Algorithm for dementia

1. Algorithm for Dementia
By
Khaled Osama Abo-Shae3ra
Lecturer of Neurology
Assiut University

2. Memory
Impairment
Apraxia
Executive
dysfunction
Aphasia Agnosia
Cognitive Domains

3. Definition
Sustained and significant deterioration of two or more
domains of cognitive functions, one of which must be
memory, in an alert person.
While Delirium
is fluctuating
Interfere with normal
work or social function
While MCI not affect
daily activity
While in Delirium
Pt. is inattentive
Memory impairment
Executive dysfunction
Aphasia
Apraxia
Agnosia

4. Not
Delirium
Not
Depression
Not
MCI
• Within hours; global memory failure; fluctuating
• Poor insight; lethargic or hyper-vigelence;
disoriented; visual & auditory hallucination
• Inattentive during cognitive tasks
• Impaired activity of daily living
• Within weeks; specific memory gaps; worse at
morning;
• Good insight; normal alertness, orientation &
perception
• Poor effort during cognitive tasks
• Impaired activity of daily living
• Within months; recent memory impairment
• Good insight; normal alertness, orientation &
perception
• Good effort during cognitive tasks
• Normal activity of daily living

5. ? Dementia
Exclude: Normal aging,
MCI, Depression, DeliriumClinical evaluation, MMS, ADL
Dementia
Non-progressive
or regressive
Post CNS insult
• as post Anoxic,
Encephalitic,
Meningitic,
Traumatic,
Toxic, SAH,
Irradiation,
Ictal, ECT
Rapidly
progressive
Treatable
• as metabolic,
toxic,
Un-treatable
• as Prion, SSPE,
PMLE, Rapidly
prog. degen.
Stepwise
progression
Vascular
• Acquired: as
strategic
infarction
• Inherited: as
CADASIL,
Fabry’s
Slowly
Progressive
Degenerative
• Pure dementia:
as ALZ, FTLD
• Dementia Plus
– ˃ 40 year
– 20-40 year
– ˂ 20 years
History, Drug,
Toxic Screening
Lap, MRI, EEG,
CSF
Clinical, CT, MRI Clinical, MRI

6. Rapidly progressive dementia

7. Rapidly progressive dementia
1. Exclude treatable causes
Metabolic
Endocrinal
Nutritional
Immune
Drugs
Toxins
Intracranial
disorders
Uremic, Hepatic, Electrolyte, Acid-base disorders, Anemia,
Polycythemia, Cardiac or pulmonary dysfunction
Hypo- , Hyper-glycemia, Hypothyroidism, Hypopituitarism,
Adrenal insufficiency
Vitamin B12 or Folate deficiency, Pellagra
SLE, Temporal arthritis, Behcet’s, Sarcoidosis
Lithium, Halopridol, Phenytoin, Barbiturate, Atropine,
Propranolol, Methyl dopa
Alcohol (Bockouts, Wernicke, Korsakoff, Marchafava Bgnami),
CO, Organophosph., Lead, Hg, Arsenic, Thalium, Manganese
Subdural hematoma, Tumors, Abscess, Chronic meningitis,
Encephalitis, Cerebrovascular insuffeciency, Normal pressure
hydrocephalus, Non-convulsive status, Multiple sclerosis

8. Rapidly progressive dementia
2. With myoclonus, periodic EEG
a) Adult → Prion
b) Child → SSPE
3. With suppressed immunity, subcortical lesions
→ PMLE
4.Without all of the above
→ Rapidly progressive degenerative variant
Lewy body dementia, Multiple system atrophy,
Alz, Dementia MND

9. Stepwise progressive dementia

10. Stepwise progressive dementia
1. Without systemic manifest. or family h.
a) Strategic infarction
- Large vessels:

11. Stepwise progressive dementia
1. Without systemic manifest. or family h.
a) Strategic infarction
- Large vessels:

12. Stepwise progressive dementia
1. Without systemic manifest. or family h.
a) Strategic infarction
- Large vessels:

13. Stepwise progressive dementia
1. Without systemic manifest. or family h.
a) Strategic infarction
- Large vessels:
- Watershed:

