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.