A 68 year old man is admitted to the hospital for delirium associated with a urinary tract infection. Upon adequate treatment of the infection, the patient's mental status improved significantly, though he is noted to remain paly disoriented. He also has an impairment in sho-term memory, difficulties in naming simple objects, and impaired concentration. His family members confirm an 8-month history of gradual progressive decline in cognitive abilities, which they attribute to old-age. However, the man is no longer able to manage his finances and has gotten lost while driving to the grocery store on two occasions. Prior to discharge from the hospital, the nursing staff repos that the patient continues to have urinary incontinence, though his infection has resolved. He is also noted to have a very unsteady gait, requiring assistance when walking. No other obvious signs or symptoms are present. Which disorder most likely accounts for this patient's dementia?
Normal pressure hydrocephalus is a potentially reversible cause of dementia that causes gait disturbances (unsteady or shuffling gait), urinary incontinence, and dementia. Enlargement of the ventricles with increased cerebrospinal fluid (CSF) pressure is found, and therapeutic lumbar punctures may significantly improve symptoms. Creutzfeldt-Jakob disease is a rare diffuse degenerative disease that usually affects people in their 50s, and the usual course is about one year. The terminal stage is characterized by severe dementia, generalized hypeonicity, and profound speech disturbances. It is one of several diseases presumably caused by prions. Huntington disease is a hereditary disease associated with progressive degeneration of the basal ganglia and the cerebral coex. It is transmitted in an autosomal dominant pattern. The onset of Huntington disease occurs between 35 and 50 years of age, or later in rare cases. This disease is characterized by progressive dementia, muscular hypeonicity, and bizarre choreiform movements. Parkinson disease is characterized primarily by motor dysfunction, but dementia may be a pa of the disorder. The characteristic motor symptoms (bradykinesia, flat facies, resting tremor, shuffling gait, etc) are caused by degeneration of the nigrostriatal dopaminergic tract. Ref: Ropper A.H., Samuels M.A. (2009). Chapter 30. Disturbances of Cerebrospinal Fluid and Its Circulation, Including Hydrocephalus, Pseudotumor Cerebri, and Low-Pressure Syndromes. In A.H. Ropper, M.A. Samuels (Eds), Adams and Victor's Principles of Neurology, 9e.
Creutzfeldt-Jakob disease
Huntington disease
Normal pressure hydrocephalus
Parkinson disease