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1/17. 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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keywords = ataxia
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2/17. Occurrence of subdural hematoma and resolution of gait disturbance in a patient treated with shunting for normal pressure hydrocephalus.

    A 66-year-old man with gait disturbance was diagnosed with normal pressure hydrocephalus (NPH) and treated with ventriculoperitoneal shunting using a programmable valve. The valve ultimately set at a pressure of 40 mm H(2)O after higher settings no longer relieved symptoms. However, this pressure setting was excessively low and was associated with occurrence of bilateral subdural hematomas. Paradoxically, this event was associated with stable improvement of gait. Our patient's gait disturbance was unassociated with muscle weakness, spasticity, cerebellar ataxia, or Romberg's sign, and, therefore, was consistent with a frontal gait disorder. Cerebral cortical blood flow as measured after shunting by single photon emission computed tomography (SPECT) was slightly increased from the value before shunting, possibly because of intracranial hypotension related to the valve setting. Lasting improvement of gait in our case may be a result of increased blood flow in the supplementary motor area (SMA).
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ranking = 0.39107328502445
keywords = cerebellar ataxia, ataxia
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3/17. A child with miller fisher syndrome.

    We describe the case of a six-year-old girl who presented with a 3-day history of diplopia and gait disturbance following a febrile flu-like illness. On examination she was found to have ataxia, areflexia and ophthalmoplegia, and a diagnosis of miller fisher syndrome was made after the exclusion of other conditions. This report outlines the frequency of miller fisher syndrome and lists the differential diagnoses that should be considered in australia. In addition, the occurrence of pupillary dysfunction is discussed.
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ranking = 0.14285714285714
keywords = ataxia
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4/17. Axial lateropulsion as a sole manifestation of lateral medullary infarction: a clinical variant related to rostral-dorsolateral lesion.

    A 63-year-old woman presented with an isolated axial lateropulsion as a sole manifestation of lateral medullary infarction. She had no vertigo, nystagmus, dysphagia, hiccup, facial/hemisensory loss, horner syndrome, and limb ataxia. Brain MRI showed a small infarct selectively involving the most dorsolateral portion of the rostral medulla. This patient illustrates that lateral medullary infarction may present as an isolated lateropulsion. The possible mechanism of an isolated lateropulsion is described.
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ranking = 0.14285714285714
keywords = ataxia
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5/17. 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 = 0.39107328502445
keywords = cerebellar ataxia, ataxia
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6/17. 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.
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ranking = 0.14285714285714
keywords = ataxia
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7/17. Intraspinal synovial cyst at the craniocervical junction.

    A cystic lesion adjacent to the dens with compression of the lower portion of the medulla oblongata was found on MRI in a 75-year-old male patient with a 2-month history of occipital pain and gait disturbance. Clinically, the patient showed mild tetraparesis, signs of spinal ataxia and symmetrical hyperreflexia. Following subtotal removal of the cyst via left-sided suboccipital craniotomy and left-sided hemilaminectomy of C1 the lesion was classified as synovial cyst on histopathological examination. Postoperatively, the quadriparesis almost completely subsided and the patient is currently doing well, 33 months after surgery. Synovial or ganglion cysts adjacent to the atlantoaxial articulation with ventral compression of the cervicomedullar cord represent rare surgical or radiological entities. Atlantoaxial synovial cysts have no typical radiographic appearance or specific neurological symptoms so that they are frequently misdiagnosed as intraspinal- or skull base tumour, rheumatoid lesion or ectatic vertebral artery. Since no ensuing complications or recurrences have been encountered in cases of incompletely removed cysts the less invasive operative approaches should be used to avoid destabilisation and subsequent additional surgical procedures.
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ranking = 0.14285714285714
keywords = ataxia
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8/17. Delayed manifestation of spinal metastasis: a special feature of hemangiopericytoma.

    Metastatic hemangiopericytoma to the spine is rare, and operative management of these highly vascularized tumors is challenging. review of the literature identified only seven previously reported cases. Metastases of hemangiopericytomas to the spine are diagnosed often only after a long delay following resection of the primary tumors. To emphasize the clinical and histopathological features of metastatic hemangiopericytoma to the spine and to review treatment options, a case of a delayed metastatic hemangiopericytoma to the cervicothoracic spine is reported: a 48-year-old woman presented with a large metastatic hemangiopericytoma to the cervicothoracic spine causing left-sided cervicobrachialgias and gait disturbance. magnetic resonance imaging studies revealed a large left-sided dumbbell-shaped intraspinal and extraspinal tumor from C6 to T2. The patient underwent two-staged total removal of the cervicothoracic mass with posterior stabilization and subsequent radiotherapy. Histopathological findings revealed a malignant hemangiopericytoma which had identical features to the primary cranial meningeal tumor removed 8 years earlier. The radicular symptoms and the gait ataxia disappeared, postoperatively. One year later, however, new metastases were present. patients with hemangiopericytoma should be controlled regularly for local recurrence and systemic tumor spread. The best available treatment for delayed metastasis to the spinal column is complete tumor removal followed by postoperative radiotherapy.
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ranking = 0.14285714285714
keywords = ataxia
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9/17. Successful treatment by spinal cord stimulation for gait disturbance in a patient with diffuse axonal injury.

    The authors present a case of diffuse axonal injury (DAI) treated by cervical spinal cord stimulation (C-SCS) for gait disturbance. The patient had right hemiparesis of moderate degree, mild ataxia, ideational apraxia and gait disturbance, when admitted to our hospital for rehabilitation. He could not walk by himself, nevertheless neurorehabilitation was done for four months. xenon-CT was examined by C-SCS loading and the changes of regional cerebral blood flow were significantly increased in both hemispheres, especially in the thalamus. C-SCS was performed continuously on condition of 25 Hz, 200 microsec and 0.5 V, daily for a month. Neurological deficits, especially gait disturbance due to ideational apraxia, were gradually improved after initiation of C-SCS, and the patient could walk by himself. We speculate that C-SCS played a role in triggering improvement of gait disturbance at the chronic stage in our case, and SCS may be helpful for neurorehabilitation of focal symptoms after DAI.
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ranking = 0.14285714285714
keywords = ataxia
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10/17. Identification of a SACS gene missense mutation in ARSACS.

    The authors describe two patients in a Japanese family with autosomal recessive spastic ataxia of Charlevoix-Saguenay. They presented early onset spastic ataxia, sensorimotor neuropathy, nystagmus, slurred speech, and hypermyelinated retinal nerve fibers. The authors identified a homozygous missense mutation (T7492C) in the SACS gene, which resulted in the substitution of arginine for tryptophan at amino acid residue 2498 (W2498R).
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ranking = 0.28571428571429
keywords = ataxia
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