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)

Acute cerebellar ataxia is a relatively common neurologic disorder among children. Our aim was to characterize the clinical picture, etiology, and prognosis of acute cerebellar ataxia. The medical records of all children with a diagnosis of acute cerebellar ataxia hospitalized in our center and Hasharon Medical Center from 1990 to 2001 were reviewed. The diagnosis of acute cerebellar ataxia was based on the following criteria: acute onset of ataxia with or without nystagmus; absence of known genetic predisposing factors, such as familial degenerative disorders; and absence of drug intoxication, bacterial meningitis, and metabolic disorders. Thirty-nine children were identified; 54% were male; mean age at presentation was 4.8 +/- 3.8 years. All patients were observed for at least 1 year. A prodromal febrile illness was noted in 74.4%: varicella, 31%; mumps, 20%; nonspecific viral infection, 15.4%; mycoplasma, 5%; Epstein Barr virus, 3%. Latency from the prodromal illness to the onset of ataxia was 8.8 +/- 7.4 days. The most common associated neurologic findings were nystagmus and dysmetria. Full gait recovery took less than 2 weeks on average, and the longest duration of neurologic signs was 24 days (mumps-related). Acute cerebellar ataxia in childhood is a self-limited disease. The recovery was faster than that reported in previous publications and was complete in all children without any neurologic sequelae. Imaging studies are needed only in atypical presentation or if there is no spontaneous improvement after 1 to 2 weeks.
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PMID:Post-infectious acute cerebellar ataxia in children. 1455 15

Two horses had a history of ataxia and weakness or recumbency. One recovered and was diagnosed with West Nile virus (WNV) infection by serologic testing. The other was euthanized; it had meningoencephalomyelitis, WNV was detected by polymerase chain reaction. West Nile virus infection is an emerging disease. Year 2002 is the first year in which cases have been seen in Saskatchewan.
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PMID:Clinical West Nile virus infection in 2 horses in western Canada. 1514 4

An apparently novel neurological disease clinically characterized by shaking, tremors, seizures, staggering gait, and ataxia was first observed in farmed mink kits in Denmark in 2000 and subsequently in Sweden, Denmark, and Finland in 2001, and again in Denmark in 2002. Lymphoplasmacytic encephalomyelitis was found in the affected kits. The lesions were most severe in the brainstem and cerebellum and consisted of neuronal degeneration and necrosis, neuronophagia, focal and diffuse gliosis, perivascular cuffs formed by lymphocytes, plasma cells and macrophages, and segmental loss of Purkinje cells. Testing was conducted to determine the cause of the disease, including general virological investigations (virus culture, negative-staining electron microscopy, immunoelectron microscopy, polymerase chain reaction for herpesviruses, adenoviruses, pestiviruses, and coronaviruses), tests for specific viral diseases (canine distemper, Borna disease, Louping ill, West Nile virus infection, tick-borne encephalitis, Aleutian disease), tests for protozoa (Toxoplasma gondii, Neospora caninum, Encephalitozoon cuniculi), bacteria (general culture, listeria, Clamydophila psittaci), and intracerebral inoculation of neonatal mice. The results of all these investigations were negative. One group of 3 mink kits inoculated intracerebrally with brain homogenate of affected mink developed clinical signs and histological lesions similar to those observed in naturally infected mink. Based on the histopathological features, it is postulated that the disease is caused by a yet unidentified virus.
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PMID:Investigations into shaking mink syndrome: an encephalomyelitis of unknown cause in farmed mink (Mustela vison) kits in Scandinavia. 1530 41

A male alpaca acutely developed signs of anorexia and fever. Within 2 days, neurologic signs (head tremors and asymmetric ataxia) developed. West Nile virus (WNV) infection was considered a primary differential diagnosis on the basis of 6 previous cases on nearby alpaca farms on which animals had similar clinical signs. Four days after the male alpaca became ill, a female alpaca from the same farm developed similar neurologic signs. In addition to anti-inflammatory and supportive treatments, both alpacas received a transfusion of llama plasma with antibodies against WNV Seven days after the onset of clinical signs, the female alpaca had made a full recovery; however, the more severely affected male died. West Nile virus infection was confirmed post mortem by use of reverse transcriptase-polymerase chain reaction assay and immunohistochemical staining.
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PMID:West Nile virus infection in two alpacas. 1548 54

A 3-yr-old male white-tailed deer (Odocoileus virginianus) with a history of ataxia and tremors was submitted to the Tifton Veterinary Diagnostic and Investigational Laboratory (The University of Georgia, Tifton, Georgia, USA) for necropsy. Gross findings were unremarkable. Histologically, the brain had multifocal lymphoplasmacytic perivascular inflammation, scattered gliosis, and rare satellitosis. Mild hemorrhage and congestion in the retropharyngeal lymph nodes and mild lymphoid depletion in the tonsil were present. A reverse transcription-polymerase chain reaction test performed on brain yielded a positive result for West Nile virus. This represents the first report of fatal West Nile virus infection in a white-tailed deer.
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PMID:Fatal West Nile virus infection in a white-tailed deer (Odocoileus virginianus). 1582 31

