1. Vascular dementia (Multi-infarct dementia)
The second most common (15-25%) form of dementia.
Caused by microvascular disease in the brain that produces multiple small infarcts.
A substantial infarct burden must accumulate before dementia develops.

2. Risk factors
Stroke DM HT
APOe4
M:W is 2:1

3. Clinical manifestations
Step-wise loss of function as the microinfarcts add up
no specific pattern of cognitive deficits.
Neuropsychological testing may not differentiate vascular Dementia from AD.
Lateralizing signs: Spasticity, hemiparesis, ataxia, pseudobulbar palsy (inability to control facial movement)
Depression, anger and paranoia are common.

4. Treatment No truly effective treatment
Physical and emotional support, proper nutrition, exercise, and supervision
cholinesterase inhibitors (successful tt)
AntiHTN may prevent onset of vascular demintia

5. VASCULAR DEMENTIA VERSUS ALZHEIMER’S
Since vascular dementia is caused by small brain infarcts, patients also have focal neurological symptoms (such as hyperreflexia or paresthesias).
Onset is usually more abrupt than Alzheimer’s.
Greater preservation of personality.
Can reduce risk by modifying risk factors (such as smoking, hypertension, and diabetes).

6. Lewy body dementia LBD The third most common dementia (10-15%)
Caused by lewy bodies and lewy neurites in the brain (primarily in the basal ganglia).
LEWY BODIES

7. Clinical manifestations
Progressive cognitive decline.
Waxing and waning of cognition.
Parkinsonism.
Visual hallucinations.
Sensitivity to neuroleptics (antipsychotics).
Paranoid delusions
REM sleep behavior disorders is common.
Core features

8. Diagnosis Strong similarity to Parkinson disease dementia: In LBD:
In PD:
Onset of Parkinsonism
12 months
Dementia
Dementia

9. Treatment
Cholinesterase inhibitors may help improve visual hallucinations.
Psycho-stimulants, L-Dopa and Carbi-Dopa, and dopamine agonists may improve cognition, apathy and psychomotor slowing.
Atypical Neuroleptics have been slightly effective in stopping delusions.
Clonazepam treatment for REM sleep behavioral disorders.

10. Huntington’s Disease
Autosomal dominant genetic disorder that results in progressively disabling cognitive, physical, and psychological functioning, ultimately resulting in death after approximately 15 years
Involves loss of GABA-ergic neurons in basal ganglia
CLINICAL MANIFESTATIONS
Onset: 35 to 50 years of age
Hallmarks:
Progressive dementia
choreiform movements (dancelike flailing of arms and legs)
Muscular hypertonicity
Depression and psychosis very common

11. PATHOLOGY
Trinucleotide repeat (CAG) on short arm of chromosome 4; primarily affects basal Ganglia. Involves loss of GABA-ergic neurons in basal ganglia
DIAGNOSIS
MRI shows caudate atrophy (and sometimes cortical atrophy). Genetic testing and MRI are diagnostic.
TREATMENT
There is no effective treatment available (supportive only).

14. Parkinson’s Disease
* Progressive disease with prominent neuronal loss in substantia nigra, which provides dopamine to the basal ganglia, causing physical and cognitive impairment.
Approximately 30% of patients with Parkinson’s disease develop dementia.
50% will suffer from depression.
Slightly more in men

16. CLINICAL MANIFESTATIONS
Bradykinesia (slowness in the execution of movement)
Cogwheel rigidity
Resting tremor
Mask facial expression
Shuffling gait
Dysarthria (abnormal speech)

18. ETIOLOGY
Idiopathic (most common)
Traumatic
Drug- or toxin-induced
Encephalitic
Familial (rare)
PATHOLOGY AND PATHOPHYSIOLOGY
Loss of cells in the substantia nigra of the basal ganglia, which leads to a decrease in dopamine and loss of the dopaminergic tracts

19. TREATMENT Levodopa—degraded to dopamine by dopadecarboxylase
Pharmacologic
Levodopa—degraded to dopamine by dopadecarboxylase
Carbidopa—peripheral dopadecarboxylase inhibitor prevents levodopa from being converted to dopamine before it reaches the brain.
Amantadine—mechanism unknown
Anticholinergics—help relieve tremor
Dopamine agonists (bromocriptine, etc.)
Monoamine oxidase (MAO)-B inhibitors (selegiline)—inhibit breakdown of dopamine
Levodopa crosses the
blood–brain barrier (BBB).
Carbidopa does not.
Carbidopa prevents
conversion of levodopa to
dopamine in the periphery.
Once levodopa crosses BBB,
it is free to be converted to
dopamine.
Antipsychotic medications exacerbate demintia in parkinson dis

21. Creutzfeldt-Jakob disease CJD
A rapidly progressive degenerative disease of the CNS caused by accumulation of abnormal forms of prions.
Affects older patients, and may be inherited, sporadic or acquired.
A small percentage of pt. have become infected through corneal transplant

22. Clinical manifestations
Rapidly progressive dementia 6-12 months after onset of symptoms.
More than 90% of patients have myoclonus (sudden spasm of muscles).
Basal ganglia and cerebellar dysfunctions are common.
Personality changes, immature behavior and paranoia are early signs.
Rapid progression to stupor, coma and death in a matter of months to a few years.

23. Diagnosis
Definitive: Pathological demonstration of spongiform changes of brain tissue.

24. Probable: The presence of rapidly progressive dementia and Periodic Generalized Sharp Waves on EEG + two or more of the following
Myoclonus.
Cortical blindness.
Ataxia, pyramidal signs or extrapyramidal signs.
Muscle atrophy.
Mutism.

25. Incurable and Invariably fatal!
Treatment
Incurable and Invariably fatal!

26. Pick’s Disease/ Frontotemporal Dementia (FTD)
Typically between years.
20-30% are familial.
Mean duration of illness to death: 4-6 years
CLINICAL MANIFESTATIONS:
Profound changes in personality and social conduct.
Disinhibited verbal, physical and sexual behavior.
Echolalia, overeating, oral exploration of inanimate objects.
Lack of emotional warmth, empathy and sympathy.
Poor insight about behavioral alterations.
Cognitive deficits in attention, abstracting, planning and problem solving.
Memory, language and spatial functions are well preserved.
Kluver bucy syndrome * hyerphagia , hypersexuality

27. PATHOLOGY
Atrophy of frontotemporal lobes.
Pick bodies— intraneuronal inclusion bodies (not necessary for diagnosis of FTD)
TREATMENT:
* Anticholinergic medications and antidepressants have consistently shown to improve behavioral symptoms but not cognition.

29. HIV associated dementia
HAD is the most common dementia caused by infectious disease.
The prevalence increases as the patient lives longer.
Caused by:
The direct effect of the virus on the cells.
Infections due to neutropenia

30. Risk factors:
Duration of the illness.
Low CD4.
High viral loads
Clinical manifestations:
Rapid decline in cognition, behavior and motor ability.
Poor memory and impaired concentration.
Psychomotor retardation.
Apathy and social withdrawal.
Depression.
Preserved language.
Treatment:
HAART
Psychostimulants.

31. Normal Pressure hydrocephalus
NPH is a reversible cause of dementia. Patients have enlarged ventricles with no increased CSF pressure.
Etiology
idiopathic
secondary to obstruction of CSF reabsorption sites due to( trauma, infection, or hemorrhage).
* Clinical triad:
1. Gait disturbance (often appears first) Wobbly
2. Urinary incontinence Wet
3. Dementia (mild, insidious onset) Wacky
TREATMENT
Relieve increased pressure with shunt.
Of the clinical triad, the dementia is least likely to improve.

32. Thank You