
BASAL GANGLIA HYPERINTENSITY
​
AUTOIMMUNE
CRMP5 AB
HYPERINTENSITY (T2/ FLAIR)
BASAL GANGLIA
​
CASPR2 AB
HYPERINTENSITY (T2/ FLAIR)
UNILATERAL BASAL GANGLIA
​
NMDA AB
HYPERINTENSITY (T2/ FLAIR)
THALAMUS/ PULVINAR
BASAL GANGLIA
​
LGI1 AB
HYPERINTENSITY (T2/ FLAIR)
CJD LIKE IMAGING
​
AMPA AB
HYPERINTENSITY (T2/ FLAIR)
CJD LIKE CORTICAL RIBBON
​
MITOCHONDRIAL
LEIGH (SURF1)
PDHD DEFICIENCY
​
KEARN SAYRE' SYNDROME
​
CoQ10/COQ2/COQ6/COQ9/PDSS2 GENE MUTATIONS
​
AMINOACIDURIA
GLUTARIC ACIDURIA TYPE1
HYPERINTENSITY (T2/ FLAIR)
PUTAMEN/ POSTERIOR PUTAMEN
WHITE MATTER HYPERINTENSITY
SPARING U-FIBRES
MACROCEPHALY
BATWING FRONTO-TEMPORAL ATROPHY
SUBDURAL HYGROMAS
CERVICAL MYELOPATHY
​
L2 H-GLUTARIC ACIDURIA
HYPERINTENSITY (T2/ FLAIR)
CAUDATE/ PUTAMEN
GLOBUS PALLIDUS/ DENTATE
CENTRIPETAL WHITE MATTER
U-FIBRE INVOLVED FIRST
CEREBELLAR WHITE MATTER
MACROCEPHALY
​
METHYLMANOLIC ACIDAEMIA
HYPERINTENSITY (T2)
GLOBUS PALLIDUS CYSTIC CHANGES
​
PAROXYSMAL DISORDER
PDE10A
PAROXYSMAL KINESOGENIC DYSTONIA
HYPERINTENSITY (T2)
BASAL GANGLIA
​
ECHS1 MUTATIONS
PAROXYSMAL EXERCISE INDUCED DYSTONIA
LEIGH LIKE SYNDROME
HYPERINTENSITY (T2)
GLOBUS PALLIDUS HYPERINTENSITY/ CYSTIC CHANGES
​
MINERAL DEPOSITION DISORDERS
WILSON
HYPERINTENSITY (T2)
BASAL GANGLIA/ THALAMUS/ PONS/ CORTEX
WHITE MATTER HYPERINTENSITY
SUBCORTICAL/ CEREBELLAR
MCP (RARE)
HYPERINTENSITY (T1)
IN HEPATIC FAILURE
BASAL GANGLIA​
​
SLC30A2/ SLC39A19
HYPERINTENSITY (T1)
BASAL GANGLIA
HYPERINTENSITY (T2)
GENERALIZED WHM/ CEREBELLAR
SPINAL CORD/ DORSAL PONS
​
VITAMIN/ COFACTOR DISORDERS
COBALAMIN C DEFICIENCY
HYPERINTENSITY (T1)
GLOBUS PALLIDUS
​
BT-BGD (SLC19A3)
HYPERINTENSITY (T2)
BILATERAL BASAL GANGLIA
​
LYSOSOMAL
GM1 GANGLIOSIDOSIS
HYPERINTENSITY (T1)
GLOBUS PALLIDUS
HYPOINTENSITY (SWI)
GLOBUS PALLIDUS
WISH BONE SIGN
POSTERIOR PUTAMEN T2 HPERINTENSITY
​
​
BASAL GANGLIA HYPOINTENSITY
​
BRAIN MINERALIZATION DISORDERS
PKAN
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
SUBSTANTIA NIGRA
EYE OF TIGER
​
SCA28(AFG3L2)
HYPOINTENSITY (SWI/ GRE)
EYE OF TIGER
​
PLAN (PLA2G6)
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
PUTAMEN/ CAUDATE
SUBSTANTIA NIGRA
ATROPHY
CEREBELLAR ATROPHY
OPTIC CHIASMA
HYPERTROPHY
CLAVA (ENLARGEMENT OF GRACILE AND CUNEATE NUCLEUS BY AXONAL SWELLING ANS SPHEROIDS)
HYPERINTENSITY (T2/ FLAIR)
CEREBELLAR CORTEX
MRI MAY BE NORMAL
​
MPAN
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
SUBSTANTIA NIGRA
HYPERINTENSITY (T2/ FLAIR)
MEDIAL MEDULLARY LAMINA
​
BPAN (SENDA)
HYPOINTENSITY (SWI/ GRE)
SUBSTANTIA NIGRA >
GLOBUS PALLIDUS
HYPOINTENSITY (T1)
