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MCP HYPERINTENSITY

 

CORTICAL/ SUBCORTICAL DISORDERS

KRABBE DISEASE

HYPERINTENSITY (T2/ FLAIR)

PERIVENTRICULAR HYINTENSITY

DEEP GREY MATTER HYPERINTENSITY

MCP/ DENTATE

OPTIC CHIASMA ENLARGEMENT

LUMBOSACRAL PLEXUS ENLARGEMENT

HYPERINTENSITY (T2)

TIGROID PATTERN

​

NIID (NOTCH2NLC GGC REPEAT)

HYPERINTENSITY (DWI)

CORTICOMEDULLARY JUNCTION

RIBBON LIKE HYPER-INTENSITIES 

HYPERINTENSITY (T2/ FLAIR)

LEUKODYSTROPHY

MCP HYPERINTENSITY

​

POLR3B 

ATROPHY 

CEREBELLUM/ VERMIS

HYPERINTENSITY (T2/ FLAIR)

MIDDLE CEREBELLAR PEDUNCLE

HYPOMYELINATION OF CEREBRAL

AND CEREBELLUM

​

CEREBELLUM DISORDERS

MSA-C

HYPERINTENSITY (T2/ FLAIR)

MCP

HOT CROSS BUN

ATROPHY

PONS 

​

FXTAS

HYPERINTENSITY (T2/ FLAIR)

MCP/DENTATE

CORPUS CALLOSUM (SPLENIUM)

PONS HYPERINTENSITY (FEMALES)

HYPERINTENSITY (DWI)

CORTICOMEDULLARY JUNCTION

RIBBON LIKE HYPER-INTENSITIES 

ATROPHY

CEREBELLAR ATROPHY

​

POLG1

HYPERINTENSITY (T2/ FLAIR)

INFERIOR OLIVARY NUCLEI

CEREBELLAR WHITE MATTER

MCP/ DORSAL THALAMUS

​

SCA48 (STUB1)

HYPERINTENSITY (T2/ FLAIR)

DENTATE + MCP HYPERINTENSITY: CRAB SIGN

ATROPHY

POSTERIOR VERMIS

​

METABOLIC

HEPATOCEREBRAL SYNDROME

HYPERINTENSITY (T2/ FLAIR)

MCP HYPERINTENSITY 

HYPERINTENSITY (T1)

GLOBUS PALLIDUS

​

INFECTION

PML

HYPERINTENSITY (T2/ FLAIR)

SHRIMP SIGN (UNILATERAL)

MCP

SPARING OF DENTATE NUCLEUS

SENSITIVY 83%, SPECIFICITY 100%

​

AUTOIMMUNE

SSPE

HYPERINTENSITY (FLAIR)

CORTICAL/ WHITE MATTER

PONS (RARE)/ MCP

HOT CROSS BUN

​

​

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SPASTICITY

DENTATE

 

T1 HYPERINTENSITY

FAHR

HYPERINTENSITY (T1) 

HYPOINTENSITY (SWI)

DENTATE/ BASAL GANGLIA

​

SCA20

HYPERINTENSITY (T1) 

HYPOINTENSITY (SWI)

DENTATE

​

GADOLINIUM

HYPERINTENSITY (T1) 

DENTATE

​

T2 HYPERINTENSITY

ALEXANDER'S DISEASE

JUVENILE ONSET

HYPERINTENSITY (T2/ FLAIR)

FRONTAL WMH

DENTATE/ CEREBELLUM/ MCP

ADULT ONSET

ATROPHY

MEDULLA AND SPINAL CORD

​

KRABBE DISEASE

HYPERINTENSITY (T2/ FLAIR)

PERIVENTRICULAR HYINTENSITY

DEEP GREY MATTER HYPERINTENSITY

MCP/ DENTATE

OPTIC CHIASMA ENLARGEMENT

LUMBOSACRAL PLEXUS ENLARGEMENT

HYPERINTENSITY (T2/ FLAIR)

TIGROID PATTERN 

​

WILSON'S DISEASE (ATP7B)

