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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

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