APPROACH TO DEMENTIA

Guide: DR.PARIDHI SHIVDE Canditate: DR.SARATH MENON .R DEPARTMENT OF NEUROLOGY, MGM MEDICAL COLLEGE ,INDORE.

DEMENTIA- OUT OF ONES MIND

DEMENTIA- the disease with acquired deterioration in cognitive/ intellectual abilities without impairment of consciousness Cognitive deficit represent a decline from previous level of functioning

DSM

IV DIAGNOSTIC CRITERIA

1. Memory impairment
2. At least one of the following:
y y y y

Aphasia Apraxia Agnosia Disturbance in executive functioning

3. Disturbance in 1 and 2 interferes with daily function 4. Does not occur exclusively during delirium

EPIDEMIOLOGY

~ 5 to 8 % at age 65 to 70 ~ 15 to 20 % at age 75 to 80 up to 40 to 50 % over age 85

Alzheimer's disease is most common dementia 50-75%

Dementia with Lewy bodies 15 to 35 %

Vascular dementia

5 20 %

ETIOLOGY
NEUROAlzheimer's Ds; Dementia with Lewy Bodies; FrontoDEGENERATIVE temporal dementia; Parkinsons Ds VASCULAR Infarction; Hemodynamic insufficiency

NEUROLOGICAL Multiple Sclerosis; Normal Pressure Hydrocephalus ENDOCRINE NUTRITIONAL INFECTIOUS METABOLIC TRAUMATIC TOXIC AGENTS Hypothyroidism Def. of Vit. B12, Thiamine, Niacin HIV; Prion Ds; Neurosyphilis; Cryptococcus Hepatic/ Renal Insufficiency; Wilsons Ds Subdural Haematoma; Dementia pugilistica Alcohol; Heavy Metals; Anticholinergic Med; CO

CORTICAL VS. SUBCORTICAL DEMENTIA

Cortical
y

Symptoms: major changes in memory, language deficits, perceptual deficits, praxis disturbances,lack of extrapyramidal features Affected brain regions: temporal cortex (medial), parietal cortex, and frontal lobe cortex Examples: Alzheimers disease, diffuse Lewy body disease, vascular dementia, frontotemporal dementias

CORTICAL VS. SUBCORTICAL DEMENTIA Subcortical

y

Symptoms: behavioral changes, impaired affect and mood, motor slowing, executive dysfunction, less severe changes in memory, extra pyramidal findings Affected brain regions: thalamus, striatum, midbrain, striatofrontal projections Examples: Parkinsons disease, progressive supranuclear palsy, normal pressure hydrocephalus, Huntingtons disease, Creutzfeldt-Jakob disease, chronic meningitis

MIXED
Both

cortical and sub-cortical area involved. Example: vascular dementia, Dementia with Lewy bodies, Corticobasal degeneration, Neurosyphilis

REVERSIBLE DEMENTIA
y y y y y y y y

D= E= M= E= N= T= I= A=

Delirium Emotions (depression)& Endocrine Disease Metabolic Disturbances Eye & Ear Impairments Nutritional Disorders Tumors, Toxicity, Trauma to Head Infectious Disorders Alcohol, Arteriosclerosis

IRREVERSIBLE DEMENTIA
y y y y y y y

Alzheimers Lewy Body Dementia Picks Disease (Frontotemperal Dementia) Parkinsons Vascular Huntingtons Disease Jacob-Cruzefeldt Disease

HOW TO DIAGNOSE A CASE OF DEMENTIA

IN NEUROLOGY OPD

Clinical history Symptoms analysis Focussed physical examination Cognitive and neuropsychiatric examination Laboratory evaluation

CLINICAL SYMPTOMS

COGNITIVE IMPAIRMENT

ANXIETY PERSONALITY CHANGES PSYCHOSIS SLEEP DISTURBANCES

FUNCTIONAL IMPAIRMENT

NEURO-PSYCHIATRIC MANIFESTATIONS BEHAVIOURAL DISTURBANCES MOOD CHANGES

FOCUSED HISTORY
Chronology of the problem- from loved ones - mode of onset abrupt vs gradual - progression - stepwise vs continous decline - duration of symptoms Medical history Family history Socio-economic history Evaluation for toxic agent exposure

PHYSICAL EXAMINATION
Neurological examination-mobility and balance assessment Focal neurological deficits Extra-pyramidal signs Vision & hearing screening Cardiac and pulmonary evaluation

COGNITIVE & NEUROPSYCHIATRIC

EXAMINATION

Folstein Mini-Mental Status Examination (MMSE)

