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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 

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 

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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