Twenty-three susceptible newborn kittens were inoculated with feline panleukopenia virus on the day of birth and were sacrificed from 18 hr to 43 days postinoculation (DPI). Macroscopic lesions included thymic atrophy in animals examined at 4 to 14 DPI and cerebellar hypoplasia and degeneration in animals examined at 22 to 43 DPI. Clinical signs of ataxia were not observed in the four kittens with cerebellar lesions sacrificed at 22 to 43 days of age. Intranuclear inclusions were present in a variety of cell types in the organs examined from kittens that died or were sacrificed at 4 to 14 DPI. The most severely infected and depleted tissues were the thymus, spleen, mesenteric lymph nodes, and the cerebellum, whereas the bladder, testes, ovaries, and uterus were the least susceptible to panleukopenia virus infection. Specific fluorescence was demonstrated with panleukopenia antiglobulin conjugate in various cell types in tissues from 2 to 22 DPI and only in cerebellar Purkinje cells of kittens sacrificed at 29, 36, and 43 DPI. The virus replicated in the cells of all layers of blood vessels (endothelial, muscular, and connective tissue cells), suggesting that this is the route of dissemination of the agent throughout the body.
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PMID:Pathogenesis of Feline Panleukopenia Virus in Susceptible Newborn Kittens II. Pathology and Immunofluorescence. 1655 64

During the past decade, numerous molecular mediators of neurodegenerative diseases and neurological disorders have been identified and validated, yet few novel therapies have emerged and the unmet medical needs remain high. These molecular mediators belong to target classes such as ion channels, neurotransmitters and neurotransmitter receptors, cytokines, growth factors, enzymes and other proteins. In some cases, substantial pre-clinical validation exists, but the molecular target has not been readily druggable with small molecules, proteins or antibodies. RNA interference represents a therapeutic approach applicable to such non-druggable targets. Both non-viral and viral delivery strategies are being undertaken for in vivo silencing of molecular targets by RNA interference, which has resulted in robust efficacy in animal models of Alzheimer's disease, ALS, Huntington's disease, spinocerebellar ataxia, anxiety, depression, neuropathic pain, encephalitis and glioblastoma. These proof-of-concept data in animal models, together with the commencement of clinical trials using RNA interference for macular degeneration and respiratory syncytial virus infection, point to the potential of direct RNA interference for neurological disorders and neurodegenerative diseases.
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PMID:Therapeutic potential of RNA interference for neurological disorders. 1681 77

Acute disseminated encephalomyelitis (ADEM) is a rare acute inflammatory demyelinating disorder of central nervous system characterized by multifocal white matter involvement. Children and young adults are more commonly affected. The onset of ADEM usually follows a viral infection or immunization after a mean period of 7-14 days. The pathogenesis is not clear but several evidences support the autommune aetiology. ADEM is characterized by multifocal neurological signs and occasionally it rapidly progresses to coma. Magnetic resonance imaging (MRI) is useful to confirm the diagnosis. Treatment is based on intravenous high dose methylprednisolone, which usually leads a rapid improvement. Recently the use of i.v. immunoglobulins has also been suggested. We report a case of a 2-year-old girl with sudden onset of neurological symptoms (irritability, drowsiness, hemiparesis, ataxia, strabismus) after an upper respiratory tract infection. MRI showed the presence of multiple high signal areas in the brain and in the spinal cord. High doses of methylprednisolone (10 mg/Kg) i.v. determined a rapid and persistent improvement of neurological signs and symptoms.
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PMID:[Acute disseminated encephalomyelitis (ADEM): report of a clinical case]. 1692 56

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system. In majority of cases onset occurs from 5-15 years of age. In a nonimmunized population the average onset is 8 years. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half before age 2 years. SSPE generally occurs 5-10 years after measles infection. In the early stages of the disease behavioral and personality changes is followed by myoclonic jerks and convulsions. In late stages dementia, stupor and coma develops. Diagnosis is achieved by typical clinical findings, measles antibody titer increase in cerebrospinal fluid (CSF) and serum, high amplitude, slow, sharp waves in EEG. Prognosis is poor and death ensues in about 3 yr after the diagnosis. Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor. Due to suggestive radiological and clinical findings and a history of recent mumps infection he was thought to have acute disseminated encephalomyelitis initially and given treatment. But due to clinical deterioration and detection of anti measles IgG in serum and CSF, SSPE diagnosis was confirmed. With this SSPE case presenting initially as ADEM, the authors tried to emphasize that presentation of SSPE may clinically and radiologically be diverse and a thorough differential diagnosis is mandatory for a definite diagnosis.
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PMID:Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis. 1720 44

A 72-year-old male with liver cirrhosis and hepatocellular carcinoma experienced general fatigue. Four days later he was admitted to our hospital because of dizziness, dysbasia and left facial palsy (day 1). On day 6, a neurological examination revealed left trigeminal neuralgia, left medial longitudinal fasciculus (MLF) syndrome, skew deviation, hypacusia, tongue deviation and left limb ataxia. Magnetic resonance imaging of the brain including diffusion-weighted imaging showed previous lacunar infarctions at the left thalamus and pons. The immunological investigation for viral infection in his serum samples showed high titers of IgM antibody against cytomegalovirus (CMV). Cerebrospinal fluid (CSF) investigation revealed mononuclear pleocytosis, elevated protein levels and high titers of IgG antibody against the varicella-zoster virus (VZV). Anti-CMV antibody measurement and CMV-DNA detection by the polymerase chain reaction in CSF revealed that the central nervous system (CNS) was not infected by CMV. We diagnosed this case as brainstem encephalitis following multiple cranial neuropathy associated with CMV and VZV infections. The neurological symptoms gradually improved with aciclovir and prednisolone therapy. The titers of antibody for CMV in his serum samples normalized 4 months later after onset. Although there was no evidence of CMV infection in the CNS was obtained, parainfection or autoimmune mediated responses followed by viral infections might have led to brainstem encephalitis with multiple cranial nerve involvements in our patient.
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PMID:[A case of brainstem encephalitis following multiple cranial neuropathy in a hepatocellular carcinoma patient--association with cytomegalovirus and varicella-zoster virus infection]. 1804 5


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