MMSE

SCORE RANGE 24-30 Normal 18-23 Mild 10-17 Moderate < 10 Severe

INVESTIGATIONS
ASSESSMENTS RATIONALE

Labs: Complete blood count, serum electrolytes, Rule out correctable or contributory causes of renal and hepatic function, glucose, albumin and protein, vitamin B12 and folate, rapid plasma reagin dementia (syphilis), thyroid- stimulating hormone, urinalysis Imaging: Computed tomography without contrast or magnetic resonance imaging Neurological examination Rule out infarcts, mass lesions, tumors, and hydrocephalus Correlate imaging findings with clinical examination Mini-Mental State Examination: Screening test of cognitive function

Neuropsychological testing

DIFFERENTIAL DIAGNOSIS

DELIRIUM

DEPRESSION FACTITIOUS D/O

MILD COGNITIVE IMPAIRMENT (MCI) AGE-RELATED COGNITIVE DECLINE MENTAL RETARDATION

ALCOHOL ABUSE

SCHIZOPHRENIA

DIAGNOSTIC APPROACH
COGNITIVE IMPAIRMENT ?
YES

DETERIORATION FROM A PREVIOUSLY HIGHER LEVEL ?

YES

NO

MENTAL RETARDATION

CONSCIOUSNESS ALTERED?
NO MULTIPLE COGNITIVE FUNCTIONS INVOLVED ? YES

YES

DELIRIUM

NO

APHASIA AMNESTIC D/O, etc

DEMENTIA

DEMENTIA SUB TYPES

CASE 1
70 yr old female present with progressive memory loss for past 1 yr.She also complaints of difficulty in naming objects and driving car and house keeping.for the past 1 month she has difficulty in dressing ,eating and gets agitated easily and wanders around at night. MMSE 15/30 Neurological exam- normal Vision & hearing- normal

MRI FINDINGS- DIAGNOSIS?

ALZHEIMERS DISEASE (AD)

About

70% of all cases of dementia in elderly Incidence increases with age Occurs in up to 30% of persons >85 years old Characterized by:
Progressive loss of cortical neurons y Formation of amyloid plaques (beta-amyloid is major component) (betaand intraneuronal neurofibrillary tangles (hyperphosphorylated tau proteins is major constituent constituent)
y

DIAGNOSTIC CRITERIA FOR DEMENTIA OF THE ALZHEIMER TYPE (DSM-IV, APA, 1994)

A. Development of multiple cognitive deficits 1. Memory impairment 2, other cognitive impairment B. These impairments cause dysfunction in In social or occupational activities C. Course shows gradual onset and decline D. Deficits are not due to: 1. Other cns conditions 2. Substance induced conditions F. Do not occur exclusively during delirium G. Are not due to other psychiatric disorder

CLINICAL MANIFESTATION

Begin with memory impairment

language visuospatial skills

Anosognosia- unaware of difficulties Cognitive decline-driving,shopping,house-keeping Language impaired- naming,comprehension then - fluency Apraxia- seq. motor task cant perform Visuo spatial deficits Delusions ,capgras syndrome late stages End stage-rigid,mute ,incontinent & bed-ridden

AD DIAGNOSIS

exam & neuropsychological testing Brain imaging: brain atrophy due to extensive neuronal loss and hippocampal atrophy Diagnosis confirmed by histology of post-mortem brain Plaques & tangles in hippocampus & cerebral cortex.
Neurological

CASE 2
76 yr old male presented in neuro opd with c/o progressive memory loss,emotional lability,gait disturbance for past 5 months h/o of 3 episodes of cerebrovascular accidents + recent attack 7 months back h/o HTN,DM,CAD+ O/E- incresed tone in all limbs,power 3+ in RT.UL &LL. 4+ in LT side, B/L extensor plantar

VASCULAR DEMENTIA

Refers to cognitive decline caused by ischemic, hemorrhagic, or oligemic injury to the brain as a consequence of cerebrovascular or cardiovascular disease. Part of a spectrum of vascular disease causing cognitive impairment, which also includes mild cognitive impairment of vascular origin & mixed Alzheimer's disease plus cerebrovascular disease. Kraepelin first described arteriosclerotic dementia in 1896

DIAGNOSIS & CLINICAL FEATURES (NINDSAIREN)

VASCULAR DEMENTIA
Multi-infarct dementia- recurrent strokes - step wise progression - HTN,DM,CAD MRI- multiple areas of infarction Binswangers d/s Diffuse white matter disease - lacunar infarction C/F:confusion,personality changes,psychosis pyramidal signs & cerebellar signs + gait disorder,urinary incontinence,dysarthria emotional lability

CASE 3
55 YR old woman presented with 2yr history of progressive alteration in social behavior. The pt had h/o social disinhibation,abusive language,euphoria.there is complaints of excessive food intake and weight gain for past 1 yr and pt was taken to psychiatrist once. o/e- vitals stable..neurological exam WNL MMSE-18/30

