Neurodegenerative Diseases

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Pathology of the Ad Brain

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narrowed gyro and widened sulci

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Region of the temporal lobe site where most neural lost

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hippocampus

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biochemical pathology of the AD brain

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depletion of acetylcholine

loss of cholinergic neuron

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Drug Treatments for AD

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acetylcholinesterase inhibitors: cognex, aricept, exelon, reminyl

glutamate NMDA receptor antagonist: memantine

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neurofibrillary tangles

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paired helical filaments made from tau

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neuritic plaques

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made up of beta-amyloid protein

may be good or bad

found in neuronal, glial, endothelial

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ALS pathophysilogy

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degeneration of upper motor neurons(cerebral cortex) and lower motor neurons (anterior horn cells of the spinal cord or lower brain stem)

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amyotrophic

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muscle atrophy

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lateral sclerosis

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loss of myelinated fiber bundles and scarring in the lateral white columns of the spinal corde

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clinical manifestations of ALS:

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sensory and cognitive functions remain intact

muscular weakness and muscle atrophy

progressive bulbar palsy: speech, chewing and swallowing

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glutamate excitotoxicity ALS

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glutamate transporter re-uptakes glutamate into axon terminal

glutamate transporter defective in ALS

Riluzole (Rilutek): drug which block release of glutamate and prolongs life of patient

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neurofilament gene defect ALS

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found in 1% of all ALS cases

produces abnormal cytoskeleton

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superoxide dismutase gene defect (SOD) ALS

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SOD is a free radical scavenger

free radicals destroy membranes

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antibodies to calcium channels (ALS)

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calcium influx is unregulated

increased intracellular calcium becomes toxic to neurons

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Most recently identified gene mutations associated with ALS

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TDP-43

C9orf72

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Multiple Sclerosis (MS)

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progressive demyelinating disorder of the central nervous system(CNS) by an immune response involving inflammation. Scaring which leave plaque like deposits the CNS and deterioration of axons

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visual manifestations of MS

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unilateral blindness 2-3 weeks

eyes turned to left, right eye lags

eyes turned to right, left eye lags

convergence unimpaired

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brainstem or cerebellar manifestations of MS

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wide-based gait; teeters back and forth

knee-jerk

finger to nose test fail

intention tremor

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spinal cord manifestations of MS

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spastic gait

Lhermitte's sign: sudden sensation of electric shock down spine and along arms when patient flexes neck

neurogenic bladder

loss of position sense

paraplegia, wheelchair

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sensitization of MS

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when a CD4+ class II MHC antigen presenting cell presents an antigen to a T lymphocyte

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activation of MS

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the proliferation of sensitized T cells

alpha 4 beta 1

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inflammation of MS

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mediated by helper T cells and macrophages

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demyelination

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mediated by tumor necrosis factor-alpha, nitric acid, antibodies from B cells and complement

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general mechanism of action of Tysabri for MS

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TYSABRI prevents white blood cells from crossing the blood-brain barrier and attacking the CNS

TYSABRI may work by interrupting the activity of inflammatory cells through inhibition of alpha4-integrin–mediated adhesion

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general mechanism of action of interferon beta (Betaseron) from MS

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relapsing forms of MS interleukin 1

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general mechanism of action for Ampyra for MS

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potassium channel blocker and stops leakage and improves conduction of impulse

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Gullain-Barre syndrom

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auto immune response against peripheral nervous system

occurs after a viral illness or immunization

is reversible

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Gullain-Barre syndrom clinical phases

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1. tingling of hands and feet

2.hard to arise from chair

3. areflexia, weakness, sensory loss

4. respiratory monitoring

5. ventilation

6. full recovery

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Huntington's Disease (HD)

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Autosomal dominant with mutated huntington gene located on chromosome 4

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Pathology of HD

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degeneration of the GABA neurons in the caudate nucleus and putamen of the basal ganglia

35 yrs old- after child bearing age

produces chorea(dance like movements) hyperkinetic movement disorder

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Huntington's disease gene

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codes for the protein huntingtin

37-100 extra glutamine residues (CAG)

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the drug used to treat HD to help quench the unwanted motor activity by blocking dopamine receptors

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Haloperidol

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Apoptosis of HD

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the poly-glutamate fragments stimulates this in the caudate nucleus neurons

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Parkinsons Disease (PD)

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loss of dopaminergic neurons located in the substantia nigra that form the nigrostriatal pathway

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Basal ganglia of PD

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group of forebrain nuclei comprised of the caudate nucleus, putamen, and globus pallidus

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striatal efferent nerve fibers

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axons originating in the striatum that project out to the globes pallidus and substantial nigra.

synthesize the neurotransmitter GABA, converge to thalamus which project upon the motor areas of cerebral cortex

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normal function of the basal ganglia

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function to regulate the output of the motor areas of the cerebral cortex

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Cardinal signs of parkinsons disease

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1. resting remor

2. muscular rigidity

3. bradykinesia/akinesia

4. postural instability

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Hoes-Yahr stages of Parkinsons disease

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I. Unilateral tremor, rigidity, bradykinesia or postural abnormailities

II. bilateral temor, rigidit, bradykinesia, or postural tremor

III. first signs of deteriorating balance, but still fully independent

IV. requires help with some or all activities of daily living, unable to live alone

V. confined to wheelchair or bed unless assisted.

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Causes of parkinsons disease

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idiopathic: paralysis; shaking palsy. this is most common form of PD from an older person

Drug induced: medications, tranquilizers, anti-psychotics, anti-hypertensive agents toxins: MPTP

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parkinsons disease histochemical changes

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1. loss of melanin in substantial nigra pars compacta

2. loss of dopamine cell bodies in substantia nigra

3. depletion of dopamine content in striatum

4. lewy body formation in nigral neurons

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normal nigrostriatal pathway

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dopamine to basal ganglia. the nigra has lost most of its pigment

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progressive loss of dopamine in the striatum of PD

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80% loss of dopamine content in the striatum appears to be a critical threshold before symptoms appear

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Treatment for Parkinsons Disease

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dopamine replacement: Sinemet

dopamine receptor agonist: Parlodel, Permax, Requip, Mirapex

Ablation Therapy: older method and being replaced

Deep brain stimulation: probes implanted in the basal ganglia can instantly dimish the symptoms of PD

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Sinemet for PD

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combination of levodopa and carbidopa

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Levodopa for PD

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precursor to dopamine

CAN be transported across blood brain barrier

converted to dopamine in dopaminergic axon nerve terminals in brain tissue

conversion to dopamine in peripheral circulation can be prevented by action of carbidopa

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Carbidopa for PD

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inhibits decarboxylase enzymes circulating in blood but not in brain

does not cross blood brain barrier

given with levodopa to prevent conversion to dopamine in blood

prevents high levels of circulating dopamine and decreases vomiting

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dopamine for PD

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active neurotransmitter synthesized by nigrostriatal neurons

it CAN NOT cross the blood brain barrier

vomiting is stimulated when dopamine is given orally in high doses.