Cases reported "Gait Ataxia"

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11/22. MR imaging findings of spinal posterior column involvement in a case of miller fisher syndrome.

    Summary: The site of lesions causing ataxia in miller fisher syndrome (MFS) remains in dispute. A 43-year-old man manifested rapidly progressive left-sided ptosis, bilateral abducens palsy, areflexia, and severe ataxia. Initial MR imaging showed confined lesions of the cauda equina with gadolinium enhancement. A diagnosis of MFS was made, and the patient underwent immunotherapy. His ophthalmoplegia disappeared, but other symptoms remained. Five months after onset, MR imaging disclosed lesions confined to the spinal posterior column, which were considered to result from involvement of posterior nerve roots of the cauda equina and to be responsible for his remaining severe ataxia.
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12/22. GALOP syndrome: case report with 7-year follow-up.

    An elderly woman complaining of a gait disorder was found to have the GALOP syndrome (gait ataxia, late-onset polyneuropathy). She exhibited mild distal weakness and sensory loss in the legs, a positive Romberg, and an unsteady gait. serum immunofixation disclosed a monoclonal IgM-kappa protein. There was specific IgM binding to galopin, a central nervous system white matter antigen. Periodic treatment with intravenous immunoglobulin has alleviated her neurologic symptoms. She has now been followed for 7 years and maintained significant improvement in neurologic symptoms and signs.
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13/22. Paroxysmal dysarthria and ataxia after midbrain infarction.

    The authors describe a patient who showed paroxysmal dysarthria and right-limb ataxia after midbrain infarction. SPECT imaging showed marked hypoperfusion in the left parietal lobe while the patient was having frequent paroxysmal attacks. After treatment with phenytoin, the symptoms and hypoperfusion in SPECT imaging improved. The authors conclude that dysfunction of the cerebellothalamocortical pathway after midbrain infarction may cause paroxysmal dysarthria and ataxia.
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14/22. Fenestration of vertebral artery at the craniovertebral junction in down syndrome: a case report.

    STUDY DESIGN: Case report of a down syndrome patient with right vertebral artery fenestration and abnormalities of the craniovertebral junction. OBJECTIVES: Describe the utility of 3-dimensional computed tomography angiography for evaluating vertebral artery anomalies before surgery. SUMMARY OF BACKGROUND DATA: Previous reviews evaluating catheter angiograms identified various anomalies of vertebral artery at the craniovertebral junction. The frequency of vertebral artery anomalies is increased in patients having osseous anomalies at the craniovertebral junction. down syndrome is associated with a high incidence of bone abnormalities at the craniovertebral junction, but there have been no published reports of vertebral artery anomalies per se at the craniovertebral junction. methods: A 16-year-old woman with trisomy 21 presented with gait abnormalities and myelopathy in association with bone abnormalities at the craniovertebral junction, including hypoplastic odontoid and ossiculum terminale. Computed tomography angiography showed that right vertebral artery bifurcated after exiting the C2 transverse foramen with one branch passing through the C1 transverse foramen, whereas the other turned posteromedially and entered the spinal canal between C1 and C2. RESULTS: Occipito-C2 posterior fusion was performed with a rod and screw system. Intraoperatively, the course of the anomalous right vertebral artery was identified by Doppler angiography, and the surgical approach was modified to allow safe pedicle screw insertion while avoiding vertebral artery injury. After surgery, myelopathy resolved within 3 months. CONCLUSIONS: Before corrective surgery of craniovertebral junction anomalies in patients with down syndrome, the possibility of vertebral artery anomalies associated with abnormal craniovertebral junction anatomy should be considered. With preoperative 3-dimensional computed tomography angiography, we can precisely identify the anomalous vertebral artery and modify the surgical approach to reduce the possible risk of intraoperative vertebral artery injury in advance.
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15/22. The cerebellum in schizophrenia: a case of intermittent ataxia and psychosis--clinical, cognitive, and neuroanatomical correlates.

    The contribution of cerebellar brain circuits to schizophrenia has been previously alluded to in the literature. This study examines current reappraisals of cerebellar involvement in cognition and behavior. An individual with documented developmental cerebellar anomalies who developed schizophrenic symptoms in late adolescence is described. Psychiatric, medical, and cognitive assessments were conducted to document the multifactorial contributions and manifestations of this dysfunction. Using this case as an example, the putative role of cerebellar dysfunction in the pathogenesis and clinical understanding of schizophrenic and psychotic illnesses is explored.
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16/22. Cervical spine meningioma presenting as otalgia: case report.

