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368 Cards in this Set

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Neural Tube Defects
=Associated w/ low FOLIC ACID intake during pregnancy

**Elevated alpha-fetoprotein in amniotic fluid + maternal serum = indicates possible NTD
Spina Bifida Occulta
=Failure of bony spinal canal to close but NO structural herniation

**Usually seen at lower vertebral levels
Meningocele
=meninges herniate through spinal canal defect
Meningomyelocele
=meninges + SC herniate through spinal canal defect
BRAIN LESIONS
**I will give you the AREA of the lesion

=you say the CONSEQUENCE
Broca's Area
=Motor (i.e. nonfluent/expressive) aphasia with GOOD comprehension

**i.e. speech is difficult to initiate, nonfluent, labored
=they know what they want to say but have trouble getting it out
Wernicke's Area
=Sensory (i.e. fluent/receptive) aphasia w/ POOR comprehension

**may vary from insertion of a few incorrect words to an outpouring of jargon
How to remember Broca vs. Wernicke's?
BROca's is BROken speech.

Wernicke's is Wordy but makes no sense.
Arcuate fasciculus
**Conduction aphasia
=poor repitition w/ good comprehension
=fluent speech

**i.e. the arcuate fasciculus runs between Broca's and Wernike's
=SO, the patient can understand what is being said BUT cannot repeat it/repeats it incorrectly
Amygdala (=bilateral)
**Kluver-Bucy Syndrome:
=hyperorality
=hypersexuality
=disinhibited behavior
Frontal Lobe
**Personality changes and deficits in concentration, orientation, and judgement.

**May have reemergencce of primitive reflexes.
Right Parietal Lobe
**Spatial Neglect Syndrome
=agnosia of the contralateral side of the world --> i.e. leads to neglect of the left side of the visual field

**SO, patient may only eat food on the right side of the plate or only draw the right side of the clock.
Reticular Activating System
COMA
Mamillary Bodies (=bilateral)
Wernicke-Korsakoff Syndrome
Basal Ganglia
**May result in a tremor at rest, chorea, or athetosis (i.e. slow writhing movements of hands or feet--seen in end stages of Huntington's).
Cerebellar Hemisphere
Intention tremor, limb ataxia
Cerebellar Vermis
Truncal ataxia, dysarthria

**Ok, so think:
=Cerebellar hemispheres are locating LATERALLY and so affect LATERAL LIMBS

=Vermis is located CENTRALLY and so affects the CENTRAL body
Subthalamic Nucleus
Contralateral hemiballismus (i.e. large amplitude flinging movements usually of arms/legs)
Chorea
**Chorea = dancing (Greek)

**Sudden, jerky purposeless movements

**Characteristic of BASAL GANGLIA lesion = i.e. Huntington's Disease
Athetosis
**Slow, writhing movements, especially of fingers.

**Characteristic of basal ganglia lesions

**Athetos = not fixed
Hemiballismus
**Sudden, wild flailing of 1 arm.

**Chacteristic of CONTRALATERAL SUBTHALAMIC NUCLEUS lesion.
=loss of inhibition of thalamus through globus pallidus
Aphasia
1) Broca's
2) Wernicke's
Broca's Aphasia
=Nonfluent aphasia w/ intact comprehension

**Broca's Area = inferior frontal gyrus
Wernicke's Aphasia
=Fluent aphasia w/ impaired comprehension

**Wernicke's Area = Superior Temporal Gyrus
Involving the CEREBRAL CORTEX:
1) Alzheimer's Disease
2) Pick's Disease
Alzhemier's Disease
**MOST COMMON CAUSE of dementia in the elderly

**Associated with:

1) Senile Plaques
=extracellular
=B-amyloid core

2) Neurofibrillary Tangles
=intracellular
=abnormally phosphorylated tau protein

**Don't forget that AD may cause amyloid angiopathy --> INTRACRANIAL HEMORRHAGE!
Genetics of AD
**Familial Form (10%) associated w/ genes on chromosomes 1, 14, 19 (ApoE4 allele) and 21 (p-App gene)

COLOR IMAGE 41
What is the SECOND most common cause of dementia in the elderly?
MULTI-INFARCT DEMENTIA
Pick's Disease
**Characterized by:
1) Dementia
2) Aphasia
3) Parkinsonian symptoms

**Associated with PICK BODIES:
=intracellular, aggregated TAU protein

**Specific for FRONTAL + TEMPORAL LOBES
Diseases involving BASAL GANGLIA + BRAINSTEM:
1) Huntington's Disease
2) Parkinson's Disease
Huntington's Disease:
**Autosomal dominant inheritance.

