Table 6 Diagnostic alternatives in relation to the pattern of lesions on brain MRI (from ref. [74], modified)

Region of white matter involvement

Clinical conditions

Letter: It permits to identify the occurrence of the same disease in other rows

 Frontal

- Alexander disease

- metachromatic leukodystrophy

- megalencephalic leukoencephalopathy with subcortical cysts

- variant adrenoleukodystrophy

a

b

 Parieto-occipital

- Adrenoleukodystrophy

- Krabbe disease

- paroxisomal disorders

- mitochondrial disorders

c

d

e

 Temporal

- CADASIL

- Aicardi-Goutières syndrome

- Cytomegalovirus encephalitis

- megalencephalic leukoencephalopathy with subcortical cysts

f

g

 Periventricular

- Hemophagocitic lymphohisiocytosis

- metachromatic leukodystrophy

- leukoencephalopathy with brainstem-spinal cord involvement and elevated lactate

- vanishing white matter disease

- CADASIL

b

h

f

 Basal ganglia

- mitochondrial disorders

- biotinidase deficiency

- Wilson disease

- Bilateral striatal necrosis

- Biotin-responsive basal ganglia disease

- Aicardi-Goutières syndrome

e

g

 Thalamus

- Malignancies

- Hepatic encephalopathy

- Acute necrotizing encephalopathy

- leukoencephalopathy with thalamus and brainstem involvement and elevated lactate

- Fabry disease

i

l

m

n

 Brainstem

- Malignancies

- Rhombencephalitis

- Tuberculosis

- Bechet disease

- Alexander disease

- leukoencephalopathy with thalamus and brainstem involvement and elevated lactate

- leukoencephalopathy with brainstem-spinal cord involvement and elevated lactate

i

a

m

h

Involvement of white matter tracts

 Corticospinal

- adrenoleukodystrophy

- adrenomyeloneuropathy

- Krabbe disease

- adult-onset leukoencephalopathy with axonal spheroids and pigmental glia

c

d

 Dorsal column

- leukoencephalopathy with thalamus and brainstem involvement and elevated lactate

- vitamin B12/copper deficiency

m

 Subcortical U-fibers

- Canavan disease

- Kearns-Sayre syndrome

 External capsule/insula

- Acute necrotizing encephalopathy

- CADASIL

l

f

 Corpus callosum

- Susac syndrome,

- Krabbe disease

d

Spinal cord

 Hyperintensity

- Sarcoidosis

- Systemic lupus erythematosus

- CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids)

- infections

 Atrophy

- leukoencephalopathy with thalamus and brainstem involvement and elevated lactate

- Alexander disease

- adult polyglucosan body disease

m

a