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44 Cards in this Set

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What are the most common causes of dementia in the elderly?
1) Alzheimers
2) Multi-infarct Dementia
others: folate deficiency, thiamine deficiency, neoplasia, Wilsons disease
What pathology is associated with Alzheimers disease?
Extracellular senile plaques with B-amyloid core. (swollen eos. nerve cell processes cortex, hippocampus, amygdala).

Intracellular Neurofibrillary tangles (abnormally phosph tau protein)

granulovacuolar degeneration (pyramidal cells hippocampus)

Hirano bodies (intracytoplasmic dendritic eosinophilic actin inclusions)

Amyloid Angiopathy

Reduced neurons in NUCLEUS BASALIS OF MEYNERT

Deficiency CAT (choline acetyl transferase) and decreased Ach in cortex & hippocampus (why tacrine and achesterase drugs used to tx)
What is the genetics of Alzheimers disease?
10% familial form assoc w:
Chrom 1,14,19 (APOE4 allele)
& Chromosome 21(p-App gene)

APP gene product = path. AB and normal P3 (mutation = more of path. product)

Chromosome 19 - APOE4 protein, 14 and 1 code for presenilens linked to family early onset alzheimers
Pick's disease: pathology and where localizes?
Pick bodies (intracytoplasmic inclusion bodies)
FRONTAL and TEMPORAL lobes
Huntington's disease Pathology
autosomal dominant - Chrom 4
CAG repeats, anticipation
atrophy of caudate
Frontal/temporal cortex (neuronal depletion gliosis)
(loss Ach/Gaba-ergic)

athetoid mvmnts, personality/emotional changes leads to hypertonicity, fecal/urinary incontinence, anorexia/wt loss, dementia, death
Parkinson's disease
Lewy bodies
depigmented Substantia Nigra
TRAP (tremor, rigidity, akinesia, postural instability)
masked facies, micrographia, shuffling gait
Causes of Parkinson's?
Idiopathic
MPTP/Dopa inhibiting drugs
Shy-Drager
Von Economo encephalitis
Dementia pugilistica (trauma)
Friedrich's Ataxia
Autosomal recessive
cerebellar ataxia
dysarthria, nystagmus
ass: scoliosis, pes cavus, heart disease, diabetes
Olivopontocerebellar ATROPHY
cerebellar ataxia plus dysarthria plus PARKINSONIAN symptoms (neuronal loss Substantia Nigra and cerebellum)
poly GLN triplet repeats
Amyotrophic Lateral Sclerosis (ALS)
LMN & UMN signs - Lou Gehrig's disease

LMN signs: atrophy muscles from denervation, fasciculations

UMN: hyperreflexia, spasticity, Positive Babinski

Usually first involves muscles hand, arm shoulder
Rapid progression w/ death in 1-6 years
Werdnig Hoffmann disease
birth- floppy baby
tongue fasciculations
Polio
LMN signs
Epidural Hematoma
Middle meningeal artery rupture, 2ndary to temporal bone fracture
Biconvex disc, does not cross suture lines
unconscious, lucid interval, then signs increasing ICP
Subdural Hematoma
Rupture Bridging veins
delayed, insidious symptoms
Elderly, Blunt Trauma, Alcoholics
Crescent shaped, crosses suture lines
Subarachnoid Hemorrhage
Rupture of (berry) aneurysm or AVM
"Worst headache of life"
Bloody/xanthochromic spinal tap
Parenchymal Hematoma
HTN (charcot-bouchard aneurysms), amyloid angiopathy, diabetes, tumor
Berry/Saccular aneurysms
Aneurysm at Bifurcation of
a) anterior comm carteries (most common site)
RUPTURE--> hem. stroke/subarachnoid hemorrhage
What are Berry aneurysms associated with?
Adult Polycystic Kidney Disease
Ehler's Dahnlos
Marfan's
Demyelinating Diseases:
Multiple Sclerosis - Pathology
Periventricular plaques
preservation of axons
loss of oligodendroglia
reactive astrocytic gliosis
Protein (IgG) in CSF (oligoclonal bands on analysis)
MS - Presentation
SIN (scanning speech, intention tremor, nystagmus)
Optic Neuritis
MLF-Internuclear Ophthalmoplegia
Hemiparesis
Hemisensory symptoms
Bladder/bowel incontinence
Who gets MS and treatment
Women in 20s or 30s
Greater prevalence further from equator
TX - prednisone, IFN B, natalizub (tysabri) - Ab to integrin adhesion molecules
Demyelinating Diseases:
Progressive Multifocal Leukoencephalopathy
PML - JC virus
2-4 percent AIDS patients- reactivation of latent virus
Demyelinating Disease
Metachromic Leukodystrophy
Sphingolipidosis
Deficiency Arylsulfatase A, acculumulation cerebroside sulfate