HYPERINTENSITY (T2)

BASAL GANGLIA/ THALAMUS/ PONS

CORTEX/ DENTATE

WHITE MATTER HYPERINTENSITY 

SUBCORTICAL/ CEREBELLAR

MCP (RARE)

HYPERINTENSITY (T1)

IN HEPATIC FAILURE

BASAL GANGLIA

​

GLUTARIC ACIDURIA TYPE1

HYPERINTENSITY (T2/ FLAIR)

PUTAMEN/ POSTERIOR PUTAMEN

WHITE  MATTER HYPERINTENSITY

SPARING U-FIBRES

CERVICAL MYELOPATHY

MACROCEPHALY

BATWING FRONTOTEMPORAL ATROPHY

SUBDURAL HYGROMAS

​

L2 H-GLUTARIC ACIDURIA

HYPERINTENSITY (T2/ FLAIR)

CAUDATE/ PUTAMEN

GLOBUS PALLIDUS/ DENTATE 

CENTRIPETAL WHITE MATTER

U-FIBRE INVOLVED FIRST

CEREBELLAR WHITE MATTER

MACROCEPHALY

 

ATAXIA WITH OCULOMOTOR APRAXIA

HYPERINTENSITY (T2/ FLAIR)

DENTATE

​

CEREBROTENDINOSIS XANTHOMATOSIS

HYPERINTENSITY (T2/ FLAIR)

PERIVENTRICULAR

POSTERIOR LIMB INTERNAL CAPSULE

CEREBRAL PEDUNCLES

ANTERIOR PONS

HYPOINTENSITY (T2) 

CENTRAL DENTATE

​

COENZYME Q10 DEFICIENCY

COQ8A DEFICIENCY MOST COMMON (ADCK3)

ATROPHY 

CEREBELLUM â€‹

HYPERINTENSITY (T2/ FLAIR)

DENTATE/ PONS  

​

LEIGH SYNDROME

ATROPHY 

CEREBELLUM 

HYPERINTENSITY (T2/ FLAIR)

DENTATE

PERIAQUEDUCTAL

PONTINE TEGMENTUM

MEDULLA OBLONGATA

POSTERIOR PUTAMEN

​

FXTAS

HYPERINTENSITY (T2/ FLAIR)

MCP/DENTATE

CORPUS CALLOSUM (SPLENIUM)

PONS HYPERINTENSITY (FEMALES)

HYPERINTENSITY (DWI)

CORTICOMEDULLARY JUNCTION

RIBBON LIKE HYPER-INTENSITIES 

ATROPHY

CEREBELLAR ATROPHY

​

CONGENITAL DISORDER OF GLYCOSYLATION (GDC1a)

ATROPHY

CEREBELLUM

HYPERINTENSITY (T2)

DENTATE NUCLEUS

​

SCA48 (STUB1)

HYPERINTENSITY (T2/ FLAIR)

DENTATE + MCP HYPERINTENSITY: CRAB SIGN

POSTERIOR VERMIS ATROPHY

​

SPG7

CEREBELLAR ATROPHY

HYPERINTENSITY (T2)

DENTATE

​

METRONIDAZOLE

HYPERINTENSITY (T2/ FLAIR)

DENTATE

PERIAQUEDUCTAL GREY MATTER

TECTUM (INFERIOR COLLICULUS)

NPH/ MVN

7/6 CN NUCLEI

MAMMILLARY BODIES

MEDIAL NULEUS OF THALAMI

FRONTAL CORTEX

​

WERNIKE 

HYPERINTENSITY (T2/ FLAIR)

DENTATE

PERIAQUEDUCTAL GREY MATTER

TECTUM (INFERIOR COLLICULUS)

NPH/ MVN

7/6 CN NUCLEI

MAMMILLARY BODIES

MEDIAL NULEUS OF THALAMI

FRONTAL CORTEX

​

WEST NILE ENCEPHALITIS

HYPERINTENSITY (T2/ FLAIR)

