'Eye of the Tiger' Sign on MRI & Jerk-Locked Premyoclonus Spikes & Multifocal Epileptiform Spikes: Causes & Reasons

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Patient File

Sex unknown, Age unknown

Reported Symptoms

'Eye of the Tiger' Sign on MRI, Jerk-Locked Premyoclonus Spikes, Multifocal Epileptiform Spikes,

Negated

None

Unsure

None

Possible Causes

Benign Adult Familial Myoclonic Epilepsy

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

[…] and wave complexes Jerk-locked premyoclonus spikes Enhancement of the C-reflex Hypoplasia of the corpus callosum EEG abnormality Eyelid myoclonus Cortical myoclonus Status [mendelian.co]

[…] cortical spikes detected by the jerk-locked back-averaging) ( Ikeda et al., 1990 ). [academic.oup.com]

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Generalized Clonic or Tonic-Clonic Seizures

premyoclonus spikes Hand tremor Neuronal loss in central nervous system Cutaneous photosensitivity Hyperreflexia Heterogeneous Weight loss Dystonia Splenomegaly Thrombocytopenia [mendelian.co]

[…] callosum Muscular hypotonia Infantile onset Intellectual disability, moderate Intention tremor Progressive neurologic deterioration Absence seizures Nevus EEG abnormality Jerk-locked [mendelian.co]

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Epilepsy

Inter-ictal multifocal spikes or sharp and slow waves in nearly all EEGs. [epilepsy.com]

Ictal EEG Epilepsia partialis continua is notorious with regard to lack of clinico-EEG correlations; epileptiform abnormalities may or may not be concomitant with the jerks [epilepsy.com]

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Juvenile Myoclonic Epilepsy

We present a case of a boy with juvenile myoclonic epilepsy (JME) who presented with features of non-convulsive status epilepticus (NCSE). This case highlights the fact that NCSE, even though not a common occurrence in JME, should be kept in mind when a patient with previous history of seizures presents with[…] [ncbi.nlm.nih.gov]

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West Syndrome

We report a 4-month-old female infant who developed West syndrome eleven days after administration of a histamine H1 antagonist, oxatomide, for atopic dermatitis. It has been reported that some histamine H1 antagonists induce seizures in epileptic patients. The age, the interval between oxatomide administration, and[…] [ncbi.nlm.nih.gov]

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Idiopathic Generalized Epilepsy

OBJECTIVES: Idiopathic generalized epilepsy (IGE) arises from paroxysmal dysfunctions of the thalamo-cortical network. One of the hallmarks of IGE is the absence of visible abnormalities on routine magnetic resonance imaging (MRI). However, recent quantitative MRI studies showed cortical-subcortical structural[…] [ncbi.nlm.nih.gov]

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Myoclonic-Astatic Epilepsy

Leitthema First Online: 20 March 2014 Zusammenfassung Die myoklonisch-astatische Epilepsie (MAE, Doose-Syndrom, Epilepsie mit myoklonisch-atonischen Anfällen) ist ein seltenes, elektroklinisches Syndrom des frühen Kindesalters, das bei zuvor gesunden, normal entwickelten Kindern auftritt. Jungen sind häufiger betroffen[…] [doi.org]

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Organic Brain Syndrome

Rogers MP, et al. Psychosom Med. 1986 Jul-Aug. Abstract Systemic mastocytosis is a disease characterized by an excessive accumulation of mast cells, and associated with skin lesions, flushing, diarrhea, tachycardia, and psychiatric manifestations. In order to define more clearly the psychiatric manifestations, ten[…] [ncbi.nlm.nih.gov]

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Encephalopathy

Posterior reversible encephalopathy syndrome is an encephalopathy that can be clinically characterized by headache, altered mental status and/or seizures. Neuroimaging demonstrates usually reversible bilateral subcortical vasogenic occipital-parietal edema. Exact pathophysiology remains unclear but is commonly[…] [ncbi.nlm.nih.gov]

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Juvenile Absence Epilepsy

We describe a 68-year-old woman who had typical absence seizures since 14 years of age. The absences were refractory to treatment and persisted into adulthood, with no seizure-free periods until seizure control at 59 years of age. After six years of being seizure-free, she presented with an episode[…] [ncbi.nlm.nih.gov]

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Uremic Encephalopathy

BACKGROUND AND PURPOSE: Uremic encephalopathy is a metabolic disorder in patients with renal failure. The purpose of this study was to describe the MR imaging findings of uremic encephalopathy. MATERIALS AND METHODS: This study retrospectively reviewed MR imaging findings in 10 patients with clinically proved uremic[…] [ncbi.nlm.nih.gov]

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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]

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Insulin Overdose

We report a very rare case of acute pulmonary edema caused by hypoglycemia from insulin overdose during an attempted suicide. A 16-year-old woman with type 1 diabetes was brought to our hospital because of hypoglycemic coma. She exhibited severe hypoxia; upon intubation, bloody froth poured out of the tube.[…] [ncbi.nlm.nih.gov]

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Toxic Encephalopathy

Neurotoxicity following paradichlorobenzene (PDCB) exposure is rare and can occur in patients with pica and mothball or toilet cake ingestion. We present a rare case of toxic encephalopathy due to PDCB mothball inhalation and ingestion and describe the rapidly progressive leukoencephalopathy seen on computed[…] [ncbi.nlm.nih.gov]

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Subcortical Band Heterotopia

Subcortical band heterotopia (SBH) is a neuronal migration disorder. DCX mutations are responsible for almost all familial cases, 80% of sporadic female cases, and 25% of sporadic male cases of SBH, and are associated with more severe gyral and migration abnormality over the anterior brain regions. Somatic mosaicism[…] [ncbi.nlm.nih.gov]