**Characterized by:
1) Chorea
2) Dementia

**Caused by atrophy of the CAUDATE NUCLEUS
=loss of GABAergic neurons--lose ACh + GABA
Genetics of HD:
**Caused by a trinucleotide repeat--CAG--on chromosome 4

=CAG --> "Caudate loses ACh + GABA"
Parkinson's Disease
**Associated w/ Lewy bodies (=eosinophilic intracytoplasmic inclusion found in the remaining dopaminergic neurons) + depigmentation of the substantia nigra pars compacta (=and thus loss of dopaminergic neurons)

**RARE cases have been linked to exposure to MPTP--a contaminant in illicit street drugs
Symptoms of PD:
TRAP

=Tremor (at rest)
=Rigidity (cogwheel)
=Akinesia/bradykinesia
=Postural instability
Diseases involving SPINOCEREBELLAR region:
1) Olivopontocerebellar Atrophy

2) Friedreich's ataxia
Diseases involving the MOTOR NEURON:
1) Amyotrophic Lateral Sclerosis ("Lou Gehrig's dz.)

2) Werdnig-Hoffmann Disease

3) Polio
ALS
=associated w/ BOTH UMN and LMN signs
=NO SENSORY deficit
Werdnig-Hoffman Disease
=Autosomal Recessive inheritance --> associated w/ degeneration of anterior horns

**Presents are BIRTH as a "FLOPPY BABY" + tongue fasciculations

**Median age of death = 7 months
Polio
=Follows infection w/ poliovirus

**Associated w/ degeneration of the anterior horns
=LMN signs
What is Poliomyelitis?
**Caused by POLIOVIRUS
=transmitted via the fecal-oral route
=replicates in the oropharynx and small intestine before spreading through the bloodstream to the CNS
=in the CNS --> leads to destruction of cells in the ANTERIOR HORN of the SC (=lower motor neuron destruction)
Symptoms:
=Malaise
=Headache
=Fever
=Nausea + abdominal pain
=Sore throat

+ Signs of LMN lesions:
=Muscle weakness + atrophy
=Fasciculations
=Fibrillation
=Hyporeflexia
Findings:
**CSF w/ lymphocytic pleocytosis (=i.e. increased white cell count) w/ slight elevation of protein

**Virus can be recovered from the stool OR throat.
Demyelinating + Dysmyelinating Diseases
1) MS
2) PML
3) Acute Disseminated (postinfectious) Encephalomyelitis
4) Metachromatic leukodystrophy
=a lysosomal storage disease
5) Guillian-Barre Syndrome
Multiple Sclerosis: Epidemiology
**Most often affects WOMEN in their 20's and 30's
=more common in WHITES

**Increased prevalence with INCREASED DISTANCE from the equator
Histology:
**Periventricular plaques (=areas of oligodendrocyte loss + reactive gliosis) w/ an intial SPARING of axons
Presentation:
**Most patients have a relapsing-remitting course

**Patients can present intitially w/:
=Optic Neuritis --> painful blind eye
=MLF Syndrome --> internuclear ophthalmoplegia
=Hemiparesis
=Hemisensory symptoms--numbness or weakness
=Bowel/bladder incontinence

COLOR IMAGE 47
What is internuclear opthalmoplegia?
=i.e. MLF Syndrome

**Occurs when you have demyelination of the medial longitudinal fasciculus --> causes binocular diplopia = double vision w/ both eyes open!
The "CLASSIC" Triad of MS:
MS is a SIN!

1) Scanning speech
=staccato-like speech where syllables of words are separated by pauses

2) Intention tremor

3) Nystagmus

**Patients will also have elevated protein (IgG) in their CSF.
COLOR IMAGE 47
Treatment of MS:
B-interferon OR immunosuppressant therapy
Progressive Multifocal Leukoencephalopathy
**Associated w/ the JC virus

**Seen in 2-4% of AIDS patients
=i.e. reactivation of latent viral infection
Guillain-Barre Syndrome
**Inflammation + demyelination of peripheral nerves + motor fibers of ventral roots (=sensory effect less severe than motor) CAUSING:

=symmetric ascecnding muscle weakness beginning in DISTAL LOWER EXTREMITIES
Other Symptoms:
**Facial diplegia (=paralysis of the face) is present in 50% of cases.