    OBJECTIVE AND IMPORTANCE: Cervical spine meningiomas have not been reported to present as otalgia. It is important to include otalgia in the differential diagnosis and workup, especially when more common causes of ear pain have been excluded. CLINICAL PRESENTATION: A 66-year-old woman presented to her primary care physician with severe ear pain. She underwent routine diagnostic testing and eventually was referred to a neurologist. After conservative management failed, the patient underwent cervical spine magnetic resonance imaging, which revealed a large meningioma encompassing C2-C3. INTERVENTION: The patient underwent a cervical laminectomy with complete resection of the tumor. She experienced immediate postoperative resolution of her symptoms. CONCLUSION: This case illustrates the importance of aggressive evaluation of otalgia when routine diagnostic studies are inconclusive. Cervical meningiomas are associated with significant potential morbidity and should be excluded early in the diagnostic process.
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17/22. Interferon alfa treatment for sjogren's syndrome associated neuropathy.

    Treatment response to interferon alfa (IFNalpha) is described in three consecutive cases of two forms of sjogren's syndrome associated neuropathy (SSN)-two with sensory ataxic ganglionopathy and one with sensorimotor neuropathy with demyelinating features. All responded well to IFNalpha in terms of neuropathic symptoms, sicca symptoms, antibody titres, and findings in salivary gland biopsy specimens. IFNalpha thus showed promise in treating both SSN and the underlying sjogren's syndrome.
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18/22. Thalamic infarct presenting with thalamic astasia.

    Astasia, inability to stand unsupported despite good strength, resembles the marked balance impairment of patients with vestibulocerebellar disease. We describe a patient with unilateral thalamic infarct that presented with astasia. A 76-year-old hypertensive woman was admitted to our hospital because of marked unsteadiness. On neurological examination, she could not stand unsupported and the woman's body swayed back and forth markedly. The swaying was not compensated for by her taking a step forward or backward, and she frequently collapsed when support was withdrawn. diffusion-weighted magnetic resonance image revealed a discrete infarct within the right posterolateral thalamus. brain single photon emission computerized tomography revealed markedly decreased regional cerebral blood flow within in the right thalamus with concomitant left superior cerebellar region. We discuss the possible pathomechanisms of thalamic astasia.
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19/22. Methyl bromide intoxication causes reversible symmetric brainstem and cerebellar MRI lesions.

    Methyl bromide is toxic to the central and peripheral nervous systems. A patient with occupational exposure to this agent is described. MRI showed strikingly symmetric brainstem and cerebellar lesions. The patient's clinical course and the topography and resolution of his MRI abnormalities suggest that this condition is an energy deprivation syndrome.
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20/22. Noncommunicating spinal extradural arachnoid cyst causing spinal cord compression in a child.

    Extradural arachnoid cysts in the spine are relatively uncommon causes of spinal cord compression in the pediatric population that are thought to arise from congenital defects in the dura mater. Most reports describe such cysts communicating with the intrathecal subarachnoid space through a small defect in the dura. The authors describe the case of a child who presented with spinal cord compression caused by a large spinal extradural arachnoid cyst that did not communicate with the intradural subarachnoid space. An 11-year-old girl presented with urinary urgency, progressive lower-extremity weakness, myelopathy, and severe gait ataxia. magnetic resonance imaging of the spine demonstrated a large extradural arachnoid cyst extending from T-8 to T-12. The patient underwent a thoracic laminoplasty for en bloc resection of the spinal extradural arachnoid cyst. Intraoperatively, the dura was intact and there was no evidence of communication into the intradural subarachnoid space. Postoperatively, the patient's motor strength and ambulation improved immediately, and no subsequent cerebrospinal fluid leak occurred. Noncommunicating spinal extradural arachnoid cysts are extremely rare lesions that can cause spinal cord compression in children. Because the dura remains intact, they can be removed entirely without subsequent dural repair. The authors review the literature and discuss the proposed underlying mechanisms of formation of these arachnoid cysts.
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