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63 Possible Causes for 'Eye of the Tiger' Sign on MRI, Some Response to L-Dopa Therapy

  • X-linked Parkinsonism-Spasticity Syndrome

    T2-weighted fast spin echo (G) and T1-weighted (H) brain MRI scans from a child with PKAN demonstrating the "eye of the tiger" sign (courtesy of Susan J.[ojrd.biomedcentral.com] T2 weighted brain MRI shows an ”eye of the tigersign corresponding to pallidal hypointensity with a high signal center.[neuroweb.us] Onset can be later, with more rigidity in atypical forms of PKAN [ 12 ], but the typical MRI findings of the "eye of the tiger" sign suggest the diagnosis.[ojrd.biomedcentral.com]

  • Hallervorden-Spatz Syndrome

    Iron deposition in conjunction with destruction of the globus pallidus gives rise to the characteristic eye-of-the-tiger sign in MRI.[ncbi.nlm.nih.gov] […] of the tiger" sign.[ncbi.nlm.nih.gov] It has been postulated that pantothenate kinase 2 mutations underlying all cases of classic Hallervorden-Spatz syndrome are always associated with the eye-of-the-tiger sign[ncbi.nlm.nih.gov]

    Missing: Some Response to L-Dopa Therapy
  • Aromatic L-Amino Acid Decarboxylase Deficiency

    The response to L-dopa therapy varies among affected individuals. Some people respond quickly and completely to L-dopa therapy seeing a full reversal of symptoms.[rarediseases.org] […] of the tigersign on MRI.[invitae.com] In some cases, mostly in individuals with the severe form of tyrosine hydroxylase deficiency, L-dopa therapy may prove ineffective initially.[rarediseases.org]

  • Neurodegeneration with Brain Iron Accumulation

    Individuals with PKAN and HARP syndrome, which is considered part of the PKAN disease spectrum, show a characteristic "eye of the tiger''sign on MRI, a central region of hyperintensity[orpha.net] sign in cranial MRI, this case most likely represented an idiopathic form of NBIA but atypical PKAN may be also considered.[ncbi.nlm.nih.gov] For all of the disorders in this category, T 2 -weighted brain MRI would distinguish PKAN based on the presence of the "eye of the tiger" sign.[ncbi.nlm.nih.gov]

    Missing: Some Response to L-Dopa Therapy
  • Pallidopyramidal Syndrome

    Response to L-dopa therapy is variable but a significant response was reported in some of these diseases (2-5).[docslide.net] The mode of inheritance is variable; some are autosomal domi- nant (4, 5), some are autosomal recessive (1,6,7, 10, 11) or X-linked recessive (8,9).[docslide.net]

    Missing: 'Eye of the Tiger' Sign on MRI
  • McLeod Neuroacanthocytosis Syndrome

    […] of the tiger" sign on MRI characteristic Pigmentary retinopathy PRNP HDL1 (OMIM 603218 ) AD Phenotype may be indistinguishable from HD.[ncbi.nlm.nih.gov] Pantothenate kinase 2 mutation with classic pantothenate-kinase-associated neurodegeneration without ‘eye-of-the-tigersign on MRI in a pair of siblings.[e-jmd.org] […] prominent speech defects, psychiatric disturbance, & more gradual disease progression In 8%: acanthocytosis Usually childhood or adolescent onset Basal ganglia iron deposition "Eye[ncbi.nlm.nih.gov]

    Missing: Some Response to L-Dopa Therapy
  • Benign Adult Familial Myoclonic Epilepsy

    T2 weighted brain MRI shows an ”eye of the tigersign corresponding to pallidal hypointensity with a high signal center.[neuroweb.us]

    Missing: Some Response to L-Dopa Therapy
  • Mitochondrial Membrane Protein-Associated Neurodegeneration

    None of our pa-tients exhibited the eye of the tiger sign on T2-weighted MRI.[docslide.net] MRI showed iron deposition in the globus pallidus and substantia nigra without the eye-of-the-tiger sign, which is typical for PKAN, the most frequent form of NBIA.[push-zb.helmholtz-muenchen.de] MRI showed iron deposition in the globus pallidus and substantia nigra without the eye‐of‐the‐tiger sign, which is typical for PKAN, the most frequent form of NBIA. 2012 Movement[ingentaconnect.com]

    Missing: Some Response to L-Dopa Therapy
  • Juvenile Paralysis Agitans of Hunt

    Parkinsonismo precoce associado a lesões palidais de tipo "eye-of-the-tiger" "Eye-of-the-tiger" sign at MRI in a patient with early-onset parkinsonism Egberto Reis Barbosa[scielo.br]

    Missing: Some Response to L-Dopa Therapy
  • Jankovic Rivera Syndrome

    Pantothenate kinase 2 mutation with classic pantothenate-kinase-associated neurodegeneration without ‘eye-of-the-tigersign on MRI in a pair of siblings.[e-jmd.org] T1WI showed hyperintensity in all cases of HSS in addition to the “eye‐of‐the‐tigersign on T2WI.[doi.org] Conclusion MRI showed prominent signal abnormalities in the GP bilaterally in HSS.[doi.org]

    Missing: Some Response to L-Dopa Therapy

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