NEUROLOGY 02

A 66-year-old female is brought to you by her concerned son. She has increasing confusion with loss of mobility and stiff limbs. She does not have any problems with her joints. She tends to cry out for no reason. She often screams and says that she is seeing a lion roaring in the backyard. In addition, she often sees cats in her room, even though there are none. She was treated with haloperidol, but that aggravated her rigidity. She also has significant memory loss. She is a non-smoker. She has no significant past psychiatric history. She is alert, but disoriented and quite agitated. On examination, increased tone and normal reflexes with coarse resting tremors are noted in the extremities. Her visuospatial abilities are quite impaired. Her vitals are, BP: 136/72 mm of Hg; PR: 98/min; RR: 16/min. Her CBC, electrolytes, creatinine, glucose, LFTs, TSH and B12 levels are within normal range. Serology for syphilis is negative. Based on this history and examination what is the most likely diagnosis?
A. Lewy body dementia
B. Alzheimer’s disease
C. Multi infarct dementia
D. Neurosyphilis
E. Pick’s disease

Explanation:
Dementia with Lewy body (DLB) is characterized by fluctuating cognitive impairment. Parkinsonism is also seen with poor response to dopaminergic agonist therapy. Hallucinations are visual and bizarre. The central feature required for the diagnosis of dementia with Lewy bodies (DLB) is progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function. Prominent or persistent memory impairment may not necessarily occur in the early stages but is usually evident with progression. Deficits on tests of attention and of frontal-subcortical skills and visuospatial ability may be especially prominent.

Two of the following core features are essential for a diagnosis of probable DLB, and one is essential for possible DLB:
a.Fluctuating cognition with pronounced variations in attention and alertness
b.Recurrent visual hallucinations that are typically well formed and detailed
c.Spontaneous motor features of Parkinsonism

Features supportive of the diagnosis are:
1.Repeated falls
2.Syncope
3.Transient loss of consciousness
4.Neuroleptic sensitivity
5.Systematized delusions

Alzheimer’s disease is a progressive dementia with associated risk factors including: age; female gender; positive family history; head trauma; Down’s syndrome. Typical first symptoms are: subtle memory loss; language difficulties; apraxia; followed by impaired judgment; and personality changes. Treatment is specifically targeted to specific symptoms and includes psychosocial intervention and pharmacological therapy.

Multi infarct dementia accounts for 15-20% cases of dementia. There is cognitive dysfunction with motor and sensory neurological dysfunction. Risk factors are: older age, male sex, black race, cigarette smoking, hypertension, diabetes and vasculitis. Treatment is generally directed towards the risk factors.

Neurosyphilis appears in 15-20% of late syphilis cases. There are four types. In general paresis type, there is general involvement of cerebral cortex. There is usually decrease in concentrating power, memory loss, dysarthria, tremors of finger and lips, irritability, mild headaches. In addition, there are personality changes with the patient becoming slovenly, irresponsible, confused and psychotic.

Pick’s disease is one of the groups of diseases labeled as Fronto temporal dementia. Patients manifest personality change (euphoria, disinhibition, apathy) and compulsive behaviors such as peculiar eating habits or hyperorality. Memory is impaired with visual-spatial functions usually intact.