MRI

FRONTOTEMPORAL DEMENTIAS
begins with marked behavioral disturbances, unlike AD Classic form Picks disease Patients frequently hot-tempered and socially disinhibited memory & visuo spatial skills spared Impaired planning,judgement and language Echolalia + Overlap with PSP,CBD, motor neuron disease Illness progresses for years, like AD Inevitable decline MRI- lobar atrophy of frontal and/or temporal About 50% of patients have family history
Often

CASE 4
82 yr old male came to opd with c/o progressive decline in memory for the past 6-8 months.He also complaints of having decreased sleep and occasional nightmares.He occasional sees his deceased wife at times. o/e- vitals stable ,rigidity of limbs+ - gait- slow stepping gait,bradykinesia+

MMSE- 21/30 WHAT IS THE DIAGNOSIS?

DIFFUSE LEWY BODY DISEASE

Patients

have clinical parkinsonism with early and prominent dementia Lewy bodies found in brain stem, limbic system, and cortex Visual hallucinations and cognitive fluctuations common, capgras syndrome & REM sleep disorder Longstanding PD without cognitive decline develop dementia Better memory but severe visuospatial deficit Patients sensitive to adverse effects of neuroleptics May be second most common cause of dementia after AD

PARKINSONS DISEASE
About

50% of patients have dementia by 85 years old executive function disproportionately

Affects

Dementia

occur in later stage, or as a result of co morbidities- AD,DLB or side effects of drug depression & anxiety -memorizing lobe dysfnct- complex tasks,planning, & mathematical skills spared late onset,akinetic-rigid,severe depression - advanced stage

Associated Frontal

Language

Predictors-

CASE 5
65 YR old male presented to neuro opd with c/o gait disturbance for past 1 yr. On history taking his son complained his father is having memory loss for past 6 months and it is progressing. The pt also c/o of urinary incontinence+ Neurolog exam- no focal deficits MMSE- 23/30

MRI-

DIAGNOSIS ?

NORMAL PRESSURE HYDROCEPHALUS

Triad 1. Dementia: typically subcortical 2. Gait instability 3. Urinary incontinence Walk with feet stuck to floor Symptoms progress over weeks to months CT shows ventricular enlargement with no eviddence of cortical atrophy

NORMAL PRESSURE HYDROCEPHALUS

Most important test therapeutic LP

1. 2.

Remove large amount of CSF Examine gait and cognitive function Patients improve within minutes to hours of removal of 30 to 40 mL of spinal fluid Trauma or subarachnoid hemorrhage

y y

Ventriculoperitoneal shunt may correct if:

Cause is derangement of CSF hydrodynamics

CASE 6
50 YR old woman was admitted with c/o progressive memory loss and gait problem ,slurred speech within one month; The pt also had behavioral problem insomnia,agitation,aggression duration of 3 weeks.the pt also c/o abnormal jerky hand movements for past 1 month o/e- limb & gait ataxia +, reflexes-exagg. - tone increased all limbs, plantar b/l extensor - no focal weakness

MMSE- 16/30

MRI-

DIAGNOSIS?

CRUETZFELDT-JAKOB SYNDROME(CJD)
Rapid progressive dementing prion disorder Focal cortical signs, rigidity Onset between 40- 75 years 90% has MYOCLONUS vs 10% in AD Progressive dementia and personality changes over weeks to months Death <1 year from first symptom EEG- diffuse slowing and periodic sharp waves or spikes MRI- basal gangla abnormalities CSF- detect specific aminoacid sequence (PrPSc)

DISORDERS OF MEMORY FUNCTION (AMNESTIC DISORDERS)

Aging

Mild loss of memory: names and dates y Most sensitive indicator of cognitive change: poor performance on delayed-recall tasks y Verbal fluency remain intact and vocabulary may increase
y

DISORDERS OF MEMORY FUNCTION

Transient
y y y y y y y

global amnesia

Dramatic memory disturbance Affects patients >50 years Usually have only one episode, lasting 6 to 12 hrs. Complete temporal and spatial disorientation Orientation for person preserved May be confused with psychogenic amnesia, fugue state, or partial complex status epilepticus May be due to vascular insufficiency to hippocampus or midline thalamic projections

DISORDERS OF MEMORY FUNCTION

Head
y

injury

Retrograde amnesia > antegrade amnesia y With time, memories usually return but rarely to recall events surrounding trauma
Korsakoffs
y

syndrome

Near-total inability to establish new memory y Patients confabulate about recent events y Immediate memory NL,attention NL y Most common cause: thiamine and other nutritional deficiencies with chronic alcoholism

PSEUDO DEMENTIA
Severe depression Memory & language intact Vegetative symptoms insomnia,anergy, - loss of appetite Abrupt onset

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