**AUTONOMIC FUNCTION may be severely affected:
=cardiac irregularities
=HTN or hypotension

**Almost all patients survive --> majority recover completely after weeks to months
Findings:
1) Elevated CSF protein w/ normal cell count
="albuminocytologic dissociation"

2) Elevated protein --> papilledema
Treatment:
**Respiratory support CRITICAL until recovery

**Additional treatment:
=plasmapheresis
=IV immune globulins
What is thought to cause GBS?
**Associated w/ infections --> lead to an immune attack of peripheral myelin

=i.e. herpesvirus OR campylobacter jejuni infection

Also linked to inoculations + stress
Seizures:
1) Partial Seizures
2) Generalized Seizures
Partial Seizures
**ONE area of the brain

1) Simple Partial Seizure
=consciousness intact

2) Complex Partial
=Impaired consciousness

**Motor = blinks, twitches, etc.
**Sensory = altered hearing, smelling, seeing
**Autonomic
**Psychic

**Partial seizures CAN SECONDARILY GENERALIZE!
Generalized Seizures
**DIFFUSE

1) Absence seizures
=petit mal
=blank stare

2) Myoclonic
=quick, repetitive jerks

3) Tonic-Clonic
=grand mal
=alternating stiffening + movement

4) Tonic = stiffening

5) Atonic = "drop" seizures
CHILDREN:
=genetic
=infection
=trauma
=congenital
=metabolic
ADULTS:
=tumors
=trauma
=stroke
=infection
ELDERLY:
=stroke
=tumor
=trauma
=metabolic
=infection
What is Epilepsy?
**Disorder of recurrent seizures

NOTE: febrile seizures are NOT epilepsy!
Intracranial Hemorrhage:
=Epidural hematoma
=Subdural hematoma
=Subarachnoid hemorrhage
=Parenchymal hematoma
Epidural Hematoma
**Rupture of middle meningeal artery --> often secondary to fracture of the temporal bone

**Have a LUCID interval

**CT shows a "biconvex disk" not crossing suture lines

COLOR IMAGE 44
Subdural Hematoma
**Rupture of bridging veins
=i.e. venous bleeding w/ delayed onset of symptoms

**Seen in:
=Elderly
=Alcoholics
=Blunt trauma
=Shaken baby

**Predisposing factors:
=Brain atrophy, shaking, whiplash

**Crescent-shaped hemorrhage that crosses suture lines.

COLOR IMAGE 43
Subarachnoid Hemorrhage
**Rupture of an aneurysm (=usually a berry aneurysm) OR an AVM

**Patients complain of the "WORST HEADACHE OF MY LIFE"

**Bloody or xanthochromic (=yellow tinge --> indicates hemorrhage) spinal tap
Parenchymal Hematoma
**Caused by:
=HTN
=Amyloid angiopathy
=DM
=Tumor
Where do Berry Aneurysms occcur?
**At the bifurcation of the Circle of Willis
=most common site is the bifurcation of the ANTERIOR COMMUNICATING ARTERY

**Rupture (=most common complication) leads to hemorrhagic stoke/subarachnoid hemorrhage.
What are berry aneurysms associated with?
1) Adult polycystic kidney disease

2) Ehlers-Danlos Syndrome

3) Marfan's Syndrome

Other Risk Factors:
=Advanced age
=HTN
=Smoking
=Race --> higher in BLACKS

COLOR IMAGE 46
Primary Brain Tumors
**Clinical presentation is due to MASS EFFECTS:
=seizures
=dementia
=focal lesions

**Primary brain tumors RARELY undergo metastasis

**1/2 of all adult brain tumors are METASTASES!
Location of Primary Brain Tumors:
**Majority of adult primary tumors are SUPRATENTORIAL

**Majority of child primary tumors are INFRATENTORIAL
Most Common ADULT Brain Tumors (in order of MOST common --> LEAST common):
1) Glioblastoma multiforme
=grade IV astrocytoma