DENTATE/ CEREBELLUM

BILATERAL THALAMUS

​

KELCH-LIKE P -11

HYPERINTENSITY (T2/ FLAIR)

PONS/ MCP

DENTATE

​

MULTIPLE SCLEROSIS

HYPERINTENSITY (T2/ FLAIR)
HYPOINTENSITY (T1) 

​

​T2 HYPOINTENSITY

ACERULOPLASMINAEMIA (CP)

HYPOINTENSITY (SWI/ GRE)

BASAL GANGLIA

RED NUCLEUS

THALAMUS

CAUDATE

CORTICAL PENCIL SIGN

HYPERINTENSITY (T2/ FLAIR)

WHITE MATTER HYPERINTENSITY

CEREBELLAR ATROPHY

​

NEUROFERRITINOPATHY (FTL1)

HYPOINTENSITY (SWI/ GRE)

BASAL  GANGLIA

RED NUCLEUS 

THALAMUS

CEREBRAL/CEREBELLAR ATROPHY

CYSTIC DEGENERATION OF BASAL GANGLIA

EYE OF TIGER

CORTICAL PENCIL SIGN

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HOT CROSS BUN

 

MSA-C

HYPERINTENSITY (T2/ FLAIR)

MCP

HOT CROSS BUN

ATROPHY

PONS 

​

SCA2

ATROPHY

BASAL PONS > TEGMENTUM

HYPERINTENSITY (T2/ FLAIR)

HOT CROSS BUN SIGN

​

SCA3

ATROPHY

PONS TEGMENTUM

CEREBELLUM/. BRAIN STEM

HYPERINTENSITY (T2/ FLAIR)

HOT CROSS BUN SIGN

​

SCA34 (ELOVL4)

​

SSPE

HYPERINTENSITY (FLAIR)

CORTICAL/ WHITE MATTER

PONS (RARE)/ MCP

HOT CROSS BUN

​

MA2 AB

​

KELCH LIKE P -11 AB

HYPERINTENSITY (T2/ FLAIR)

PONS/ MCP

DENTATE

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SUPERIOR VERMIS ATROPHY

​

ALCOHOL

​

SCA28 (AFGL32)

​

SCA29 (ITPR1)

INFANTILE ONSET

 

LATE ONSET FRIEDREICH'S ATAXIA

​

CANVAS

​

ARSACS

SCA IMAGING

​

SCA2

ATROPHY

BASAL PONS > TEGMENTUM

HYPERINTENSITY (T2/ FLAIR)

HOT CROSS BUN SIGN

​

SCA3

ATROPHY

PONS TEGMENTUM

CEREBELLUM/. BRAIN STEM

HYPERINTENSITY (T2/ FLAIR)

HOT CROSS BUN SIGN

​

SCA6

CEREBELLAR  ATROPHY

BRAIN STEM NORMAL

HYPERINTENSITY (T2/ FLAIR)

HOT CROSS BUN SIGN

 

SCA20

CALCIFICATION

DENTATE

HYPERINTENSITY (T2/ FLAIR)

INFERIOR OLIVARY NUCLEUS

HOT CROSS BUN SIGN

​

SCA48 (STUB1)

HYPERINTENSITY (T2/ FLAIR)

DENTATE + MCP HYPERINTENSITY: CRAB SIGN

ATROPHY

POSTERIOR VERMIS

​

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CEREBELLAR + MCP HYPERINTENSITY

​

ALEXANDER'S DISEASE

JUVENILE ONSET:

HYPERINTENSITY (T2/ FLAIR)

FRONTAL WMH

DENTATE/ CEREBELLUM/ MCP

ADULT ONSET:

ATROPHY OF MEDULLA AND SPINAL CORD

​

LBSL (LMNB1)

LEUKOENCEPHALOPATHY OF BRAINSTEM

AND SPINAL CORD AND LACTATE ELEVATION

ADULT ONSET LEUKODYSTROPHY

​

CEREBROTENDINOSIS XANTHOMATOSIS

HYPERINTENSITY (T2/ FLAIR)

