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40 Possible Causes for 'Eye of the Tiger' Sign on MRI, Psychomotor Retardation

  • 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]

  • Aromatic L-Amino Acid Decarboxylase Deficiency

    […] of the tigersign on MRI.[invitae.com] These patients were born to healthy and non-consanguineous parents, and presented oculogyric crises, paroxysmal dystonic attacks, and severe psychomotor retardation since[ncbi.nlm.nih.gov] FTL PANK2 PLA2G6 WDR45 Neurodegeneration with brain iron accumulation ( NBIA ) is a group of disorders characterized by iron accumulation in the basal ganglia causing the “eye[invitae.com]

  • Fucosidosis

    MRI images at 6.5 years of age were reminiscent of pallidal iron deposition ("eye-of-the-tiger" sign) seen in neurodegeneration with brain iron accumulation (NBIA) disorders[ncbi.nlm.nih.gov] In type 2, patients have angiokeratoma, milder psychomotor retardation and neurologic signs, longer survival, and normal salinity in the sweat.[ncbi.nlm.nih.gov] MRI and MR spectroscopy are of substantial diagnostic value. Pallidal lesions resembling the eye-of-the-tiger sign suggest brain iron accumulation.[medlink.com]

  • Infantile Neuroaxonal Dystrophy

    The MRI usually shows an area of hyperintensity in the medial globus pallidus that has been called the ‘eye of the tigersign but this is not pathognomonic.[disorders.eyes.arizona.edu] .: Axonic and synaptic changes in a case of psychomotor retardation: An electron microscopic study. J. Neuropath. exp.[link.springer.com] .: Axonic and synaptic changes in case of psychomotor retardation; an electron microscopic study. J. Neuropath. exp.[springerlink.com]

  • 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] Analysis of the WDR45 gene revealed a novel heterozygous missense mutation in one female NBIA patient showing psychomotor retardation with secondary decline.[ncbi.nlm.nih.gov] 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]

  • Canavan Disease

    A brain MRI at four years of age showed low-signal intensity rings surrounding central hyperintense T2-weighted signal in bilateral globus pallidi, (“eye of the tigersign[ncbi.nlm.nih.gov] Clinically, she presented progressive psychomotor retardation, cerebellar signs, pyramidal signs and relative megalencephaly.[ncbi.nlm.nih.gov] CD results in spongiform degeneration of the brain and severe psychomotor retardation, and the affected children usually die by the age of 10.[ncbi.nlm.nih.gov]

  • Spastic Ataxia with Congenital Miosis

    The appearance was distinct from the 'eye of the tiger' sign seen in NBIA1 and Karak syndrome.[findzebra.com] Retardation Aniridia - Ptosis - Intellectual Disability - Familial Obesity Aniridia - Cerebellar Ataxia - Intellectual Disability Aniridia - Absent Patella Aniridia Peters[explorer.opentrials.net] psychomotor retardation, went to a school for the disabled, and later lived in an assisted facility.[findzebra.com]

  • Infantile Neuroaxonal Dystrophy 1

    The MRI usually shows an area of hyperintensity in the medial globus pallidus that has been called the ‘eye of the tigersign but this is not pathognomonic.[disorders.eyes.arizona.edu] This psychomotor retardation, along with the hypotonia, is typically what leads to the dementia, vision loss and spastic tetraplegia.[breathepsychology.wordpress.com] A particular change called the eye-of-the-tiger sign, which indicates an accumulation of iron, is typically seen on magnetic resonance imaging (MRI) scans of the brain in[76.162.247.120]

  • 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] Orlin and Ruth Gershoni‐Baruch, Rambam–Hasharon syndrome of psychomotor retardation, short stature, defective neutrophil motility, and bombay phenotype, American Journal of[doi.org] 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]

  • Revesz Syndrome

    Thus, the eye-of-the-tiger sign is strongly correlated with PANK2 mutations (P On the basis of this correlation, we assessed the value of the brain MRI alone in predicting[nejm.org] Additional features of RS include sparse hair, psychomotor retardation, and intrauterine growth restriction.[symptoma.com] , ataxia due to cerebellar hypoplasia, cerebral calcifications, extensor hypertonia, progressive psychomotor retardation and apparent chromosome instability.[medical-dictionary.thefreedictionary.com]

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