A 52‐Year‐Old Man with Myoclonic Jerks

A 52-year-old man with an unremarkable clinical history started to complain of daily somnolence, unusual irritability and some memory lapses. A few months later, he had fever and developed confusion with myoclonic jerks of the left limbs. Upon admission a diagnosis of encephalitis was made. MRI FLAIR images (Figure 1a) showed cortical and sub-cortical abnormal signal intensity in parasagittal posterior frontal and anterior parietal regions, compatible with ischemic damage in watershed areas. On coronal T1weighted image (insert in Figure 1a) cortex was hyperintense for petechial hemorrhage. Multivoxel proton magnetic resonance spectroscopy (H-MRS) demonstrated lactic acid in the lesions. The CSF showed 10 white cells/mL with normal protein and glucose. He was treated with antibiotics, acyclovir, steroids and immunoglobulins and improved so much that he was discharged to a rehabilitation facility. The patient could play chess and walk around with the help of a walking frame. Two months later he again had fever with confusion and inappropriate mood. His myoclonic jerks and weakness was now bilateral. He had decreased alertness and was not able to walk or stand. MRI showed additional lesions in other cerebral regions. Axial FLAIR (Figure 1b) images demonstrated acute ischemic stroke in the right temporo-parietal area and in right parietal region at the vertex (insert in Figure 1b). DWI showed a marked hyperintensity due to cytotoxic edema. A small ischemic area was present in the right pulvinar (arrowhead in Figure 1b). A total body CT scan showed liver cirrhosis with left pleural effusion. The patient was treated again with antibiotics, acyclovir and antiepileptics without any improvement and a flaccid tetra paresis with muscular atrophy and areflexia developed in few weeks. The patient was then admitted to our institution with indication for brain biopsy. He was alert, in bad general condition and with fecal incontinence. He responded with vocalization and grimacing to painful stimuli. Hypotonic tetraplegia, diffuse muscle atrophy and areflexia were present with hypotonia more evident in the left limbs. An EMG study provided evidence of a severe axonal sensory-motor polyneuropathy. An EEG showed low voltage activity unresponsive to stimuli as in the alpha-coma condition. Brain MRI demonstrated large gliotic areas and cortex atrophy in the frontal and parietal regions and in the right temporo-parietal area as the result of ischemic strokes, and did not show additional lesions.