PERIVENTRICULAR

POSTERIOR LIMB INTERNAL CAPSULE

CEREBRAL PEDUNCLES

ANTERIOR PONS

HYPOINTENSITY (T2) 

CENTRAL DENTATE

​

HEROIN

 

COCAINE

​

HISTIOCYTOSIS

​

HEPATOCEREBRAL SYNDROME

HYPERINTENSITY (T2/ FLAIR)

MCP HYPERINTENSITY 

HYPERINTENSITY (T1)

GLOBUS PALLIDUS

​

OTHER DISORDERS

 

DRPLA

HYPERINTENSITY (T2/ FLAIR)

CEREBELLUM/ PONS

CEREBRAL PERIVENTRICULAR AND SUBCORTICAL WM

ATROPHY

CEREBELLAR/ BRAIN STEM

DENTATE CAUSING DILATION OF 4TH VENTRICLE

​

ADRENOMYELONEUROPATHY

ATROPHY

CEREBELLAR/ SPINAL CORD

​

CANVAS (RFC1-AAGGG) 

ATROPHY

DORSAL AND ANTERIOR VERMIS

​

FRIEDREICH ATAXIA

ATROPHY

SPINAL CORD ATROPHY

LATE STAGE CEREBELLAR ATAXIA

DENTATE ATROPHY

HYPOINTENSITY (T2/ SWI)

DENTATE IRON DEPOSITION

​

MT-ATP6 ATAXIA

HYPERINTENSITY (T2/ FLAIR)

CEREBELLAR BOTTOM OF FISSURE

​

CEREBROTENDINOSIS XANTHOMATOSIS
HYPERINTENSITY (T2/ FLAIR)

PERIVENTRICULAR

POSTERIOR LIMB INTERNAL CAPSULE

CEREBRAL PEDUNCLES

ANTERIOR PONS

HYPOINTENSITY (T2) 

CENTRAL DENTATE

​

NIID (NOTCH2NLC GGC REPEAT)

HYPERINTENSITY (DWI)

CORTICOMEDULLARY JUNCTION

RIBBON LIKE HYPER-INTENSITIES 

HYPERINTENSITY (T2/ FLAIR)

LEUKODYSTROPHY

MCP HYPERINTENSITY

​

​

​

SCA
METABOLI
AUTOIMMUNE
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HEREDITARY SPASTIC PARAPARESIS

​

SPG7

CEREBELLAR ATROPHY

HYPERINTENSITY (T2)

DENTATE

​

SPG11

THIN CORPUS CALLOSUM,

HYPERINTENSITY (FLAIR)

EAR OF LYNX

​

SPG15

THIN CORPUS CALLOSUM,

HYPERINTENSITY (FLAIR)

EAR OF LYNX

​

SPG35 (FA2H)

CEREBELALR /BRAINSTEM ATROPHY

​

SPG47/SPG50/SPG51/SPG52 

HYPERINTENSITY (FLAIR)

THIN SPLENIUM CC 

EAR OF GRIZZLY (FORCEPS MINOR)

PERIVENTRICULAR WHM

​

ARSACS (SACS)

HYPOINTENSITY (T2) 

PONS CENTRAL

HYPERINTENSITY (FLAIR)

HORIZONTAL PONS STRIATIONS (CST)

PERIPHERAL PONS HYPERINTENSITY

RIM OF HYPERINTENSITY AROUNF THALAMI

ATROPHY 

SUPERIOR VERMIS

SPINAL CORD

CORPUS CALLOSUM

​

CEREBROTENDINOSIS XANTHOMATOSIS
HYPERINTENSITY (T2/ FLAIR)

PERIVENTRICULAR

POSTERIOR LIMB INTERNAL CAPSULE

CEREBRAL PEDUNCLES

ANTERIOR PONS

HYPOINTENSITY (T2) 

