Dr. H. Pernodjo Dahlan Sp.

S(K)
Department of Neurology School of Medicine Gadjah Mada University / Sardjito Hospital 2004

Definition
A clinical syndrome characterized by an acquired persistent impairment in at least three of the following domains of function: Language Memory Visuospatial skills Executive abilities Emotion

Dementia is a syndrome that can be produced by a wide variety of disorders: Reversible or irreversible Mild, moderate or marked severity Progressive, a stable course, partial remission of cognitive deficits

Contrasting Features of Dementia and Delirium

Feature
Onset Course Duration Attention Language Speech Visual Hallucinations Tremor Myoclonus

Delirium
Fluctuating Limited Impaired Incoherent

Dementia
Persistent Chronic Intact until advanced stages More coherent

Acute or sub acute Insidious

Slurred dysarthria Dysarthria uncommon Common Common Common Uncommon Uncommon Occurs in only a few types of

Two General Types of Dementia

1. Cortical Dementia 2. Sub-Cortical dementia

Function Psychomotor speed Language Memory: -Recall -Recognition -Remote Executive function Depression Apathy

Distinguishing Characteristics of Cortical and Subcortical Dementias

Cortical Dementia Normal Involved -impaired -impaired -temporal gradient present Less involved Less common Less common Spared until late Cerebral cortex Subcortical Dementia Slowed Spared -impaired -spared -temporal gradient absent More involved More common More common Involved early Subcortical structures and dorsolateral prefrontal cortex projecting to head of caudate

Motor system Anatomy

Etiologies of Dementia
Dementia Alzheimers disease Vascular dementia Depression Alcohol-related dementia Metabolic disturbances Toxic disturbances Hydrocephalus Anoxia brain injury Central nervous system infections Brain tumors Brain trauma Subdural hematoma Other Frequency (%) 50-60 10-30 5-15 1-10 1-10 1-10 1-5 1-2 1-2 1-2 1-2 1-2 10-20

Alzheimers Disease
AD is a progressive neurodegenerative disorder that produces a clinical syndrome termed dementia of the Alzheimer type (DAT)

AD
An insidious onset Progresses gradually to death Age related Increasingly after age 65 years Survival: 10 years from the time of diagnosis

The Earliest Changes

Impaired memory A change in personality characterized by indifference Disoriented easily Anomia

Final Phase
No memory function Language is reduced Echolalia Palilalia Incoherent verbalization Loses the ability to walk Incontinence Delusions Anxiety Dysphoria Agitation

Diagnostic Criteria for Definite Alzheimers Disease

Clinical criteria for probable AD Histopathologic evidence of AD (autopsy or biopsy)

Diagnostic Criteria for Probable Alzheimers Disease

Onset between ages 40 and 90 years No disturbance of consciousness (e.g. not in a delirium) Dementia established by clinical examination and documented by mental status questionnaire Dementia confirmed by neuropsychological testing Progressive worsening of memory and other cognitive functions Deficits in memory and in at least one other cognitive function (language, visuospatial abilities, calculation, executive function, praxis, gnosis) Absence of systemic disorders or other brain disease capable of producing a dementia syndrome (disease such as Parkinsons disease and frontal lobe degeneration are identified and excluded by clinical examination; other potential causes of dementia are detected and excluded by laboratory test and neuroimaging)

Diagnostic Criteria for Possible Alzheimers Disease

Presence of a systemic disorder or other brain disease capable of producing dementia but not thought to be the cause of patients dementia Gradually progressive decline in a single intellectual function in the absence of any other identifiable cause (e.g. memory loss, aphasia)

Unlikely AD
Sudden onset Focal neurologic sign Seizure or gait disturbance early in the course of the illness

Diagnostic Test
No diagnostic test for AD Serum, urine and routine CSF test are normal EEG: slowing of dominant posterior rhythm, increasing theta and delta CT Scan and MRI: non specific cerebral atrophy SPECT: diminished cerebral blood flow PET: diminished neuronal metabolism in AD, most severe changes inferior parietal and parietotemporal junction areas

Two Basic Processes of Pathogenesis of AD

1. Abnormalities of the amyloid precursor protein (APP) or of the metabolism to amyloid lead the accumulation of -amyloid in neuritic plaques and leptomeningeal blood vessel. Amyloid may be toxic to neurons and contributes to cell death. There is abnormal phosphorilation of the tau protein associated with neuron microtubules, leading to formation of neurofibrillary tangles. Inheritance of mutations of chromosomes 21 and 14 appears to cause the abnormalities of amyloid processing, whereas inheritance of the APOE-4 allele of chromosome 19 may facilitate both accumulation of amyloid and formation of neurofibrillary tangles

2.

Standard Assesment of The Dementia Patient

Laboratory Tests: Complete blood count Erythrocyte sedimentation rate Electrolyte, blood glucose, BUN Serun calcium and phosphorus TSH Serum Vitamin B12 level Fluoroscent treponemal antibody absorbtion test (FTA-ABS) Neuroimaging: CT or MRI

Standard Assesment of The Dementia Patient

Useful systemic Assessments: Electrocardiograms Chest X-ray Urinalysis Optional tests: Lumbar puncture EEG and quantitative EEG SPECT PET Serum antiphospholipid antibody Serum or urine drug tests Radionuclide cisternography Serum HIV antibodies