Cases reported "Movement Disorders"

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1/45. Facial dyskinesia induced by auditory stimulation: a report of four cases.

    INTRODUCTION: The Vibroacoustic Disease (VAD) is an occupational pathology induced by long-term (>10 yr) exposure to large pressure amplitude (> or = 90 dB SPL) and low frequency (< or = 500 Hz) noise. During studies contributing to the characterization of VAD, abnormal facial movements induced by repetitive auditory stimuli were observed in one individual employed as an aeronautical mechanic. The goal of this study was to investigate the existence of movement disorders triggered by auditory stimuli in a group of VAD-diagnosed patients. methods: Sixty VAD patients received auditory and median nerve stimulation. Simultaneously, EEG monitoring was performed. RESULTS: Abnormal myoclonus movements were triggered by auditory stimuli in four patients. EEG recordings were normal in all 60 patients. Stimulation of the median nerves did not trigger any abnormal movements. CONCLUSION: These data suggest that this facial dyskinesia has a subcortical origin.
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2/45. Epidural spinal cord stimulation for the treatment of painful legs and moving toes syndrome.

    A case of 51-year-old male with painful legs and moving toes syndrome was successfully treated with epidural spinal cord stimulation. He was previously treated with varieties of medication, epidural block, transcutaneous nerve stimulation, lumbar sympathetic block, with no or only a transient effect. Epidural spinal cord stimulation was applied by means of percutaneously inserted epidural electrodes connected to a percutaneous extension for 2-week test stimulation period and later to a permanent device (ITREL). pain and involuntary movement were relieved almost completely during the stimulation and the effect was still evident 6 months later.
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3/45. Intralabyrinthine schwannomas.

    OBJECTIVE: To describe the patient presentation, radiographic findings, and treatment results in a series of eight patients with a diagnosis of intralabyrinthine schwannoma, and to review the presentation of other cases of intralabyrinthine schwannoma in the English otolaryngologic literature. methods: Retrospective review of patient records, operative reports, and radiologic studies, and review of the literature. RESULTS: Eight patients with a variety of otologic symptoms including progressive hearing loss, episodic vertigo, and tinnitus were found to have a schwannoma involving the vestibule or cochlea. Surgery was performed to remove the tumors from four patients with nonserviceable hearing. The patients experienced significant improvement in their vertigo and tinnitus after surgery. observation and serial magnetic resonance imaging were adequate treatment of the four patients with serviceable hearing. In the literature review, 447 cases of intralabyrinthine schwannoma were identified, and the presentations were similar to those in the cases described here. CONCLUSION: Intralabyrinthine schwannomas are rare tumors that arise from the distal portion of either the vestibular nerve or the cochlear nerve. Consequently, the cochlea, the semicircular canals, the vestibule, or a combination of these structures may become involved with these lesions. Transmastoid labyrinthectomy or a transotic approach can be used to remove intralabyrinthine tumors from patients with nonserviceable hearing and severe vertigo or tinnitus. In addition, these surgical approaches should be used if the tumor grows to involve the internal auditory canal. observation is an appropriate option for patients who have serviceable hearing.
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4/45. Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature.

    Bobble-head doll syndrome is a rare and unique movement disorder encountered in children. It is characterized by continuous or episodic involuntary forward and backward and side to side movement of the head at the frequency of 2-3 Hz. neuroimaging in most of the cases reveals third ventricular tumors, suprasellar arachnoid cysts, aqueductal stenosis and other lesions in the region of the third ventricle along with communicating hydrocephalus. In most of the circumstances, the problem starts in the first decade of life and diversion of cerebrospinal fluid by shunt operation is very often accompanied by dramatic improvement. We report one case where bobbing of the head started at around 12 years of age. Additionally, there was evidence of partial left abducens nerve palsy, tremor in the outstretched hands, difficulty in finger-nose test and tandem walking, hyperreflexia and extensor plantar response. He was unconscious on two occasions and there was evidence of gross hydrocephalus along with a thin membranous web, running transversely across the lower part of the aqueduct of Sylvius without any cerebrospinal fluid flow void. Ventriculo-peritoneal shunt abolished the abnormal movements. We propose that the aqueductal web was the offending agent for the pathogenesis of bobble-head doll syndrome in our case and this lesion has not been identified in the cases reported so far. Relevant literature in this regard has also been reviewed.
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5/45. Painful legs and moving toes syndrome associated with herpes zoster myelitis.

    A 75-year-old woman developed painful legs and moving toes syndrome (PLMT) 16 months after the onset of herpes zoster (HZ) myelitis. Although the scattered extensive lesions due to HZ myelitis were observed to be eccentric near the posterior horn on MRI, these changes had disappeared upon the development of PLMT. Combined median and tibial nerve somatosensory evoked potentials demonstrated abnormal findings only in the tibial nerve stimuli, suggesting that a severe alteration occurred in the somatosensory fibers coming selectively from the lower legs. These findings suggest plasticity in the ascending somatosensory pathway including the posterior horn cells, probably involving the interneuron networks, for the lower legs may underlie the development of PLMT associated with HZ myelitis.
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6/45. movement disorders after intervertebral disc surgery: coincidence or causal relationship?

