syndrome in the setting of anabolic steroids: an unusual cause of bilateral footdrop.Acute exertional compartment syndrome is the result of muscle ischemia within a tight fascial compartment. We report a 22-year-old boxer, with recent intake of anabolic steroids, who developed acute exertional compartment syndrome of the lower legs following an assault from which he had to run away. He presented with bilateral footdrop. Nerve conduction studies (NCS) and electromyography (EMG) were consistent with bilateral deep and superficial peroneal neuropathies, but magnetic resonance imaging (MRI) demonstrated hemorrhagic necrosis of the pretibial muscles. This case illustrates that the differential diagnosis for footdrop includes not only central and peripheral nervous system and muscle causes, but also compartment syndromes.- - - - - - - - - - ranking = 1.2380087340092keywords = nervous system (Clic here for more details about this article) |
10/13. Extradural spinal arachnoid cysts associated with spina bifida occulta.INTRODUCTION: Spinal extradural arachnoid cysts are an uncommon cause of neural compression in children. Even more uncommon is the association of such cysts with spina bifida occulta. MATERIAL: Two girls, 12 and 8-years-old, presented with left leg pain, deteriorating gait, clinical signs of left L5 and S1 root compression, without bladder or bowel symptoms. The first patient had left foot drop. The second patient had muscle wasting and smaller left foot with pes cavus. Radiographs showed spina bifida occulta of S1 in both. MRI revealed an extradural cyst at the S1 level, indenting the thecal sac and the L5 and S1 roots. At operation in both patients a large arachnoid cyst arising from a small dural defect in the axilla of the left S1 root was compressing and displacing it and the dural sac. It was removed and the defect was repaired. The first patient improved with complete recovery of the foot drop. An MRI at 12 months showed no cyst recurrence. The second patient made good recovery initially, but at 10 months developed recurrent symptoms. An MRI scan showed recurrence of the cyst with root compression. On repeat exploration a different dural defect was identified in a more anterior position and was repaired. DISCUSSION: The coexistence of extradural arachnoid cyst and corresponding bifid spinal segment has not been described previously. It raises the suspicion that the dural defect giving rise to the arachnoid cyst may be due to segmental dural dysgenesis in the context of the dysrhaphic neuroectodermal malformation.- - - - - - - - - - ranking = 0.2keywords = malformation (Clic here for more details about this article) |
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