Central and peripheral demyelination with ataxia, dementia
Guillan Barre Pathology
Inflammation and demyelination of peripheral nerves and motor fibers of ventral roots (more motor than sensory)

Assoc w:
Infections (herpes, C.jejuni)
Inoculations
Stress
Guillan Barre Symptoms/presentation
Symmetric ascending muscle weakness beginning distal lower extremeties
50% facial diplegia
Autonomic affected - cardiac irreg, HTN, hypotension

ALBUMINO-CYTOLOGIC DISSOCIATION (elevated CSF protein with normal cell cnt)

elevated protein-->papilledema
Poliomyelitis - virus pathology
Fecal-oral transmission
replicates oropharynx & S.Int
Through blood to CSF

Destroys anterior horn cells
->LMN destruction

Virus can be recovered from stool or throat
Poliovirus symptoms
Malaise, headache, fever
nausea, abd pain, sore throat
LMN signs -
muscle wknss/atrophy
fasciculations
fibrillation
hyporeflexia
Polio Findings
CSF w/ lymphocytic pleocytosis
Slight elevation protein
Simple Partial Seizure
Awareness intact
motor/sensory/autonomic/
psychic

Partial = affects one area of brain, can secondarily generalize
Complex Partial
Impaired awareness
Type of seizure? Action?

Absence

Myoclonic
both are generalized

absence=petit mal=blank stare
Abrupt Onset, 3-4 HZ spike&wave EEG
common in kids, 80% grow out of

myoclonic=quick, repet jerks
Tonic-Clonic

Tonic

Atonic
Tonic-Clonic=GrandMal= alternating stiffness & mvmnt

Tonic=Stiffening (NOT BREATHING DURING THIS PHASE)

Atonic=drop seizure

All are Generalized
Causes of seizures in following groups:

Children
Genetic
Infections/Febrile
Trauma
Congenital
Birth Trauma
Metabolic
Causes of seizures in ADULTS
Tumors
Trauma
Stroke
Infection
Causes seizures in Elderly
Stroke
Trauma
Tumor
Metabolic
Infection
Broca's Aphasia
expressive
nonfluent aphasia w/ intact comprehension

INFERIOR FRONTAL GYRUS
Wernicke's Aphasia
Receptive
Fluent with impaired comprehension

SUPERIOR TEMPORAL GYRUS
Horner's Syndrome
Sympathectomy of face (lesion above T1)

Ptosis
Anhidrosis (absence sweating and flushing-rubor one side face)
Miosis

PAM is Horny (horners)

Hypothalamus projects to interomediolateral column, which then projects to sup cervical ganglion and then to sweat glands/eye/etc.
Syringomyelia
Enlargement of central canal of spinal cord

BILATERAL loss Pain and Temp (crossing fibers ant. comm lost) in upper extremities w/ Touch preserved
What does syringomyelia often present with?

What level is it common?
Arnold Chiari malformation

C8-T1
Tabes Dorsalis
Degeneration dorsal columns&dorsal roots due to tertiary SYPHILIS (dorsal roots/ganglia degen first followed by wallerian degen of dorsal columns)
What symptoms are associated with Tabes Dorsalis?
impaired proprioception

locomotor ataxia

Charcot's joints (loss position sense = stumbling)

shooting/lightning pain

Argyll-Robertson pupils

Absence DTR
What other forms of neurologic disease can Syphilis demonstrate?
Meningovascular (perivascular infiltrates, necrotic granulomas on meninges)

General Paralysis of Insane- shrunken, firm cortex w/ thick leptomeninges
What forms of neuro disease can TB produce?
Pott's Disease - epidural TB abscess leads to vertebral collapse (TB spondylitis)
can cause chord compression

Intraparenchymal Tuberculoma

Tuberculous Meningitis (basilar fibrosing)