14. Stepwise progressive dementia
1. Without systemic manifest. or family h.
a) Strategic infarction
- Large vessels:
- Watershed:
- Small vessels:

15. Stepwise progressive dementia
1. Without systemic manifest. or family h.
a) Strategic infarction
- Large vessels:
- Watershed:
- Small vessels:
b) Periventricular lacunes & leukoariosis
- Binswanger’s disease

16. Stepwise progressive dementia
2. With systemic manifest. or family h.
A. Migraines & Stroke-like episodes
→ CADASIL
B. Parenthesia, cornea verticillata, angiokeratoma corporis
diffusum → Fabry
C. Peripheral microhemorrhages in T2*
→ Cerebral Amyloid Angiopathy

17. Slowly progressive dementia
I- Pure Dementia

18. Initial Symptom
• Memory → Alzheimer
• Personality → Pick's disease
• Motor speech → 1ry prog. non-fluent aphasia
• Sens. speech → Sementic dementia
• Sens. prosody, face recog. → Rt temp. variant
• Reading, object recog. → Post. cortical atrophy
Slowly progressive dementia
I- Pure Dementia

19. Slowly progressive dementia
II- Dementia plus

20. II- Dementia plus
Age >40 year

21. II- Dementia plus
Age >40 year
+ Parkinson. alone
+ Parkinson. + Personality change
+ Parkinson. + MND
+ Parkinson. + Down gaze palsy
+ Parkinson. + Alien limb
+ Parkinson. + Ataxia
+ MND + Gynecomastia
+ MND alone
+ Chorea + Personality change
PD dementia & advanced AD
Lewy body dementia
Progressive supranuclear palsy

22. II- Dementia plus
Age >40 year
+ Parkinson. alone
+ Parkinson. + Personality change
+ Parkinson. + MND
+ Parkinson. + Down gaze palsy
+ Parkinson. + Alien limb
+ Parkinson. + Ataxia
+ MND + Gynecomastia
+ MND alone
+ Chorea + Personality change
PD dementia & advanced AD
Lewy body dementia
Progressive supranuclear palsy
Cortico-basal degeneration
MSA & fragile-x tremors ataxia

23. II- Dementia plus
Age >40 year
+ Parkinson. alone
+ Parkinson. + Personality change
+ Parkinson. + MND
+ Parkinson. + Down gaze palsy
+ Parkinson. + Alien limb
+ Parkinson. + Ataxia
+ MND + Gynecomastia
+ MND alone
+ Chorea + Personality change
PD dementia & advanced AD
Lewy body dementia
Progressive supranuclear palsy
Cortico-basal degeneration
MSA & fragile-x tremors ataxia
FTDP-17 & Dementia of Guam
Kennedy disease

24. II- Dementia plus
Age >40 year
+ Parkinson. alone
+ Parkinson. + Personality change
+ Parkinson. + MND
+ Parkinson. + Down gaze palsy
+ Parkinson. + Alien limb
+ Parkinson. + Ataxia
+ MND + Gynecomastia
+ MND alone
+ Chorea + Personality change
PD dementia & advanced AD
Lewy body dementia
Progressive supranuclear palsy
Cortico-basal degeneration
MSA & fragile-x tremors ataxia
FTDP-17 & Dementia of Guam
Kennedy disease
FTLD with MND
Huntington Disease

25. II- Dementia plus
Age >40 year
+ Parkinson. alone
+ Parkinson. + Personality change
+ Parkinson. + MND
+ Parkinson. + Down gaze palsy
+ Parkinson. + Alien limb
+ Parkinson. + Ataxia
+ MND + Gynecomastia
+ MND alone
+ Chorea + Personality change
PD dementia & advanced AD
Lewy body dementia
Progressive supranuclear palsy
Cortico-basal degeneration
MSA & fragile-x tremors ataxia
FTDP-17 & Dementia of Guam
Kennedy disease
FTLD with MND
Huntington Disease

