NON-ALZHEIMER’S DEMENTIABobby HuffPharmD Candidate 2012

ALZHEIMERS Accounts for roughly 70% of dementia

80% in patients over 90 ~45% in patients in their 70’s

Disease is progressive and irreversible

Diagnosis confirmed upon autopsy The cortex shrivels up, damaging areas involved in thinking, planning and

remembering Shrinkage in the hippocampus, an area of the cortex that plays a key role

in formation of new memories Ventricles grow larger Disease is associated with formation of plaques and tangles in the brain

tissue

http://drpinna.com/can-diet-prevent-alzheimers-dementia-21735

NON-ALZHEIMERS Many diseases, disorders and medical

conditions share some common symptoms with Alzheimer’s Managed in many different ways

Several types Vascular dementia DLB ( dementia with lewy bodies) Parkinson’s dementia Frontotemporal dementia Huntington’s disease Dementia can also be caused by infectious

diseases, tumors, head injuries or even thyroid problems

NON-ALZHEIMER’S Not only about memory

Aphasia (language) Apraxia (in ability to performed asked tasks) Personality changes Impaired executive function (problem solving) Loss of object knowledge (agnosia) Psychosis Movement disorders

VASCULAR Accounts for roughly 18 percent of dementia

Stroke interferes with blood flow to the brain Can manifest d/t

Multiple small strokes ( infarcts) Caused by blood clots or ruptured small arteries

Large stroke (post-stroke dementia)

http://www.thirdage.com/hc/c/what-is-vascular-dementia

VASCULAR VS. ALZHEIMERS Vascular dementia can appear to be

alzheimer’s because it appears with several of the same characteristics Memory problems Confusion Disorientation Trouble following directions

How its different Recalling day to day events is impaired

But for example recalling the face of people is not impaired Alzheimer’s generally affects both

VASCULAR Signs of Vascular Dementia (stroke)

Quick onset and abrupt presentation Memory loss could lead to hallucinations, w/draw

or even aggravated/agitated moods Garbled speech, loss of coordination or weakness

on one side of the body Diagnosis

MRI or CT scan will confirm or rule out the evidence of a stroke

Treating the factors which can lead to stroke can help slow the progress of dementia These being: HTN, Hyperlipidemia, Diabetes, Smoking

VASCULAR Treatment

No medications are approved for vascular dementia The use of medications such as those used for

Alzheimer’s may provide some benefit Donepezil, Aricept etc..

In 2006 Donepezil was linked to 11 deaths in a clinical trial when used in patients who had vascular dementia. There were 11 deaths in the study group compared to 0 in the control. But other studies have showed benefit with Donepezil

DEMENTIA WITH LEWY BODIES (DLB) Occurs when protein deposits in the brain

These are called Lewy Bodies – impede cognitive fx.

Discovered by Fredrick Lewy ~1900

http://missinglink.ucsf.edu/lm/ids_104_neurodegenerative/Case2/Case2Micro.htm

DLB Widely considered as the 2nd most common

form of dementia Accounting for close to 20% of cases

DLB vs Alzheimer’s Both include

Confusion Concentration Memory impairment Hallucinations

Both are progressive but in Alzheimer’s people have good and bad days yet in DLB people can change in as little as an hour and can appear very extreme

DLB REM sleep behavior disorder

Could be an early sign of DLB Movements, gesturing and speaking while asleep

DLB Patients also have

Mobility issues similar to Parkinson’s Slow, stiff, shaky movements Difficulty balancing Shuffling gait

DLB Diagnosing

A medical workup can help identify some symptoms and narrow down the disease states

Like Alzheimer’s dementia absolute diagnosis can only be confirmed with an autopsy

Treatment No approved medications Medications used in Alzheimer’s and Parkinson’s

disease may provide some benefit in patients who have DLB

PARKINSON’S DISEASE One out of five persons with Parkinson's can

develop dementia Its due to Lewy Body formation in

Brain Brain stem

Vs. Alzheimer’s Parkinson’s is usually first diagnosed and then

the dementia related problems occur Usually doesn’t involve problems with language

PARKINSON’S DEMENTIA Diagnosed

Medical history Physical exam Neurological exam

*these are used to rule out other causes

Treatment There are no approved medications Overall therapy is designed to not only manage

the Parkinson’s disease itself but it is also designed to manage the dementia related problems

FRONTOTEMPORAL DEMENTIA Associated with more rare diseases

associated with the temporal lobes of the brain Front of the temporal lobes

Examples Picks Disease

Deposits of the tau protein (pick bodies) They can aggregate in certain lobesand lead to affects in personality, memoryand even behavior http://www.google.com/imgres?q=frontote

mporal+dementia

FRONTOTEMPORAL DEMENTIA Vs. Alzheimer’s

FTD is associated with: Impaired judgement Personality changes Various changes in mood Language barriers and problems Decrease in hobbies/activities