2) Meningioma

3) Schwannoma

4) Oligodendroglioma

5) Pituitary adenoma
Glioblastoma Multiforme (=grade IV astrocytoma)
**Most common primary brain tumor

**Prognosis grave --> less than 1-year life expectancy

**Found in CEREBRAL HEMISPHERES
=can cross the corpus callosum
="BUTTERFLY GLIOMA"

**Color image 48
Histology:
**Stain astrocytes w/ GFAP.

**"Pseudopalisading" tumor cells
=border central areas of necrosis + hemorrhage
Meningioma
**2nd most common primary brain tumor

**Most often occurs in convexities of hemispheres + parasagittal region
=arises from ARACHNOID CELLS external to the brain

**Resectable
Histology:
**Spindle cells concentrically arranged in a WHORLED pattern

**Psammoma bodies
=laminated calcifications
Schwannoma
**3rd most common

**Schwann cell origin
=often localized to the 8th nerve --> "acoustic schwannoma"

**Resectable

**NOTE:
=bilateral schwannomas found in neurofibromatosis type 2
Oligodendroglioma
**Relatively rare

**Slow growing

**Most often in FRONTAL LOBES

COLOR IMAGE 49
Histology:
**Oligodendrocytes --> "FRIED EGG" apperance:
=round nuclei w/ clear cytoplasm
=often calcified
Pituitary Adenoma
**Prolactin-secreting is the MOST COMMON form

**Usuallly located on Rathke's pouch

**Commomn sequelae:
=Bitemporal hemianopia (due to pressure on optic chiasm)
=Hyper/hypopituitarism
Childhood Primary Brain Tumors
**Most common BENIGN:
=Pilocytic (low-grade) astrocytoma

**Most common MALIGNANT
=Medulloblastoma

Others:
=Ependymoma
=Hemangioblastoma
=Craniopharyngioma
Pilocytic (=low-grade) Astrocytoma
**Diffusely infiltrating glioma
=most often found in POSTERIOR FOSSA

**Benign --> good prognosis
Histologic Key:
ROSENTHAL FIBERS
=eosinophilic corkscrew fibers
Medulloblastoma
**Highly malignant cerebellar tumor
=a form of primitive neuroectodermal tumor (PNET)

**Can compress the 4th ventricle --> causing HYDROCEPHALUS
Histology Keys:
1) Rosettes or perivascular pseudorosette pattern of cells.

**RADIOSENSITIVE
Ependymoma
**Ependymal cell tumors most commonly found in the 4th ventricle
=can cause hydrocephalus

**Poor prognosis
Histology Keys:
1) Characteristic perivascular pseudorosettes.

2) Rod-shaped blepharoplasts (=basal ciliary bodies) found near nucleus
Hemangioblastoma
**Most often CEREBELLAR

**Associated w/ von Hippel-Lindau Syndrome when found w/ RETINAL ANGIOMAS

**Can produce EPO --> leading to SECONDARY POLYCYTHEMIA
Histology
=Foamy cells + high vascularity
Craniopharyngioma
**Benign childhood tumor
=often confused w/ pituitary adenoma as it can ALSO cause bitemporal hemianopia

**MOST common childhood SUPRATENTORIAL tumor
=derived from remnants of Rathke's pouch

**Suprasellar calcifications on X-ray COMMON
Motor Neuron Signs --> Is it an UMN lesion or an LMN lesion?
Weakness? BOTH

Atrophy? LMN

Fasciculation? LMN

Reflexes?
=Increased --> UMN
=Decreased --> LMN

Tone?
=Increased --> UMN
=Decreased --> LMN

Babinski:
=Positive --> UMN
=Absent --> LMN
How to remember that:
KEY:

**In LMN, everything is LOWERED:
=less muscle mass, decreased muscle tone and reflexes, downgoing toes

**In UMN--everything is UP
=tone, DTRs, toes
What is Syringomyelia?
**Enlargement of the central canal of the spinal cord
=most common at C8 - T1

**Often presents in patients w/ Arnonld-Chiari Malformation
Results:
**Crossing fibers of spinothalamic tract are damaged:
=bilateral loss of pain and temperature sensation in upper extremities
=preseration of TOUCH sensation
What is Tabes Dorsalis?
**Degeneration of the dorsal columns and dorsal roots due to TERTIARY SYPHILIS

