Figure 1. MRI features suggestive of sCJD. Axial
T2-weighted FLAIR images (a) show very subtle cortical hyperintensities,
best appreciated in the right parietal cortex (grey arrow). Axial
diffusion-weighted images (b) show subtle but more extensive bilateral
hyperintensities in multiple cortical regions (best appreciated in the
right parietal and left parieto-occipital cortices), caudate nuclei, and
anterior putamina (white arrows), with corresponding slight
hypointensities on apparent diffusion coefficient maps (c) representing
true diffusion restriction (black arrows).
Patient then developed psychomotor agitation, sleep disturbance, severe
confusion, and was caught trying to jump out of the window. She was
started on quetiapine 50 mg once daily and lorazepam 1 mg before sleep
to alleviate neuropsychiatric symptoms. Over the next week she developed
fever of over 40°C, muscular rigidity of all extremities, muscles of
mastication and jaw, as well as autonomic instability including high
blood pressure, tachycardia, profuse diaphoresis, and sialorrhea. Blood
and urine laboratory tests, chest and abdominal imaging revealed no
signs of possible infectious processes. Serum creatine kinase (CK) level
was elevated 1225 IU/l (normal 0-145 IU/l). With most infections being
ruled out, the likely diagnosis was NMS. Antipsychotic drugs were
immediately discontinued and specific treatment for muscular rigidity
with dopamine agonist bromocriptine was started. Further supportive care
was started with intravenous fluids to maintain volemia, antipyretics
and cooling blankets to reduce hyperthermia, trihexyphenidyl to manage
extrapyramidal symptoms; doses of antihypertensive drugs were increased.
Efficacy of NMS treatment was only partial as the patient still showed
signs of subfebrile fever, dysautonomia and muscle hypertonia, although
CK levels normalized.
During the next two weeks her neurological condition worsened. She had
exaggerated, startled responses to louder noises or sudden touch
(hyperekplexia), developed akinetic mutism, and lost all voluntary motor
function; a nasogastric tube had to be placed due to dysphagia.
On the 30th day of hospitalization another follow-up MRI was performed.
It showed persistent and slightly more pronounced bilateral diffusion
restriction in multiple cortical regions, caudate nuclei, and anterior
putamina, with similar subtle cortical T2W/FLAIR hyperintensities
(Figure 2). MRI findings continued to be suggestive of typical sCJD with
slight progression of signal abnormalities even on short-term follow-up.