LECTURE 5

1. Given a patient with memory loss, recognize when the most likley diagnosis is Mild Cognitive Impairment (MCI), Alzheimer's Disease (AD), Vascular Dementia (VD), Frontotemporal lobe dysfunction , Lewy Body disease, Pesuedodementia, Creutzfeldt Jacob disease (CJD).
MCI
-Isolated memory impairment with other domains spared fo 1 or more years
-15% of patients progress to probably AD each year with 80% progressing in 6 years

AD
See objective 4

VD
-history of clinical strokes with a sudden onset with stepwise progression and lateralized findings

Frontotemporal lobe dysfunction (FTD, Pick's DZ primary progressive aphasia, semantic dementia)
-Presents at a younger age usually less than 65

Lewy Body Dementia
-difficult to separate from AD
-Early visual hallucinations
-parkinsonism, fluctuating cognition, REM behavioral disorder

Pseudodementia
-Depression is prominant
-no sundowning
-neuropsych testing deficits not severe
-many elderly patients with pseudodementia develop AD

CJD
-Rapid progression of dementia(fatal in 1 yr)
-myoclonus
-abnormal EEG
-visuoperceptive derangements
-CSF positive for 14-3-3


2. Given a patient with dementia, recognize when a patient has a potentially reversible cause.
Metabolic Disorders (can be tested for by lab screens)
B12 deficiency
Thyroid disease
Folate deficieny
Infectious Causes (culture etc.)
Syphilis
Crytococcal meningitis

3. Given the clinical presentation of a patient with dementia, recognize aphasia, apraxia, agnosia, and disturbances in executive function.
Aphasia
Broad term encompassing language deficits. Includes Anomia(difficulty naming things), word finding, comprehension deficiencies (wernicke and transcortical sensory aphasia), and expression deficiencies( brochas and transcortical motor aphasia)
Apraxia
The loss of ability to perform a learned motor task despite intact basic sensory and motor function.
Types
Ideomotor: cannot pantomime gestures to command
Ideational: cannot sequence acts of a multi-step complex action
Conceptual: loss of semantic knowledge involving tool use
Others include Dressing/construction/gait
Agnosia
Difficulty recognizing objects not just names. Prosopagnosia is a difficulty recognizing faces.
Executive function
Problems with planing, organizing, and goal oriented behavior.
Manifestations
a) trouble with check book balancing or paying bills
b) cannot prepare a large meal with several dishes
c) cannot keep house clean
d) cannot keep up with job
e) cannot complete tasks
f) perseveration (Persistent repetition of an activity, word, phrase, or movement, such as tapping, wiping, and picking.)

4. Given the clinical presentation of a patient with Alzheimer's Disease, make the appropriate diagnosis by using the NINCDS-ADRDA and DSM-IV criteria
NINCDS-ADRDA
Probable AD
-Dementia confirmed by MSE and Neurphycologic Testing.
-Deficits in at least 2 cognitive domains.
-Progressive cognitive decline.
-Normal consciousness(No delerium).
-No other explanation for symptoms.
Possible AD (When something from the list below prevents patient from meeting probable AD)
-Dementia with atypical onset/course without another explanation.
-Presence of another disease process capable of causing dementia but not the likely cause of -dementia.
-Progressive deterioration of 1 cognitive domain.
Definite AD
Probable AD with Autopsy evidence.

DSM IV
Multiple cognitive deficits including
1) memory impairment
2) and one or more of the following
-Aphasia
-Apraxia
-Agnosia
-Executive Functions

Signs Pointing Towards AD
-Deficits cause significant impairment in social and occupational settings. There is a gradual onset with continuing decline. Deficits are not attributable to other condition or substance.

Signs Pointing Against AD
-Rapid onset. Focal sensorimotor signs. Seizures or gait issues early in the disease process.

Clinical Presentation of AD
-Memory loss is the 1st thing to go (Forming new memories is impaired while recall of old memories is still intact)
-Anomia is the initial language deficit, then comes comprehension, then comes empty speech.
-Problems with perception first include easily becoming lost in new surroundings, and progressively worsen to becoming lost/confused in own home.
-Apraxia and Executive function issues also are commonly seen.
-A majority of patients with AD often have behavioral symptoms including Apathy, Irritability, and Delusions. Delusions include phantom border, paranoia, and occasionally capgras syndrome.