SUBSTANTIA NIGRA PERIPHERAL HYPERINTENSITY
FAHN
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
SUBSTANTIA NIGRA
HYPERINTENSITY (T2/ FLAIR)
WHITE MATTER HYPERINTENSITY
PONS-CEREBELLAR ATROPHY
CORPUS CALLOSAL THINNING
COPAN
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
SUBSTANTIA NIGRA
GLOBUS PALLIDUS CALCIFICATION
​
ACERULOPLASMINAEMIA
HYPOINTENSITY (SWI/ GRE)
BASAL GANGLIA
RED NUCLEUS
THALAMUS
CAUDATE
CORTICAL PENCIL SIGN
HYPERINTENSITY (T2/ FLAIR)
WHITE MATTER HYPERINTENSITY
CEREBELLAR ATROPHY
​
NEUROFERRITINOPATHY
HYPOINTENSITY (SWI/ GRE)
BASAL GANGLIA
RED NUCLEUS
THALAMUS
CEREBRAL/CEREBELLAR ATROPHY
CYSTIC DEGENERATION OF BASAL GANGLIA
EYE OF TIGER
CORTICAL PENCIL SIGN
​
KUFOR RAKEB
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
PUTAMEN/CAUDATE
MRI CAN BE NORMAL
CEREBRAL/ CEREBELLAR ATROPHY
BRAIN STEM ATROPHY
​
WOODHOUSE SAKATI SYNDROME
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
PITUITARY/ CEREBELLAR ATROPHY
HYPERINTENSITY (T2/ FLAIR)
PERIVENTRICULAR WMH
​
VAC14
HYPOINTENSITY (SWI/ GRE)
GLOBUS PALLIDUS
​
BASAL GANGLIA CALCIFICATION
​
FAHR SYNDROME
SLC20A2/ PDGFB/ PDGFRB/ XPR1/ JAM2
CALCIFICATION
BG/ THALAMUS/ CEREBELLUM/ WM
MYORG
CALCIFICATION
CENTRAL PONTINE
BG/ THALAMUS/ CEREBELLUM/ WM
​
COCKAYNE SYNDROME
ATROPHY
CEREBELLAR HEMISPHERE
CEREBELLUM
HYPERINTENSITY (T2/ FLAIR)
HYPOMYELINATION
CALCIFICATION
BASAL GANGLIA CALICIFICATION
​
SYSTEMIC/ METABOLIC
AICARDI GOUTIERES DISEASE
(AGS 17 / TREX1/IFIH1)
HYPOINTENSITY (SWI/ GRE)
SYMMETRICAL BASAL GANGLIA WHITE MATTER CALCIFICATIONS
HYPERINTENSITY (T2/ FLAIR)
WHITE MATTER HYPERINTENSITY
​
HYPOPARATHYROIDISM
​
PSEUDOHYPOPARATHYROIDISM
RED NUCLEUS CALCIFICATION
​
LEAD TOXICITY
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SYSTEMIC/ METABOLIC
HEPATOCEREBRAL SYNDROME
HYPERINTENSITY (T2/ FLAIR)
MCP HYPERINTENSITY
HYPERINTENSITY (T1)
GLOBUS PALLIDUS
​
LANGERHAN HISTIOCYTOSIS
HYPERINTENSITY (T1)
BASAL GANGLIA/ DENTATE
EXTRA AXIAL BRAIN MASS
​
METABOLIC ACIDOSIS
URAEMIC ENCEPHALOPATHY
DIABETES KETOACIDOSIS
METHANOL POISONING
PROPIONIC ACIDAEMIA
HYPERINTENSITY (T2)
PUTAMEN
EXTERNAL CAPSULE
INTERNAL CAPSULE
EXTERNAL MEDULLARY LAMINA
INTERNAL MEDULLARY LAMINA
​
SYSTEMIC LUPUS ERYTHEMATOSUS
HYPERINTENSITY (T2/ FLAIR)
BILATERAL BASAL GANGLIA
​
KERNICTERUS
HYPERINTENSITY (T1)
GLOBUS PALLIDUS (EARLY STAGE)
HYPERINTENSITY (T2)
GLOBUS PALLIDUS (LATE STAGE)
​
JAPANESE ENCEPHALITIS
HYPERINTENSITY (T2/ FLAIR)
BILATERAL THALAMUS
BASAL GANGLIA
​
CAUDATE ATROPHY
​
HUNTINGTON'S DISEASE
​
NEUROACANTHOCYTOSIS
​
FTD-FUS
​
GNAO1
DILATED FRONTAL HORN
CORPUS CALLOSUM ATROPHY
​