Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38-year-old man developing slowly progressive left facial paresis was admitted to our hospital. The clinical diagnosis of "adhesive arachnoiditis was made. The first operation was performed in December 1987, and his symptom disappeared postoperatively. Three years later, left facial paresis recurred together with trunkal ataxia. A computed tomography and magnetic resonance image revealed two tumors located at the left cerebellopontine angle region and in the left middle cerebral fossa. These two tumors were thought to arise in the different cranial nerve. Under the clinical diagnosis of acoustic neurinoma associated with facial nerve neurinoma, the two step-operation was designed for total removal of the tumor in December 1990 and January 1991. Intraoperative finding confirmed that these two tumors had the different origin. Pathologic diagnosis was compatible with neurinoma. This patient had no family history of "Neurofibromatosis".
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PMID:[Acoustic neurinoma associated with facial neurinoma--a case report]. 128 93

This retrospective study aimed to evaluate the safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with refractory or recurrent brain tumors. Nineteen heavily pretreated patients (males, n = 14; females, n = 5; median age at diagnosis 8.5 years; range, 1.4-22 years) were given intrathecal liposomal cytarabine on a compassionate use basis for recurrent refractory medulloblastoma (n = 12), mixed germ cell tumor (n = 2), central nervous system primitive neuroectodermal tumors of the pons (n = 1), anaplastic ependymoma (n = 1), anaplastic oligodendroglioma (n = 1), atypical teratoid rhabdoid tumor (n = 1), or rhabdoid papillary meningioma (n = 1). Eighteen patients received concomitant systemic radiochemotherapy. A total of 88 intrathecal injections of liposomal cytarabine (dose range, 20-50 mg) were administered with concomitant dexamethasone prophylaxis. The median number of doses per patient was four (range, 1-10). Duration of treatment ranged from (1/2) to 10 months. Eleven patients (57.9%) did not show any side effects, whereas eight patients (42.1%) developed side effects related to either chemical arachnoiditis (n = 4) or neurological progression (n = 2). Less typical treatment-related symptoms (e.g. lethargy, ataxia, and slurred speech) were observed in two patients. Treatment with intrathecal liposomal cytarabine was discontinued twice because of side effects. In conclusion, although intrathecal liposomal cytarabine was generally well tolerated, it should be used cautiously and only with dexamethasone prophylaxis in extensively pretreated patients with recurrent brain tumors. Proof of efficacy requires a prospective single-agent phase II study.
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PMID:Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study. 1961 18

Subarachnoid blood has been reported as a cause of chronic spinal arachnoiditis. Although syringomyelia has been thought to be caused by spinal arachnoiditis, reports of syringomyelia following aneurysmal subarachnoid hemorrhage (SAH) are very rare. We describe two patients with syringomyelia associated with chronic spinal arachnoiditis following SAH. From January 2001 to December 2010, 198 patients with aneurysmal SAH were treated at Kinki University School of Medicine. Two of the 198 patients had syringomyelia following aneurysmal SAH; thus the rate of syringomyelia associated with aneurysmal SAH was 1.0%. Patient 1 was a 54-year-old woman who presented with back pain, back numbness and gait disturbance 20 months after SAH. Her MRI revealed syringomyelia of the spinal cord from C2 to T10. She underwent shunting of the syrinx to the subarachnoid space. Patient 2 was a 49-year-old man, who was admitted to the hospital with headache, diplopia, hoarseness, dysphagia and ataxia five months after SAH. MRI revealed syringomyelia from the medulla oblongata to C6, and an enlargement of the lateral and fourth ventricles. After foramen magnum decompression and C1 laminectomy, a fourth ventricle-subarachnoid shunt was placed by insertion of a catheter. Spinal arachnoiditis and spinal syringomyelia are rare but important chronic complications after SAH.
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PMID:Spinal syringomyelia following subarachnoid hemorrhage. 2228 78

We present the case of a 55-year-old woman with diffuse adhesive arachnoiditis in the posterior fossa and cervicothoracic spine following posterior inferior cerebellar artery aneurysmal subarachnoid hemorrhage (SAH). She underwent aneurysm clipping with subsequent gradual neurologic decline associated with sensory disturbances, gait ataxia, and spastic paraparesis. Magnetic resonance imaging revealed diffuse adhesive arachnoiditis in the posterior fossa and cervicothoracic spine, syringobulbia, and multiple arachnoid cysts in the cervicothoracic spine along with syringohydromyelia. Early surgical intervention with microlysis of the adhesions and duraplasty at the clinically relevant levels resulted in clinical improvement. Although adhesive arachnoiditis, secondary arachnoid cysts, and cerebrospinal fluid flow abnormalities resulting in syrinx are rare following aneurysmal SAH, early recognition and appropriate intervention lead to good clinical outcomes.
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PMID:Compressive Cervicothoracic Adhesive Arachnoiditis following Aneurysmal Subarachnoid Hemorrhage: A Case Report and Literature Review. 2508 91

A male patient had been operated in 1990 at a different hospital for a suprasellar mass lesion causing progressive blindness. At exploration, the mass was felt to be inflammatory in nature with arachnoiditis and hydrocephalus. Deep yellow staining of tissues round the mass was recorded at the time of first surgery. The patient was readmitted in 1996 for progressive visual failure, ataxia, mental changes and deafness. MRI confirmed persistence of suprasellar mass and showed the characteristic findings of superficial siderosis of CNS. On reexploration, the mass had multiple vessels over the walls and contained xanthochromic fluid. It was excised and histologically examined to be an epidermoid with a cyst. The patient developed chest infection, became drowsy and died. The MRI picture, the CSF finding and the yellow staining of tissue confirmed the diagnosis of superficial siderosis of the CNS, which was first thought of after seeing the diagnostic MRI. The cause of the superficial siderosis was recurrent bleeding from the tumour.
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PMID:Superficial siderosis of the nervous system. 2950 35