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1,920 Possible Causes for 'Eye of the Tiger' Sign on MRI, Hyperreflexia, Jerk-Locked Premyoclonus Spikes

  • 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] Neurologically, opisthotonus, rigidity of extremities, dystonia, hyperreflexia, profound emaciation, and bilaterally positive Babinski signs were present.[ncbi.nlm.nih.gov] Each patient had spastic gait, dysarthria, dystonic posturing of both arms and generalized hyperreflexia, but no Kayser-Fleischer rings or retinitis pigmentosa.[ncbi.nlm.nih.gov]

    Missing: Jerk-Locked Premyoclonus Spikes
  • Mitochondrial Membrane Protein-Associated Neurodegeneration

    Most patients have progressive involvement of the corticospinal tract, with spasticity, hyperreflexia, and extensor plantar responses.[genecards.org] None of our pa-tients exhibited the eye of the tiger sign on T2-weighted MRI.[docslide.net] This is followed by the development of spastic quadriparesis and hyperreflexia and ultimately areflexia and a vegetative state by the end of the first decade.[annalsofian.org]

    Missing: Jerk-Locked Premyoclonus Spikes
  • 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] […] cortical spikes detected by the jerk-locked back-averaging) ( Ikeda et al., 1990 ).[academic.oup.com] spikes detected by the jerk-locked back averaging (JLA) method), and a good response to antiepileptic drugs. 2 BAFME was first reported in 1990 in the Japanese population[nature.com]

    Missing: Hyperreflexia
  • Aromatic L-Amino Acid Decarboxylase Deficiency

    […] of the tigersign on MRI.[invitae.com] Of those patients who present at the average age with the typical dystonic gait disorder, 20% also have hyperreflexia and apparent extensor plantar responses, as well as other[jn.nutrition.org] Association of autonomic nervous hyperreflexia and systemic inflammation in patients with Crohn's disease and ulcerative colitis.[emedicine.medscape.com]

    Missing: Jerk-Locked Premyoclonus Spikes
  • Huntington Disease-Like 2

    Neurological abnormalities could include parkinsonism ( rigidity , bradykinesia , tremor ), dysarthria , and hyperreflexia .[web.stanford.edu] 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] […] ideation Dementia Relentless progression of disease with death 15-20 years after onset Successive generations tend to have earlier onset Juvenile onset symptoms Clumsiness Hyperreflexia[arupconsult.com]

    Missing: Jerk-Locked Premyoclonus Spikes
  • Degenerative Disorder

    […] 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] ” but did have hypodensity in the globus pallidus on T2 weighted MRI brain scans, cerebellar atrophy, and often evidence of iron deposition in the red nucleus and dentate[jmg.bmj.com] Hallervorden-Spatz syndrome 13 ) or atypical features with acanthocytosis, hypoprebetalipoproteinaemia and/or orofacial dyskinesia (known as HARP syndrome 14– 16 ) but all with the “eye[jmg.bmj.com]

    Missing: Jerk-Locked Premyoclonus Spikes
  • Adult-Onset Autosomal Recessive Cerebellar Ataxia

    : Clinical features Age of onset - 43-56 years Slow progression Gait and limb ataxia Dysarthria Occular dysmetria Slow saccades Decreased vibratory sense below the knees Hyperreflexia[emedicine.medscape.com] T2 weighted brain MRI shows an ”eye of the tigersign corresponding to pallidal hypointensity with a high signal center.[neuroweb.us] […] and decreased ankle reflexes); movement disorders such as chorea, dystonia, and oculomotor abnormalities; pyramidal tract dysfunction such as extensor plantar responses, hyperreflexia[blogs.nejm.org]

    Missing: Jerk-Locked Premyoclonus Spikes
  • Canavan Disease

    Clinical manifestation is being noted for diagnosing such as presentation of joint stiffness and hyperreflexia including as well the presence of optic atrophy.[healthool.com] 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] After that, hypotonia progresses to spasticity, hyperreflexia, extensor plantar responses, and tonic extensor spasms. The extensor spasms may occur in response to noise.[ncbi.nlm.nih.gov]

    Missing: Jerk-Locked Premyoclonus Spikes
  • Neuroferritinopathy

    Disinhibition 0000734 Dysarthria Difficulty articulating speech 0001260 Dyskinesia Disorder of involuntary muscle movements 0100660 Emotional lability Emotional instability 0000712 Hyperreflexia[rarediseases.info.nih.gov] Individuals with neuroferritinopathy also show the 'eye of the tiger'' sign.[orpha.net] Children with PKAN typically manifest gait problems around age 3 and later develop progressive dystonia, dysarthria, rigidity, spasticity, hyperreflexia and extensor toe signs[nbiaalliance.org]

    Missing: Jerk-Locked Premyoclonus Spikes
  • 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] […] weakness 0003324 Gliosis 0002171 Global developmental delay 0001263 Hearing impairment Deafness Hearing defect [ more ] 0000365 Hyperactivity More active than typical 0000752 Hyperreflexia[rarediseases.info.nih.gov] On physical examination there was optic atrophy, hypertonia and hyperreflexia.[scinapse.io]

    Missing: Jerk-Locked Premyoclonus Spikes

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