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39 Possible Causes for Ataxia, Hyperintensities in the Globus Pallidus (T2-Weighted)

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  • Hallervorden-Spatz Syndrome

    The patient presented with a slowly progressive illness with seizures, extrapyramidal symptoms, cerebellar ataxia, dementia, spasticity, myoclonic movements and a severe demyelinating[ncbi.nlm.nih.gov] This virtually pathognomonic radiographic abnormality, called the eye-of-the-tiger sign, comprises hyperintensities within a hypointense medial globus pallidus on T2-weighted[ncbi.nlm.nih.gov] Although rare, cerebellar ataxia, behavioural abnormalities, parkinsonism and apraxia of eyelid opening were exclusively seen in late onset patients.[ncbi.nlm.nih.gov]

  • Wernicke Encephalopathy

    Wernicke encephalopathy (WE) is a medical emergency caused by thiamine deficiency, characterized by cerebellar ataxia, ophthalmoplegia, and cognitive disturbances that may[ncbi.nlm.nih.gov] […] in the globus pallidus.[ncbi.nlm.nih.gov] T1-weighted MR image depicts symmetric hyperintense foci in the globus pallidus (arrows).[doi.org]

  • Neurodegeneration with Brain Iron Accumulation

    From our series of patients one patient with PKAN and a progressive severe dystonic syndrome, cerebellar ataxia, retinitis pigmentosa and eventual anarthria had a novel combination[ncbi.nlm.nih.gov] Even before the globus pallidus hypointensity developed, patients with mutations could be distinguished by the presence of isolated globus pallidus hyperintensity on T2-weighted[ncbi.nlm.nih.gov] ataxia, and neuropathologically by neuronal loss, gliosis, and iron deposition in the globus pallidus, red nucleus, and substantia nigra.[ncbi.nlm.nih.gov]

  • Alexander Disease

    LETTERS Cerebellar ataxia as the first manifestation of Alexander's disease Ataxia cerebelar como manifestação inicial da doença de Alexander Sheyla Ariêh de Souza Rezende[scielo.br] In addition, there are symmetrical hyperintensity signals in the posterior part of the globus pallidus on T2-weighted images in the two symptomatic patients, which are rarely[ncbi.nlm.nih.gov] This disorder can develop as an infantile, juvenile or adult-onset form and is characterized by several clinical features, including macrocephaly, seizures, ataxia, and bulbar[ncbi.nlm.nih.gov]

  • Creutzfeldt Jakob Disease

    Common first symptoms are dementia, cerebellar ataxia, visual disturbance, and psychiatric symptoms. Seizure as the first symptom of CJD is a very rare finding.[ncbi.nlm.nih.gov] Extrapontine myelinolysis manifests predominantly as areas of bilateral FLAIR /T2-weighted imaging hyperintensity in the globus pallidus, putamen, and thalamus, which are[doi.org] We report a Japanese patient with spinocerebellar ataxia type 31 (SCA31) and sporadic Creutzfeldt-Jakob disease (sCJD).[ncbi.nlm.nih.gov]

  • Wilson Disease

    RESULTS: Most patients (62.3%) exhibited tremor and ataxia, whereas 15.1% were dystonic, and 11.3% had parkinsonism.[ncbi.nlm.nih.gov] On T1W images, these lesions are normal to hypointense but hyperintense lesions especially in globus pallidus may also be seen [ 2 ].[omcr.oxfordjournals.org] The NCS in a patient with WD and PN, cerebellar ataxia, and hepatocellular carcinoma showed axonal degeneration, though autopsy showed only small areas of demyelination in[jamanetwork.com]

  • Alpers Syndrome

    Cerebellar ataxia develops in most patients. Peripheral neuropathy develops in many and becomes increasingly common in older children and young adults.[orpha.net] The right parietal cortex appears normal in the T2-weighted images.[ajnr.org] ataxia in diseases classified elsewhere Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.[icd10data.com]

  • Kernicterus

    Wernicke's encephalopathy a neurological disorder characterized by confusion, apathy, drowsiness, ataxia of gait, nystagmus, and ophthalmoplegia; it is due to thiamine deficiency[medical-dictionary.thefreedictionary.com] In the appropriate clinical context, bilateral globus pallidus T2-weighted and FLAIR signal hyperintensity is supportive of a diagnosis of kernicterus in an infant.[imj.ie] Patients surviving kernicterus have severe permanent neurologic symptoms ( choreoathetosis, spasticity, hearing loss, ataxia, mental retardation ).[neuropathology-web.org]

  • Mercury Poisoning

    She was discharged two days later with a diagnosis of acute cerebellar ataxia. During the following 18 days, the child's condition worsened, and she was rehospitalized.[ncbi.nlm.nih.gov] The T2-weighted MRI images of the patient's brain initially showed multiple hyperintense lesions in cerebral white matter, left globus pallidus, and putamen, which also improved[ncbi.nlm.nih.gov] A 23-year woman, 2 years after injecting herself with elemental mercury developed disabling generalized myoclonus and ataxia.[ncbi.nlm.nih.gov]

  • Autosomal Dominant Progressive External Ophthalmoplegia Type 6

    Low match SPINOCEREBELLAR ATAXIA TYPE 43 Spinocerebellar ataxia type 43 is a rare autosomal dominant cerebellar ataxia type I disorder characterized by late adult-onset of[mendelian.co] T2-weighted axial MR image demonstrates bilateral hyperintense signal abnormalities in the globus pallidus (arrows).[collections.lib.utah.edu] Kearns-Sayre syndrome PEO onset Pigmentary retinopathy CSF protein 1g/L Cerebellar ataxia Heart block Sporadic (maternal transmission infrequent) Large mtDNA deletions detected[arupconsult.com]

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