DEGENERATIVE DISEASES

Penyakit degeneratif SSP dapat mengenai :

1. Substansia abu
2. Substansia putih
3. Kombinasi
Daerah yang terkena :
- Sering kali difus
- Biasanya satu area atau sistem lebih dari lainnya

KLASIFIKASI ANATOMIS

KLASIFIKASI ANATOMIS
I.
II.
III.
IV.
V.

Diseases of the gray matter

Hereditary diseases of white matter
Neuroectodermal degeneration phakomatoses
Diseases of mitochondria
Disorders of cerebrospinal fluid and circulation

I. DISEASES OF GREY MATTER

DISEASES OF THE CEREBRAL CORTEX :

DEMENTIA

DISEASES OF GRAY MATTER

DISEASES OF THE CEREBRAL CORTEX.
DEMENTIA
- A progressive deterioration of intellectual capacity resulting from
diseases of the brain
- 80% due to Alzheimer disease and multi-infarct dementia
- The third leading of death in old age group
- Clinical manifestation :
- Memory impairment (predominant) and impaired of :
- Language
- Perception
- Visuospatial function
- Calculation
- Judgment
- Abstraction
- Problem solving skills

DISEASES OF GRAY MATTER ( lanj )

DISEASES OF THE CEREBRAL CORTEX.
DEMENTIA
- In Degenerative Disease, the etiologies are unknown, but
dementia is a predominant sign
- The group of diseases with dementia :
- Alzheimer disease
- Pick disease
- Multisystem disease
- Progressive supranuclear palsy
- Huntington disease
- Lewy body disease
- Cortical basal degeneration
- Creutzfeldt-Jacob disease
- Fronto-temporal dementia
- Normal Pressure Hydrocephalus (NPH)
- Vascular dementia

Evaluation of Patients with Dementia

1. The history obtained from the patient and other ones
Early cases : punctuated by denial
Advanced cases : sparse, fragmented, hesitant

2. The neurological examination

MMSE
Primitive reflexes
Hypertonia or paratonia
Dyspraxia
Abnormal in reflexes examination

3. The general physical examination

Important to exclude signs of: hypoparathyroidism,
vascular disease with hypertension, cardiac murmurs,
carotid bruit, Vit B12 deficiency

4. Diagnostic procedures

CAUSES OF TREATABLE DEMENTIA

Therapeutic drug use :

- Anticholinergics atropine and related comp[ounds
- Anticonvulsants : phenytoin, mephenytoin, barbiturates
- Antihypertensives : clonidine, methyldopa, propanolol
- Psychotropics : haloperidol, lithium carbonate, phenothiazine
- Miscellaneous : disulfiram, bromides, paraldehyde, quinidine

Metabolic systemic disorders :

Elctrolyte or acid-base disorders, hypo/hyperglycemia, severe
anemia, polycythemia vera, hyperlipidemia, hepatic failure,
uremia, pulmonary insufficiency, hypopituarism, thyroid, adren
or parathyroid dysfunction, cardiac dysfunction, hepatolenticu
degeneration

Intracranial disorders :
Cerebrovascular insufficiency, chronic meningitis or encephali
neurosyphilis, HIV, epilepsy, tumor, abscess, subdural hemato
multiple sclerosis, NPH

CAUSES OF TREATABLE DEMENTIA ( lanj )

Deficiency states :
Vitamin B12 deficiency, folate deficiency, pellagra (niacin)
Collagen-vascular disorders :
SLE, temporal arteritis, sarcoidosis, Behcets syndrome

Exogenous intoxication :
Alcohol, CO, organophosphates, toluene, trichloroethylene,
carbon disulfide, lead, mercury, arsenic, thallium, manganes
nitrobenzene, anilines, bromine hydrocarbone

EVALUATION OF THE PATIENT WITH DEMENTIA

Complete history,
neurological and
general physical
examination

Special emphasis on
history of drug use
evidence of intoxication,
evidence of
systemic disease

Neuropsychological
examination

CBC, sedimentation rate,

electrolytes, BUN, creatinine,
glucose, calcium, phosphate,
liver enzymes, vit.B12, folate,
thyroid function, serologic test
for HIV and syphilis, urinalysis

If indicated
blood gases,
heavy metal and
intoxication screen

ECG, CT-scan, MRI,

MRA, MRI-spectroscopy,
SPEC, PET

LP, radioisotope
cisternography
if available

EEG, AEP, VEP

If available

Metastatic work-up
if indicated

Consider cerebral angiography

if CT-scan equivocal of if CVD or
arteritis is suspected

Gilroy J, 2000

DISEASES OF GRAY MATTER :

ALZHEIMER DISEASE
- Most common form of dementia
- Caused by progressive neuronal degeneration
- 70% in middle age and elderly individuals
- 1% in age 50-70 years, 50% in very elderly people
- The etiology is unknown
- USA : prevalence is 4 million people over age 65 years

ALZHEIMER DISEASE
PATHOLOGICAL APPEARANCE

Amyloid plaques
consist of -amyloid peptide
(a fragment of precursor protein)

Neurofibrillary tangles
a core of an abnormally phosphorylated form of the
microtubule binding protein tau

DSM-IV DIAGNOSTIC CRITERIA FOR AD

A. The development of multiple cognitive deficits manifested by both :
1). memory impairment
2).One (or more) of the following cognitive disturbances : a). aphasia, b).
apraxia, c). agnosia, d). disturbance in executive functioning ( planning,
organization, sequencing, abstracting )
B. The cognitive deficits in criteria A1 and A2 each cause severe impairment in
social or occupational functioning and represent a major decline from a
previous level of functioning
C. The course is characterized by gradual onset and continuing cognitive
decline
D. The cognitive deficits in criteria A1 and A2 are not due to any of the following:
1).other central nrvous system conditions that cause progressive deficits in
memory and cognition (for example, cerebrovascular disease, Parkinsons
disease, Huntingtons disease, subdural hematoma, NPH, brain tumor),
2).systemic conditions known to cause dementia (for example:
hypothyroidism, vit.B12 and folic acid deficiency, niacin deficiency,
hypercalcemia, neurosyphilis, HIV infection)
E. The deficits do not occur exclusively during the course of delirium
F. The disturbance is not better accounted for by any other Axis I disorder (for
examp-le: major depressive disorder, schizophenia)

American Psychiatric Association (APA): Diagnostic and Statistic Manual of Mental Disorders 4 th ed, 1994

TREATMENTS
1. Diet
2. Adequate care
3. Planning with family
4. Pharmacotherapy :
Sedation and antidepressant
Drug therapy (Tacrine, Donepezil HCl)