A 38‐year‐old man presented with gradual onset of forgetfulness, trembling, falling, and both visual and auditory hallucinations. A few months prior to admission, he became forgetful, had mood swings, and developed an unsteady gait with jerky limb movements. A few weeks prior to admission, he developed hallucinations. He was in a monogamous sexual relationship with a woman for 16 years. He denied intravenous drug use. He had no recent travel. Family history was unremarkable. On examination his Folstein Mini–Mental Status Examination (MMSE) score was 17 (scores < 24 suggest cognitive dysfunction). He was diffusely hypertonic and hyperreflexic and had frequent spontaneous myoclonic jerks in all extremities. Hemogram; basic metabolic, liver and thyroid function profiles; and cyanocobalamin and folate levels were all normal. Human immunodeficiency virus serology was negative. Rapid plasma reagin (RPR) done 2 days prior to admission at another hospital was negative. Magnetic resonance imaging of the brain showed moderate diffuse cerebral atrophy and nonspecific diffuse white matter hyperintensities. Electroencephalography showed moderate generalized slowing. Cerebrospinal fluid (CSF) analysis revealed moderate pleocytosis with lymphocytic predominance, elevated protein, Venereal Disease Research Laboratory (VDRL) test positive, and protein 14‐3‐3 negative. Repeat serum RPR was positive (with a titer of 1:64), and the Treponema pallidum hemagglutination (TPHA) test was positive. The patient was diagnosed with tertiary neurosyphilis and received penicillin G for 14 days. Six months after diagnosis, the patient reported improved memory and execution of complex activities, reduced episodes of myoclonus, and resolution of hallucinations. MMSE score improved to 25, RPR titer dropped to 1:16, and CSF was negative for VDRL and pleocytosis.
Myoclonus is a nonspecific symptom present in a large number of neurological diseases and can therefore be a diagnostic challenge for hospitalists. For our patient with rapidly progressive dementia with myoclonus, the differential diagnosis included prion disease, nonprion neurodegenerative diseases such as dementia with Lewy bodies, Alzheimer's disease, frontotemporal dementia, immune‐mediated central nervous system (CNS) diseases, paraneoplastic syndromes, and several rare CNS infectious and postinfectious syndromes. Classic teaching has been that no workup for dementia is complete without an evaluation for neurosyphilis. Indeed, even though the probability of neurosyphilis was low in our patient because of the initial negative RPR test, his abnormal CSF findings prompted a continued pursuit of neurosyphilis that resulted in treatment with significant improvement in symptoms.
Neurosyphilis can present as a rapidly progressive dementia with myoclonus. The CSF analysis and tests for syphilis should therefore be a part of the workup of rapidly progressive dementia in young inpatients.
L. Verda ‐ none; J. Baru ‐ none; G. Bhanushali ‐ none; B. P. Lucas ‐ none
To cite this abstract:Verda L, Baru J, Bhanushali G, Lucas B. A Case of Rapidly Progressive Dementia with Myoclonus. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 423. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/a-case-of-rapidly-progressive-dementia-with-myoclonus/. Accessed July 19, 2019.