Non Alzheimer’s Dementia

Kevin Overbeck, DOAssistant ProfessorUMDNJ –SOM NJISA

Non-Alzheimer’s Dementia

This medical student presentation is offered by the New Jersey Institute for

Successful Aging.

This lecture series is supported by an educational grant from the Donald W. Reynolds Foundation Aging and

Quality of Life program.

Learning Objectives

• Compare and contrast clinical features that distinguish vascular dementia, frontotemporal dementia, and Lewy body dementia

• Differentiate between Alzheimer's disease and all other dementias

Prevalence of Common Dementias

Image Source: Weiss BD. Elder Care: A Resource for Interprofessional providers. http://aging.medicine.arizona.edu Accessed: October 5, 2011.

Evaluation of Memory Loss

• HPI (establish timeline and clinical presenting features)

• Detailed MED review• CAM• GDS• MMSE/CDT• Pertinent physical exam findings• LABs (BMP, CBC, LFTs, TSH, B12, Folate)• Brain imaging (CT Scan or MRI [MRI

preferred])• Other considered studies – RPR, UDS,

EtOH level, EEG, CXR, EKG, HIV

Evaluation of Memory LossPatient with Known or

Suspected Memory Loss

CAM

Identify Underlying Cause of Delirium &

TreatGDS

No Delirium

SWEET 16/

MMSE

Treat Depression or

Discuss Options

LabBrain Imaging

Re-evaluate After

TreatmentNo

Action

NormalAbnormal

Equivocal or Inconsistent

with Depression

Consistent with

Depression

Impaired Not Impaired

Vascular Dementia

• Onset of cognitive deficits with a stroke• Abrupt onset of symptoms with stepwise

deterioration• Often neurological exam findings

consistent with a prior stroke• Infarcts on cerebral imaging

Vascular DementiaClinical Feature Value

Abrupt Onset 2

Stepwise Detioration 1

Fluctuating Course 2

Nocturnal Confusion 1

Preservation of Personality

1

Depression 1

Somatic Complaints 1

Emotional Incontinence

1

HTN 1

Hx Stroke 2

Associated Atherosclerosis

1

Focal Neurological Symptoms

2

Focal Neurological Signs

2

Hachinski Ischemic Index highlights clinical features

Scores > 7 suggest vascular dementia

Also of note: patients with vascular dementia are more likely to have impaired executive function than those with AD

Vascular Dementia

• HTN• DM• Statins• Aspirin

Frontotemporal Lobe Dementia

(FTD)• Atrophy of the frontal and temporal

lobes• Absence of AD pathology• Clinically: personality, behavior &

language problems• Impaired executive function

FTD

• Ages 35-70 years• Rare after age 75• Familial occurrence in ~40% of cases• More rapidly progressive disorder than

AD • Fatal Illness

FTD

• Pick’s disease is a subtype of FTD but was the first described

• Presence of Pick bodies (silver staining intracytoplasmic inclusion bodies)

Note: All FTD is associated with a serotonergic deficit and decreased dopaminergic activity has been described; acetylcholinesterase activity is relatively intact

FTD

• Inappropriate social behavior (conduct)

• <10% have short term memory impairment on testing @ initial presentation

• Poor insight into their condition

• Often mistaken for a psychiatric condition

Clinical Manifestations

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FTD: A CaseA 68 year old male presents to subacute rehabilitation following a fall and subdural hematoma. A diagnosis of FTD was made two years prior to the incident via neuropsychological testing.

Despite much improved mobility and function back to baseline, he was “made” long term care at a local nursing home, due to poor safety awareness (primarily concerns about wandering).

Admission MMSE 21/30 (deficits noted below)/abnormal CDT 2/4– 1/3 short term recall– 1/5 serial sevens– 0/1 pentagon– 8/10 orientation

FTD: A Case

“I want to see what I can do about getting out of here?” He would go to report that his wife is taking his money and “she’s trying to divorce me you know.”

Very often a visit from his wife (or a telephone call) was the only thing that could calm him down.

Other behaviors were collecting towels, poor hygiene.

Then… he ate my sandwich.

FTD

• The FDA has not approved any drugs to treat this condition.