    It is well known that brain injury or central traumatic lesions may result in the subsequent appearance of movement disorders such as dystonia or tremor. The concept that peripheral lesions to neural structures may be involved in the pathogenesis of movement disorders has been discussed controversely but has gained more widespread acceptance only recently. Here, we report on 6 patients who developed movement disorders after spinal disc surgery. The movement disorders became manifest with a delay of 1 day to 12 months after surgery. Of the six patients, 4 underwent cervical disc surgery, and 2 patients were operated on for lumbar disc herniation; 2 patients presented with paroxysmal kinesigenic segmental dystonia, 1 patient with focal dystonia, 2 with unilateral tremor, and 1 with bilateral tremor. The appearance of the movement disorder was associated with persistent dermatomal or segmental pain. In all patients, the anatomic distribution of the movement disorder was related to the nerve root or spinal segment of the corresponding disc level and the manifestation was in close temporal relation to the surgery. We conclude that spinal disc surgery may be another, thus far neglected, cause for movement disorders. The postoperative pain syndrome in all patients should be considered as an important factor of pathogenesis. overall, movement disorders associated with disc surgery appear to be rare, yet they may cause significant discomfort to the affected individual.
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7/45. Etiological and therapeutical observations in a case of belly dancer's dyskinesia.

    We report on the case of a woman with belly dancer's syndrome. This case presented two peculiarities: (1) the condition was induced by the chronic use of clebopride, and (2) abdominal dyskinesias showed a dramatic response to the application of transcutaneous electrical nerve stimulation.
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8/45. Superficial siderosis of the central nervous system following cervical nerve root avulsion: the importance of early diagnosis and surgery.

    Superficial siderosis (SS) of the central nervous system is an insidious, progressive, irreversible and debilitating neurological disorder caused by recurrent haemorrhage within the subarachnoid space. The subsequent deposition of haemorrhagic breakdown products in the spinal cord and nervous tissues leads to the loss of neurones and myelin, and to the development of a neurological deficit. In a small number of patients, the source of haemorrhage is related to traumatic cervical nerve root avulsion occurring several years prior to the onset of symptoms. Surgical ablation of the source has been shown to halt the progression of the disease, at least in the short term. We review the literature on SS secondary to cervical nerve root avulsion and report a further case in which surgical management was successful in halting disease progression. We emphasize that early detection and recognition of the initial non-progressive symptoms related to this poorly known disease, coupled with timely surgical management, minimizes the degree of neurological disability.
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9/45. Bilateral profound hearing loss due to meningeal carcinomatosis.

    meningeal carcinomatosis (MC) is an uncommon form of metastasis of solid tumors. hearing loss as the presenting symptom of MC is very uncommon. A patient with an esophageal signet ring cell carcinoma 3 years previously presented with sudden onset of profound hearing loss affecting both ears. He had no evidence of local tumor recurrence. brain magnetic resonance imaging (MRI) showed swelling and increased signal intensity on T2 weighted images of both acoustic nerves and the right trigeminal nerve. After gadolinium administration, enhancement of both acoustic and trigeminal nerves was seen. He later developed unsteadiness and head-movement-dependent oscillopsia due to vestibular areflexia and diplopia. At that time MRI showed leptomeningeal enhancement. MC was diagnosed, although cerebrospinal fluid cytology could not confirm that diagnosis. The patient died 16 weeks after the onset of deafness. In patients with progressive unilateral and bilateral hearing loss, meningeal carcinomatosis should be considered, especially if there is a history of previous malignancy.
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10/45. A case of adult onset pure pallidal degeneration. II. Analysis of neurotransmitter markers, with special reference to the termination of pallidothalamic tract in human brain.

    We analyzed neurotransmitter markers in a brain of a very rare case of pathologically confirmed adult-onset pure pallidal degeneration (PPD) as compared with 16 controls. Neurotransmitter concentrations are significantly altered in the globus pallidus (GP), subthalamic nucleus (ST) and the thalamic nuclei. Concentrations of gamma-aminobutyric acid (GABA) in the external segment (GPe) and internal segment (GPi) of GP and ST are decreased to 62, 45 and 55% of the control mean, respectively. Concentrations of glutamic acid are increased in GPi (144%) and ST (134%). choline acetyltransferase (ChAT) activities are increased in GPe (232%), GPi (218%), ST (161%), and ventroanterior (VA, 210%) and ventrolateral nucleus (VL, 193%) of the thalamus. Noradrenaline (NA) concentrations in GPe and GPi are 56 and 43% of the control mean, respectively. Dopaminergic and serotonergic systems show no remarkable change. The grid microdissection analysis demonstrates a patchy GABA distribution in the thalamus of 3 controls, whereas a small GABA-rich area in the ventro-oral nucleus (VO) according to the atlas of Hopf disappears in adult onset PPD. These results strongly suggest that (1) GP gabaergic neurons are selectively degenerated and striatopallidal GABAergic nerve terminals are hypoactive; (2) ChAT activities in GP, ST, VA and VL are increased; (3) the subthalamopallidal glutamatergic system is not hypoactive; (4) activity of the noradrenergic system in GP is decreased; and that (5) VO in the thalamus specifically receives GABAergic nerve terminals from GP in human brain.
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