5. Given the clinical presentation of a patient with a movement disorder, recognize parkinson's disease and Huntington's chorea.
Parkinson's DZ
Disorder of the Basal Ganglia with a loss of dopaminergic cells in the substantia nigra.
4 Cardinal Features
1)Resting Tremor
-4-7hz in limbs, jaw, face, tongue
-postural tremor

2) Rigidity
-Resistance to passive movement. May be seen in cogwheel fashion

3) Bradykinesia/Hypokinesia
a) masked facies. decreased eye blinking
b) lack of fidgeting at rest
c) purposeful activity is slow
d) drooling (b/c bad swallowing)
e) cant roll over in bed
f) slowed and low amplitude alternating movements

4)Posture
- decrease in stride length
- shuffling gait
- festination
- tendency to fall especially backwards

Other findings:
-eczema of the forehead
-constipation is often the very first sign preceeding everything else
-responsive to L-DOPA
-Tendency to act out dreams
-Depression

Huntington's Chorea
-Genetic defect with CAG repeats on Chromosome 4.
-Caudate and putamen are atrophic
Clinical Features (each of these are possible manifestations):
-Onset in the 4th/5th decade of life. 1/2 of the patients present with movement disorder(choreoathetosis, and much less commonly parkinsonism) and 1/2 of the patients present with psychiatric disorder
1) Choreoathetosis
-Chorea (jerky coarse movements)
-Athetosis(slow writhing movements)
-Gait(lurching, Dance-like)
2) Dementia(Frontal subcortical)
-executive impairment
-decreased concentration and attention
-memory problems due to poor retreival (unlike AD which is a storage problem)
-poor speech
3)Psychiatric
-Personality changes
-Depression/Bipolar
-Anxiety
-Psychosis
-Suicidal
4) Dysarthria/Dysphagia
5) cannot maintain portruded tongue

WESTPHAL VARIANT has parkinsonism and not choreathetosis and is juvenile onset.

6. List the differential diagnosis for Parkinson's disease and Huntington's chorea.

Parkinson's Disease Differential

Parkinsonisum Plus Syndromes
-Don't respond to L-DOPA
1)Progressive Supranuclear Palsy
-early falls
-progerssive paralysis of voluntary eye movements. development of downgaze paresis
-oculovestibular responses stay intact
-Hummingbird sign in MRI
2)Multiple System Atrophy
a. Striatonigral Degeneration-Pyramidal Dysfunction
b. Olivopontocerebellar atrophy-Cerebellar Dysfunction
c. Shy Drager syndrome-Autonomic Dysfunction (including sever orthostatic hypotension)
3)Corticobasal degeneration
-Asymmetric motor findings
-Terrible Limb Apraxia
-Alien limb (autonomous behavior of a hand or arm)

Drug induced
-Neuroleptics (common)
-Metoclopromide (common)
-alpha-methyldopa
-reserpine
-Amiodarone

Toxic
-Cyanide
-Carbon Monoxide(bilateral pallidal necrosis on imaging)
-Manganese intoxication (seen commonly with liver cirrohsis, seen in welders, bright signal in basal ganglia on T1 MRI)

Wilson's Dz
-Copper metabolism screwed up
-Kayser-Fleischer Rings
-serum ceruloplasmin down urinary copper up
-liver disease

Focal Lesions
-vascular,neoplastic, and infectious lesions that can be detected on imaging)

Huntington's DZ Differential

Sydenham's Chorea
1) Post streptococcal
2) seen in conjunction with rheumatic fecer
3) ASO positive
4)self-limited

Chorea Gravidarum (Birth control Chorea)
-Caused by estrogen, with prior sydenham's chorea as a risk factor

Hyperthyroidism

Tardive Dyskinesia
-Prior neuroleptic use
-This is a real question b/c many have prior psych dz and have taken neuroleptics, which can be confused with Huntingtons
-Tardive Dyskinesia patients can hold their tongue in protrusion whil Huntingtons patients cannot

Wilson's Dz
-See parkinsons differential

Senile Chorea
-onset >60y/o
-No neuropsychiatric symptoms

Drugs
-Birth control
-L-dopa and other Dopamine agonists
-Stimulants
-Phenytoin
-Carbamazepine
-Ethosuximide