=results in impaired proprioception + locomotor ataxia
Associated with:
1) Charcot's Joints

2) Shooting (lightening) pain

3) Argyll Robertson Pupils
=bilateral small pupils that constrict when patient focuses on near object--i.e. "accomodation" but NOT when exposed to light--not "reactive" to light

4) Absence of DTRs
What is Brown-Sequard Syndrome?
**Hemisection of the spinal cord
Findings:
1) IPSILATERAL UMN signs (corticospinal tract) below the lesion

2) CONTRALATERAL pain + temperature loss (spinothalamic tract) below lesion

3) IPSILATERAL loss of tactile, vibration, proprioception sense (dorsal column) below lesion

4) Ipsilateral loss of all sensation AT the level of the lesion

5) LMN signs AT the level of the lesion

**IF the lesion occurs ABOVE T1 --> presents w/ Horner's Syndrome
What is Horner's Syndrome?
**Sympathectomy of the face:

1) Ptosis
=slight drooping of eyelid

2) Anhidrosis
=absence of sweating
=PLUS flushing (rubor) of the affected side of the face

3) Miosis
=pupil constriction

**"PAM is horny!"
Anatomical Basis for the Symptoms you find in Horner's Syndrome:
**There is a 3-neuron OCULOSYMPATHETIC pathway:
=projects from the HYPOTHALAMUS --> intermediolateral column of the SC --> superior cervical (sympathetic) ganglion --> then finally to the pupil, SM of the eyelids, and the sweat glands of the forehead + face

**Interruption of these pathways results in HORNERS
What causes Horner's Syndrome?
**Lesion of the spinal cord ABOVE T1

Examples:
=Pancoast's tumor
=Hemisection
=Late-stage syringomyelia
Upper Extremity Nerve Injury:
Given = NERVE

Name site of injury/deficit in motion + deficit in sensation/course
RADIAL NERVE

=Site of injury/motion deficit
**Shaft of humerus

=loss of triceps brachii (triceps reflex)

=loss of brachioradialis (brachioradialis reflex)

=loss of extensor carpi radialis longus (leads to WRIST DROP)
RADIAL NERVE

=Deficit in sensation/course
Posterior brachial cutaneous + posterior antebrachial cutaneous

**Passes through supinator
MEDIAN NERVE

=Site of injury/motion deficit
**Supracondyle of humerus
=NO loss of power in any of the arm muscles

**Loss of:
=Forearm pronation
=Wrist flexion
=Finger flexion
=Several thumb movements

**Eventually = thenar atrophy
MEDIAN NERVE

=Deficit in sensation/course
**Loss of sensation over the lateral palm + thumb + the radial 2.5 fingers

**Passes through pronator teres.
ULNAR

=Site of injury/deficit in motion
**MEDIAL EPICONDYLE

=impaired wrist flexion + adduction

=impaired adduction of thumb and the ulnar 2 fingers (=CLAW HAND!)
ULNAR

=Deficit in sensation/course
**Loss of sensation over the MEDIAL PALM + ulnar 1.5 fingers

**Passes through flexor carpi ulnaris.
AXILLARY

=Site of injury/deficit in motion
**Surgical neck of humerus OR anterior shoulder dislocation

=loss of deltoid action
MUSCULOCUTANEOUS

=Site of injury/deficit in motion
**Loss of function of coracobrachialis, biceps, and brachialis muscles (=loss of biceps reflex)
MUSCULOCUTANEOUS

=course
**Passes through coracobrachialis
What Causes Erb-Duchenne Palsy?
**Traction or tear of the upper trunk of the brachial plexus (C5 and C6 roots)

=follows blow to the shoulder OR trauma during delivery
Findings:
**"Waiter's Tip" apperance of the arm.

