Table 2.

Summary of MRI findings

	Patient 1	Patient 2	Patient 3	Patient 4
T2W hyperintense signal:
Globus pallidi	++	++	++	++
Putamina	+	+	+	+
Caudate heads	+	+	+	+
Thalami	−	−	−	+
Cerebral crura	++	++	++	++
Periaqueductal gray matter	−	+	+	+
Pons	+	−	−	+
Cerebellum	−	−	−	+
Restricted diffusion:	+	+	+	+
Brain volume loss:
Supratentoria	++	+	++	++
Infratentorial	++	−	+	+
MRS (Lactate peak):	+	+	−	+
Summary	Abnormal bilateral signal in the basal ganglia, midbrain, and dendate. Dilation of ventricles, significant progression in cerebral, and cerebellar atrophy. Thinning of corpus callosum.	Abnormal symmetrical bilateral signal in globus pallidus, putamen, head of caudate nuclei, subthalamic nuclei, and cerebral peduncles. Thalamus spared. No atrophy.	Symmetrical abnormal signal in globus pallidi, cerebral crura, caudate nuclei, putamina, lentiform nuclei. Prominent sulci and volume loss.	Focal bilateral symmetrical lesions in thalami, abnormal signal in lentiform nuclei, and caudate nuclei, dilation of ventricles, prominent sulci, and volume loss.

+ Positive, ++ Markedly Positive, − Negative; MRS = magnetic resonance spectroscopy.