Case Presentation:

A 67‐year‐old man with hypertension and hyperlipidemia presented with a 3‐day history of intermittent confusion. His wife had also noticed changes in personality, irritability, ataxia of 3 weeks' duration, and impairment of short‐term memory. His medications included simvastatin 20 mg daily and atenolol 50 mg daily. On examination, his blood pressure was 168/94 mm Hg. He was alert with a flat affect and followed only simple commands. Comprehension of complex commands was impaired. Recent and remote memory were impaired. He displayed abulia and impaired judgment. Ideomotorand ideational apraxia were present. Snout reflex was positive. His extremity musculature was of normal tone, bulk, and strength. He displayed dystonic posturing of his left hand. Myoclonic jerks were noted periodically in the left upper extremity and rarely in the left lower extremity. Deep tendon reflexes were brisk, the left more so than the right. His gait was ataxic. Magnetic resonance imaging (MRI) revealed signal abnormalities in the head of the right caudate nucleus, right periventricular white matter, and also subparietal white matter bilaterally. The results of an electroencephalogram (EEG) were abnormal in the awake state because of periodic sharp waves occurring approximately once per second. The sharp waves were at times generalized and at times localized to the right centrotemporal region. The patient's routine labs for the evaluation of dementia included thyroid function testing and sero‐logic tests for syphilis and vitamin B12 levels, all of which were normal. Tests for paraneoplastic antibodies for limbic encephalitis (Hu, CV2, Ma, Yo, voltage‐gated potassium channel antibodies) were unrevealing. The test of cerebrospinal fluid (CSF) for 14‐3‐3 protein was positive. Based on the MRI, EEG, and CSF studies, the patient was diagnosed to have Creutzfeldt‐Jakob disease (CJD). He rapidly deteriorated during hospitalization, with his family opting for comfort care only.

Discussion:

Rapid progressive mental deterioration and myoclonus are the 2 cardinal clinical manifestations of CJD. Concentration, memory, and judgment difficulties are frequent early signs. Mood changes such as apathy and depression are common. Sleep disturbances may also be presenting signs, with hypersomnia more common than insomnia. With disease progression, dementia dominates the clinical picture in most patients and can advance rapidly. The differential diagnosis includes Alzheimer's disease, CNS lymphoma, gliomatosis cerebri, paraneoplastic syndromes, viral and postviral encephalitis, and toxic and metabolic encephalopathies.

Conclusions:

Clinicians should be aware of this rare cause of rapid cognitive decline in elderly hospitalized patients, particularly if routine evaluations are unrevealing.

Author Disclosure:

S. M. George, None; R. Bobba, None; T. J. Martin, None; D. Gullapalli, None; M. Bollu, None; M. M. Kazemi, None.