26. II- Dementia plus
Age 20-40 year

27. II- Dementia plus
Age 20-40 year
+ Parkin. + Dystonia
+ Chorea + Dystonia
+ Parkin. + Dyston. + Ataxia + MND
+ Dystonia alone
+ Dystonia + MND
+ Chorea + Dystonia + PN
+ Chorea + Dystonia + Ataxia
Wilson’s, Juv HD, Hallervorden

28. II- Dementia plus
Age 20-40 year
+ Parkin. + Dystonia
+ Chorea + Dystonia
+ Parkin. + Dyston. + Ataxia + MND
+ Dystonia alone
+ Dystonia + MND
+ Chorea + Dystonia + PN
+ Chorea + Dystonia + Ataxia
Wilson’s, Juv HD, Hallervorden
Machado-Joseph (SCA3)
Neuro-acantho-cytosis
DRPLA

29. II- Dementia plus
Age 20-40 year
+ Parkin. + Dystonia
+ Chorea + Dystonia
+ Parkin. + Dyston. + Ataxia + MND
+ Dystonia alone
+ Dystonia + MND
+ Chorea + Dystonia + PN
+ Chorea + Dystonia + Ataxia
Wilson’s, Juv HD, Hallervorden
Machado-Joseph (SCA3)
Neuro-ferritinopathy
Neuro-acantho-cytosis
DRPLA

30. II- Dementia plus
Age 20-40 year
+ Parkin. + Dystonia
+ Chorea + Dystonia
+ Parkin. + Dyston. + Ataxia + MND
+ Dystonia alone
+ Dystonia + MND
+ Chorea + Dystonia + PN
+ Chorea + Dystonia + Ataxia
Wilson’s, Juv HD, Hallervorden
Machado-Joseph (SCA3)
Adult NCL, Adult Peliz. Merzb.
Tay-Sachs
Neuro-ferritinopathy
Neuro-acantho-cytosis
DRPLA

31. II- Dementia plus
Age 20-40 year
+ Parkin. + Dystonia
+ Chorea + Dystonia
+ Parkin. + Dyston. + Ataxia + MND
+ Dystonia alone
+ Dystonia + MND
+ Chorea + Dystonia + PN
+ Chorea + Dystonia + Ataxia
Wilson’s, Juv HD, Hallervorden
Machado-Joseph (SCA3)
Adult NCL, Adult Peliz. Merzb.
Tay-Sachs
Neuro-ferritinopathy
Neuro-acantho-cytosis
DRPLA

32. II- Dementia plus
Age <20 year

33. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
Sialidosis
+ Organs + ↕ Gaze + Ataxia
+ Optic A. + Skin pigmentation

34. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
NCL
Sialidosis
+ Organs + ↕ Gaze + Ataxia
+ Optic A. + Skin pigmentation

35. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
NCL
MERF
Sialidosis
+ Organs + ↕ Gaze + Ataxia
+ Optic A. + Skin pigmentation

36. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
NCL
MERF
Sialidosis
Lafora body
+ Organs + ↕ Gaze + Ataxia
+ Optic A. + Skin pigmentation

37. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
NCL
MERF
Metachromatic LD
Sialidosis
Lafora body
+ Organs + ↕ Gaze + Ataxia
+ Optic A. + Skin pigmentation

38. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
NCL
MERF
Metachromatic LD
Sialidosis
Lafora body
+ Organs + ↕ Gaze + Ataxia
+ Optic A. + Skin pigmentation Adreno-LD

39. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
NCL
MERF
Krabbe
Metachromatic LD
Sialidosis
Lafora body
+ Organs + ↕ Gaze + Ataxia
+ Optic A. + Skin pigmentation Adreno-LD

40. II- Dementia plus
Age ˂ 20 year
+ PME + Cherry red spot
+ PME + Retinitis pigmentosa
+ PME + Myopathy
+ Organs + ↔ Gaze + Myoclonus
+ Optic A. alone
+ Optic A. + PN
+ PME + Lafora b. on axillary s. b.
NCL
MERF
Krabbe
Metachromatic LD
Sialidosis
Lafora body
+ Organs + ↕ Gaze + Ataxia
Gaucher III
Neimann Pick type C
+ Optic A. + Skin pigmentation Adreno-LD

41. Thank you