Rapid-Onset Hemibody Sensory Loss, Incoordination, and Muscle Jerking

A 61-year-old woman with no pertinent medical history had progressive decline in multiple neurologic domains over the course of 2 months. She had development of progressive sensory loss in her left foot that subsequently spread up the left lower extremity and into the left upper extremity; accompanied by a sense of unsteadiness. Later, jerky movements of the left leg occurred while she was lying supine and sometimes when walking. At times, her left hand would wander involuntarily. Later in the course of her symptoms, mild short-term memory loss was also noted. On examination, she was unable to recall her home address, but findings were otherwise normal. She had mild gaze-evoked nystagmus and significant saccadic intrusion of smooth pursuits. A mild upper motor pattern of weakness, action myoclonus, hyperreflexia, and moderate loss of vibration was present on the left. Gait was markedly ataxic. Repeated magnetic resonance imaging of the brain 2 months after illness onset showed right parietal cortical hyperintense signal on diffusion-weighted imaging consistent with cortical ribboning, a common diagnostic finding early in the course of prion disease. Although characteristic of prion disease, similar imaging findings have been reported in autoimmune encephalitis and in the postictal setting. However, the putamen and caudate nucleus also demonstrated subtle asymmetric diffusion-weighted imaging hyperintense signal, which in the clinicoradiologic context is highly specific for prion disease. Electroencephalography showed frequent sharp wave discharges over right posterior temporal and left occipital head regions, along with frontal intermittent rhythmic delta slowing, consistent with encephalopathy (not otherwise specified). Real-time quaking-induced conversion testing of cerebrospinal fluid was positive for misfolded prion proteins. The positive real-time quaking-induced conversion result confirmed a diagnosis of sporadic Creutzfeldt-Jakob disease. The patient’s treatment was palliative. Hospice services implemented a home palliation program. Clonazepam was prescribed to reduce myoclonus. The patient died 18 weeks after onset of her neurologic symptoms. The differential diagnosis of a rapidly progressive multifocal neurologic syndrome includes many considerations but can be focused in complex situations by first confirming lesion localization and characterization with neuroimaging or other objective studies (eg, electromyography).