• Memantine is presently being studied.

FTD vs AD

Characteristic FTD Alzheimer’s Disease

Age of Onset <70 and even < 65 Usually greater >65

Memory Deficits Late Early

Executive Function Deficits

Early Late

Behavioral Disturbance

Early Late

Brain Imaging Frontotemporal Atrophy

Diffuse Atrophy

Response to Cholinesterase Inhibitors

None Some

Family Hx ~ 40%

Weiss BD. Elder Care: A Resource for Interprofessional providers. http://aging.medicine.arizona.edu Accessed: October 5, 2011.

Lewy Body Dementia

• DLB is the 3rd most common dementia after AD

• Progressive gradual dementia• Fluctuations in cognitive function• Persistent, well-formed hallucinations• Spontaneous motor features of

parkinsonism

Lewy Body Dementia: A Case

An 81 year old former PhD psychologist is transferred to assisted living from her independent living home due to significant increased deficits in her functional status – including multiple falls, inability to self-administer medications, a reversal of the sleep-wake cycle, and profound weight loss

– Admission MMSE: 28/30 (+) deficit 3/5 serial sevens

– Admission Weight: 108 lbs

During the next four weeks there is stabilization of the sleep-wake cycle and weight gain (112 lbs)

Lewy Body Dementia: A Case

During the next year, despite several falls that responded to physical therapy she continues to need considerable more help dressing as well as orientation/cuing (i.e. meals and medication times). Observation from nursing is that she is just “lost” and even ostracized by other residents.

On two separate occasions she reported that she saw a mouse running across the ceiling. It was investigated in earnest but no evidence of mice could be found.

Lewy Body Dementia: A Case

She is transferred to a specialized dementia unit in which her behavior (now observed more closely) is vastly different than other’s with Alzheimer’s dementia.

One year later (+) cogwheel rigidity is noted.

Lewy Body Dementia

• The FDA has not approved any drugs to treat this condition.

Lewy Body Dementia vs. AD

Characteristic Lewy Body Dementia

Alzheimer’s Disease

Age of Onset >65 Usually >65

Memory Deficits Late Early

Executive Function Deficits

Early Late

Visual Disturbance 2/3’s of Patients Rare (or very late)

Brain Imaging Diffuse Atrophy Diffuse Atrophy

Response to Cholinesterase Inhibitors

Not FDA approved

Family Hx Sporadic

Parkinson’s Disease Dementia

• Estimated six-fold increased risk for becoming demented compared with people without the disorder.

• Dementia typically occurs in the last half of the PD clinical course (whereas, in DLB, it’s the presenting feature)

• Executive dysfunction is a hallmark feature

Parkinson’s Disease Dementia

• Rivastigmine (Exelon®) has been approved for use in mild-to-moderate Parkinson’s related dementia.

Normal Pressure Hydrocephalus (NPH)

• “Classic Triad” of symptoms:– Dementia– Gait Disturbance– Urinary Incontinence

• Pathologically enlarged ventricular size• Normal opening pressures on lumbar

puncture• Potentially reversible by the placement

of a ventriculoperitoneal shunt

HIV Associated Dementia

• Age is a significant risk factor in HIV-associated dementia

• Clinical Symptoms– Distractibility/Poor Concentration– Mood Changes/Apathy vs Lability– Gait Dysfunction/Clumsiness– Reduced psychomotor speed

• Absence aphasia, agnosia, & apraxia (until late disease course) helps distinguish from AD

Huntington’s Disease (HD)

• Huntington’s Disease - traced to emigrants (1630) from East Anglican village of Bures, England:

- Dominant inheritance (1 parent with gene) - CAG

- Choreoathetosis starting at 35-45 years of age- Dementia & emotional lability also

Creutzfeldt-Jakob Disease

• Rapidly progressive dementia• Pathogenesis: Prions• Typically fatal within 1-2 years after

symptom onset• Motor deficits & seizures often

occur• Not treatable• Potentially transmissible• No clear cut MRI findings

Alcoholic Dementia

• Alcoholic dementia - Cerebellar and corpus callosum atrophy- Affective prosody comprehension decline- Gait problems- Abstract reasoning decline more than

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