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Early Infantile Epileptic Encephalopathy Type 3

Epileptic encephalopathy with suppression-burst in electroencephalography (EEG) can evolve into a few types of epileptic syndromes. We present here an unusual case of early myoclonic encephalopathy that evolved into migrating partial seizures in infancy. A female neonate initially had erratic myoclonus movements,[…] [ncbi.nlm.nih.gov]

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Lennox-Gastaut Syndrome

The characteristic electroclinical features of Lennox-Gastaut syndrome (LGS) have been extensively reported, but the influence of the constellation of slow spike-and-wave (SSW) complexes with multiple seizure types (including atypical absence seizures [AAS] and tonic seizures [TS]) on patients with this syndrome[…] [ncbi.nlm.nih.gov]

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Hypoxic-Ischemic Brain Injury

Brain Dev. 2015 Feb;37(2):265-9. doi: 10.1016/j.braindev.2014.04.002. Epub 2014 Apr 29. Author information 1 Department of Pediatrics, Toyohashi Municipal Hospital, Toyohashi, Aichi, Japan. Electronic address: pisces-koya@yacht.ocn.ne.jp. 2 Department of Neuromuscular Research, National Institute of Neuroscience, National[…] [ncbi.nlm.nih.gov]

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Progressive Myoclonic Epilepsy Type 7

[…] and wave complexes Loss of consciousness Jerk-locked premyoclonus spikes Enhancement of the C-reflex Giant somatosensory evoked potentials Behavioral abnormality Dysarthria [mendelian.co]

[…] and wave complexes Jerk-locked premyoclonus spikes Enhancement of the C-reflex Hyperreflexia Cerebral atrophy Abnormal autonomic nervous system physiology Rigidity Abnormal [mendelian.co]

[…] disability, moderate Cognitive impairment Dementia Mental deterioration Rare Symptoms - Less than 30% cases EEG with photoparoxysmal response EEG with irregular generalized spike [mendelian.co]

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Epilepsia Partialis Continua

Jerk-locked back averaging (JLA) of magnetoencephalogram disclosed a spike preceding the jerk localized at the hand motor area, whereas JLA of electroencephalogram revealed [ncbi.nlm.nih.gov]

Inter-ictal multifocal spikes or sharp and slow waves in nearly all EEGs. [epilepsy.com]

[…] no premyoclonus spikes. [ncbi.nlm.nih.gov]

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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]

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Familial Infantile Myoclonic Epilepsy

premyoclonus spikes Irritability Vegetative state Intellectual disability, mild Myoclonic atonic seizures EEG with abnormally slow frequencies EEG with spike-wave complexes [mendelian.co]

[…] with photoparoxysmal response Headache Autism Progressive visual loss Retinal dystrophy Visual loss Optic atrophy Visual impairment Giant somatosensory evoked potentials Jerk-locked [mendelian.co]

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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]

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Progressive Myoclonic Epilepsy Type 8

[…] fast spikes, polyspikes and polyspike‐and‐wave discharges Slow background activity, spike‐ and polyspike‐and‐wave discharges and multifocal spikes activated by non‐REM sleep [onlinelibrary.wiley.com]

Generalized epileptiform activity Intermittent photic stimulation Negative 10 to 20 Hz provoked a photoparoxysmal response Negative 6 and 20 Hz provoked a photoparoxysmal [onlinelibrary.wiley.com]

Slow background activity mixed with low‐voltage fast activity, along with diffuse, irregular, spike‐ and polyspike‐and‐wave discharges Slow background activity and multifocal [onlinelibrary.wiley.com]

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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-tiger’ sign 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]

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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 tiger” sign corresponding to pallidal hypointensity with a high signal center. [neuroweb.us]

There are typical MRI findings, with a central hyperintensity with surrounding low signal on T2 images in the globus pallidus, giving the so-called eye-of-the-tiger sign [ncbi.nlm.nih.gov]

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Pallidopyramidal Syndrome

Sometimes the report from a radiologist can state NBIA with classic features like “eye of a tiger sign” that can ease the diagnostic burden. [annalsofian.org]

Eye of the tiger sign is not pathognomonic of pantothenate kinase associated neurodegeneration in adult cases. Brain Behav 2011;1:55-66. 58. [annalsofian.org]

[…] and an abnormal MRI brain showing iron deposition [Flowchart 1] and [Flowchart 2]. [annalsofian.org]

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Epigastric Hernia

Inter-ictal multifocal spikes or sharp and slow waves in nearly all EEGs. [epilepsy.com]

Ictal EEG Epilepsia partialis continua is notorious with regard to lack of clinico-EEG correlations; epileptiform abnormalities may or may not be concomitant with the jerks [epilepsy.com]

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Lafora Disease

Electroencephalogram (EEG) findings in LBD may include multiple spikes and wave discharges, photosensitivity, multifocal epileptiform discharges, and progressive slowing in [annalsofian.org]

Multifocal epileptiform discharges, mainly occipital in location, appear in addition to generalized bursts. [annalsofian.org]

In longitudinal EEG studies, the spike and wave pattern changes from a slow frequency of 3 Hz in the early stages to faster frequencies of 6-12 Hz as the disease progresses [annalsofian.org]

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Progressive Myoclonic Epilepsy Type 3

[…] complexes.[16] Multifocal, predominantly posterior, epileptiform discharges appear in addition to the generalized bursts. [thieme-connect.com]

The EEG becomes disorganized early in the disease course and is characterized by a slow background with superimposed generalized high-voltage spike-wave and polyspike-wave [thieme-connect.com]

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