FTD is also progressive like Alzheimer’s Yet personality and behavioral symptoms occur earlier

and disorientation occurs later It is most usually observed in the reverse order in Alzheimer’s

Also semantic memory (associated with the meanings of words and objects) as well as episodic (time) memory are affected semantic>episodic

FTD Patients with Frontotemporal dementia may

also exhibit inappropriate behavior They have little regard as to how people feel or

what they think of their actions

Diagnosed Medical exam Brain scan (may show atrophyof frontal or temporal lobes)

http://www.google.com/imgres?q=frontotemporal+dementia

FTD

Treatment No treatment Manage the patients symptoms as best as we

can in order to improve the quality of life.

http://www.riversideonline.com/health_reference/Brain-Spine-Stroke/DS00874.cfm

HUNTINGTON'S DISEASE Caused by a genetic abnormality

Huntingtin (HTT gene) Leads to the destruction of nerve cells in the

bran and lowers the levels of neurtransmitters Causes declines

Mental Emotional Behavioral

These patients are born with the defective gene Yet do not experience symptoms until middle age

A parent has a 50 % chance to pass the defective gene on to a child

GENETICS Transcription is regulated by an interplay between histone

acetyltransferases and histone deacetylases. Researchers have found that mutant huntingtin protein (Htt) inhibits histone acetylation by blocking histone acetylases or recruiting them into aggregates. The reduction in histone acetylation leads to decreased transcription.

http://www.nature.com/nature/journal/v413/n6857/fig_tab/413691a0_F1.html

HUNTINGTON'S DISEASE Vs Alzheimer’s

Both have Personality changes Mood swings Disorientation

Attention and judgment can be impaired early on when the disease presents Memory occurs later

Also commonalities with Parkinson’s Involuntary movements appear

Jerky, clumsy, irregular

http://neuroskeptic.blogspot.com/2010/10/cannabinoids-in-huntingtons-disease.html

HUNTINGTON’S DISEASE Patients may also begin fidgeting

Eventually the patient will lose the ability to walk, talk and swallow

Diagnosed HD is diagnosed by a blood test to look for the specific

gene defect

Treatment Must manage the symptoms, there is no cure or

medical treatment. Increase meds vs. quality of life Because each Huntington's patient is unique, drug

treatment must be individualized Treatment Algorithms are out there

DEMENTIA VIA INFECTIOUS DISEASE Bacterial or viral infections can enter the

brain For example HIV/AIDS this can cause a person to

have memory and concentration problems. Person’s may also see a loss of motivation and

decreased interest in things that were recently enjoyed

Creutzfeld-Jakob disease (Mad Cow) Very rare (1 in 1,000,000) Infection by transmission of a prion (infectious protein) Infect and destroy the brain’s nerve cells

Memory and behavioral changes caused by the prion progress rapidly

DIAGNOSIS OF ID Blood Tests Medical history Neurological exam EEG (electroencephalogram) Brain scan Cerebral spinal fluid analysis (spinal tap) Autopsy is a definitive diagnosis

Holes will be visible in the structure of the brain (mad cow)

http://commons.wikimedia.org/wiki/File:Variant_Creutzfeldt-Jakob_disease_(vCJD),_H&E.jpg

OTHER CAUSES OF DEMENTIA Brain tumor Head injury Thyroid disorder Metabolic disorder Endocrine disorder

SOURCES Rosenblatt A, et al. A physician's guide to the management of Huntington's disease. Huntington's Disease Society of

America. http://www.hdsa.org/images/content/1/1/11682.pdf. Accessed December 5, 2011. Gillian P. Bates Huntington's disease: Exploiting expression, Nature 413, 691-694(18 October

2001)doi:10.1038/35099656. Accessed December 10, 2011. Newman E. Michael New NIST SRM Helps Improve Diagnosis of Huntington's Disease From NIST Tech Beat: December,

2011. Accessed December 10, 2011 Spencer, Paula. Non-Alzheimer's Causes of Dementia;

http://health.msn/health-topcs/alzheimes-disease/non-alzheimers-causes-of-dementia Various Authors.http://www.mayoclinic.com/health ; Accessed December 2011 CJD (Creutzfeldt-Jakob Disease, Classic. Centers for Disease Control and Prevention. 2008-02-26. Accessed December 10,

2011. Redefining Non-Alzheimer's Dementias, http://www.mayoclinic.org/medicalprofs/non-alzheimers-dementias.html,

Accessed December 14, 2011