PMC6777289_01
Photographs of the MRI scan of the brain of the patient with SSPE. (A)
Sagittal magnetic resonance imaging (MRI) T1-weighted image of the brain showing diffuse
atrophy. (B) Axial T1-weighted post-IV gadolinium showing mild subcortical enhancement
in the right parietal lobe (blue arrows). (C) Axial MRI T2-weighted image of the brain
showing asymmetric hyperintensities best noted in the right temporoparietal region (blue
arrows). (D) Axial MRI T2-FLAIR-weighted image of the brain showing diffuse
hyperintensities in mesial temporal lobes bilaterally (blue arrows). (E) Axial MRI
T2-FLAIR-weighted image with hyperintensities best seen in the frontal lobes (blue
arrows) bilaterally.
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Cranial magnetic resonance imaging (MRI) showed diffuse brain atrophy (Figure 1A) and mild subcortical enhancement in the right parietal lobe (Figure 1B). There was also asymmetric multiple hyperintensities best noted in the right temporoparietal region (Figure 1C), mesial temporal lobes (Figure 1D), and frontal lobes bilaterally (Figure 1E).
Phenomenology: Segment 1. The patient with SSPE at admission: The
involuntary motor activity consisted of abnormal, sudden, segmental, brief multifocal,
and predominantly distal muscle jerks involving the patient's upper limbs more on
the left than the right side. The phenomenon was observed purely in wakefulness. It was
accompanied by dystonia of both legs with the knees flexed at 90 . The patient
tried to stop the abnormal movement disorder unsuccessfully using the right hand which
was only partially involved. The myoclonic jerks usually commenced sharply in the first
hour of awake and remained unchanged throughout wakefulness. There was an observable
pattern of one to two sequences of muscle contractions every 2-3 seconds
continuously. This phenomenon occurred numerous times every day for 15 days.. Segment 2. The patient from Segment 1 at Follow-up: These myoclonic jerks,
dystonia, and spasticity responded completely to the treatment with a combination of
clonazepam 0.5 mg and valproic acid 200 mg orally twice daily. At 3 months of follow-up
and with compliance to treatment, these movements have not returned.
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An electroencephalogram (EEG) showed a poorly sustained background with frequent intermittent slow delta wave activity with a frequency of approximately 4 per second observed in the frontal areas bilaterally (Figure 2 submitted as Supplemental file 2).