CENTRAL DENTATE

​

THIN CORPUS CALLOSUM

SPG11

SPG15

SPG21

SPG32

SPG35

SPG47

SPG48

​

SPORADIC

​

PRIMARY LATERAL SCLEROSIS

PRECENTRAL + PARIETAL CORTEX ATROPHY

HYPERINTENSITY (FLAIR)

CORTICOSPINAL TRACTS

​

STIFF PERSON SYNDROME

HYPERINTENSITY (FLAIR)

MESIAL TEMPORAL LOBE

​

SPINAL CORD WITH LEUKODYSTROPHY

​

ADRENOMYELONEUROPATHY

HYPERINTENSITY (T2/ FLAIR)

MOST PROMINANT IN THORACIC CORD

​

LBSL 

LEUKOENCEPHALOPATHY WITH BRAINSTEM SPINAL CORD LACTATE ELEVATION

HYPERINTENSITY (T2/ FLAIR)

PERIVENTRICULAR HYINTENSITY

POSTERIOR LIMB INTERNAL CAPSULE

DORSAL/ LATERAL COLUMN OF SPINAL CORD OR MEDULLAR OBLONGATE

SPECTROSCOPY: INCREASED LACTATE

 

ADULT POLYGLUCOSAN BODY DISEASE

AXONAL POLYNEUROPATHY

SPASTICTY

​

ALEXANDER DISEASE

JUVENILE ONSET:

FRONTAL WMH

ADULT ONSET:

ATROPHY OF MEDULLA AND SPINAL CORD

​

DARS1

HYPOMYELINATION WITH BRAIN STEM AND SPINAL HYPERINTENSITY IN CEREBRUM

HYPERINTENSITY (T2/ FLAIR)

CEREBELLUM/ BRAIN STEM. SPINAL CORD

METABOLIC

 

WERNIKE'S ENCEPHALOPATHY 

HYPERINTENSITY (T2/ FLAIR)

DENTATE

PERIAQUEDUCTAL GREY MATTER

TECTUM (INFERIOR COLLICULUS)

NPH/ MVN

7/6 CN NUCLEI

MAMMILLARY BODIES

MEDIAL NULEUS OF THALAMI

FRONTAL CORTEX

​

METONIDAZOLE TOXICITY

HYPERINTENSITY (T2/ FLAIR)

DENTATE

PERIAQUEDUCTAL GREY MATTER

TECTUM (INFERIOR COLLICULUS)

NPH/ MVN

7/6 CN NUCLEI

MAMMILLARY BODIES

MEDIAL NULEUS OF THALAMI

FRONTAL CORTEX

MITOCHONDRIAL

 

POLG1

HYPERINTENSITY (T2/ FLAIR)

INFERIOR OLIVARY NUCLEI

CEREBELLAR WHITE MATTER 

MCP/ DORSAL THALAMUS

​

MERRF

HYPERINTENSITY (T2/ FLAIR)

MEDIAL THALAMUS

DORSAL BRAIN STEM

LACTATE PEAK ON SPECTROSCOPY

​

COENZYME Q10 DEFICIENCY

COQ8A DEFICIENCY MOST COMMON (ADCK3)

ATROPHY 

CEREBELLUM â€‹

HYPERINTENSITY (T2/ FLAIR)

DENTATE/ PONS  

​

LEIGH SYNDROME

ATROPHY 

CEREBELUM 

HYPERINTENSITY (T2/ FLAIR)

DENTATE

PERIAQUEDUCTAL

PONTINE TEGMENTUM

MEDULLA OBLONGATA

POSTERIOR PUTAMEN

AUTOIMMUNE

 

GFAP IgG

HYPERINTENSITY (T2)

RADIAL LINEAR HYPERINTENSITIES 

CEREBELLUM/ PERIVENTRICULAR WHM

LONG SEGMENT CORD

CENTRAL CANAL

ADEM LIKE IMAGING

HYPERINTENSITY (T1 CONTRAST)

PUNCTATE CONTRAST HYPERINTENSITIES

SSPE

HYPERINTENSITY (FLAIR)

CORTICAL/ WHITE MATTER

PONS (RARE)/ MCP

HOT CROSS BUN

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