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30 Possible Causes for 'Eye of the Tiger' Sign on MRI, Onset in Early Infancy

  • Hallervorden-Spatz Syndrome

    However, the disease has been reported in infancy, and cases with adult onset have also been described.[emedicine.com] 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]

  • Aromatic L-Amino Acid Decarboxylase Deficiency

    The onset is early in infancy and inheritance is autosomal recessive.[uniprot.org] […] of the tigersign on MRI.[invitae.com] The onset is early in infancy and inheritance is autosomal recessive. {ECO:0000269 PubMed:14991824, ECO:0000269 PubMed:15079002, ECO:0000269 Ref.12}.[genecards.org]

  • Neurodegeneration with Brain Iron Accumulation

    Age of onset may vary from early infancy to late adulthood.[invitae.com] 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] Optic atrophy occurs in the majority of affected individuals with onset during infancy to early childhood. 22 23 Nystagmus and strabismus are also common. 23 The predominant[doi.org]

  • X-linked Parkinsonism-Spasticity Syndrome

    The age of onset can be from infancy to the eighth decade. 9 The marked interfamilial variation in age of onset was one of the early pointers to genetic heterogeneity in this[jnnp.bmj.com] 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]

  • Mitochondrial Membrane Protein-Associated Neurodegeneration

    Age of onset may vary from early infancy to late adulthood.[invitae.com] 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]

  • Benign Adult Familial Myoclonic Epilepsy

    PubMed Text format Abstract available MOGAMI Y, Suzuki Y, Murakami Y, Ikeda T, et al Early infancy-onset stimulation-induced myoclonic seizures in three siblings with inherited[amedeo.com] T2 weighted brain MRI shows an ”eye of the tigersign corresponding to pallidal hypointensity with a high signal center.[neuroweb.us] […] disorder characterized by a cluster of signs and symptoms customarily occurring together; these include type of seizure, etiology, anatomy, precipitating factors, age of onset[slideshare.net]

  • Infantile Neuroaxonal Dystrophy 1

    INAD (Infantile Neuroaxonal Dystrophy) is a rare heredo-degenerative disease with onset in early infancy (after a period of latency), clinically characterized by progressive[moh-it.pure.elsevier.com] 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] 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]

  • Degenerative Disorder

    Clinical features : Onset in early infancy, developmental delay, cherry-red spot on macula, hyperacusis, myoclonic seizures Learn more about Tay-Sachs here 3.[pedclerk.bsd.uchicago.edu] […] of the tigersign on MRI brains scan and PANK2 mutations; secondly, a group of clinically atypical cases which did not have PANK2 mutations nor the “eye of the tiger sign[jmg.bmj.com] infancy, and skin features such as skin hyperextensibility, easy bruisability, skin fragility with atrophic scars, increased palmar wrinkling.[web.archive.org]

  • Canavan Disease

    The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly[uniprot.org] 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] Supportive no treatment available treat movement disorders, seizures, and feeding problems DESCRIPTION ANC CLINICAL HISTORY Salient clinical features are onset in early infancy[web.peacelink.it]

  • Huntington Disease-Like 3

    […] and severity of disease is inversely correlated with number of CAG repeats Epidemiology usually begins between ages 30-50, but may begin as early as infancy ( juvenile HD[medbullets.com] MRI scan shows a characteristic hypointense globus pallidus with foci of hyperintensity ("eye of the tiger" sign). CAG repeat testing is normal.[online.epocrates.com] PKAN is associated with the pathognomonic ‘eye-of-the-tigersign, characterised by a central region of hyperintensity surrounded by a rim of hypointensity on coronal or axial[jnnp.bmj.com]

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