**Limb hangs by side (=paralysis of abductors), medially rotated (=paralysis of lateral rotators)

**Forearm is pronated (=loss of biceps)
Common Peroneal (L4 - S2)

=What is the deficit in motion?
**Loss of dorsiflexion --> FOOT DROP

**Deep peroneal nerve innervates ANTERIOR compartment; Superficial peroneal nerve innervates LATERAL compartment

**"PED = Peroneal Everts + Dorsiflexes --> IF injured, foot dropPED"**
Tibial (L4 - S3)

=What is the deficit in motion?
**Loss of PLANTAR FLEXION

=Tibial nerve innervates posterior compartment

**"TIP" = Tibial Inverts + Plantarflexes --> IF injured, can't stand on TIPtoes!**
Femoral (L2 - L4)

=What is the deficit in motion?
**Loss of knee extension/knee jerk
Obturator (L2 - L4)

=What is the deficit in motion?
**Loss of hip adduction.
What is Thoracic Outlet Syndrome?
**"Klumpke's Palsy"

=an embryologic defect --> can compress subclavian artery + inferior trunk of brachial plexus (C8, T1) resulting in thoracic outlet syndrome
Components:
1) Atrophy of the thenar + hypothenar eminences

2) Atrophy of the interosseous muscles

3) Sensory deficits on the medial side of the forearm + hand

4) Disappearance of the radial pulse upon moving the head toward the opposite side.
CN XII Lesion
=tongue deviates TOWARD the side of the lesion
CN V Lesion
=Jaw deviates TOWARD the side of the lesion
Unilateral lesion of the cerebellum
Patient tends to fall TOWARD the side of the lesion
CN X Lesion
=uvula deviates AWAY from the side of the lesion
CN XI Lesion
=weakness turning head to contralateral side of lesion

=shoulder droop on side of lesion
Facial Lesions
=UMN Lesion

=LMN Lesion

=Bell's Palsy
UMN Lesion
=Lesion of motor cortex or connection between cortex and facial nucleus

**Contralateral paralysis of LOWER face only
LMN Lesion
Ipsilateral paralysis of upper AND lower face
Bell's Palsy
=Complete destruction of the facial nucleus itself OR its branchial efferent fibers (=facial nerve proper)

**Can occur idiopathically--gradual recovery in most cases
=Peripheral IPSILATERAL facial paralysis w/ inability to close eye on the involved side
Bell's Palsy is seen as a complication in...
ALexander Bell w/ STD:

1) AIDS
2) Lyme
3) Sarcoid
4) Tumors
5) Diabetes
Cingulate Herniation Under Falx Cerebri
=can compress the anterior cerebral artery
Downward Transtentorial (Central) Herniation OR Uncal Herniation
=coma and death rersult when these herniations compress the brainstem

**Uncus = medial temporal lobe
What are the Clinical Signs of an UNCAL Herniation?
1) Ipsilateral Dilated pupil/ptosis
=stretching of CNIII

2) Contralateral Homonymous Hemianopia
=compression of ipsilateral posterior cerebral artery

3) Ipsilateral Paresis
=compression of contralateral crus cerebri (Kernohan's notch)

4) Duret Hemorrhages
=paramedian artery rupture via caudal displacement of the brainstem
What is internuclear opthalmoplegia?
**"MLF Syndrome"

=lesion in the medial longitudinal fasciculus (MLF)
=results in medial rectus palsy on attempted lateral gaze
=convergence normal
Explanation of the Pathology
**When looking left, the LEFT nucleus of CNVI fires, which contracts the left LR and stimulates the CONTRALATERAL (right) nucleus of CNIII via the right MLF to contract the RIGHT medial rectus
What is MLF Syndrome often seen i n?
MS
Anxiety
=Increased NE

=Decreased GABA

=Decreased Serotonin (5-HT)
Depression
=Decreased NE and Serotonin
Alzheimer's Demential
=DECREASED ACH
Huntington's Disease
=Decreased GABA

=Decreased ACh
Schizophrenia
=Increased DA
Parkinson's Disease
=Decreased DA
Orientation
1) Is the patient aware of him/herself as a person?

2) Does the patient known his or her own name?

**Order of Loss:
=1st --> time
=2nd --> place
=3rd --> person
Anosognosia
=unaware that one is ill
Autotopagnosia
=unable to locate one's body parts
Depersonalization
=body seems unreal or dissociated
Anterograde Amnesia
**The inability to remember things that occured after a CNS insult
=no new memory
Korsakoff's Amnesia
**Classic anterograde amnesia that is caused by THIAMINE DEFICIENCY
=bilateral destruction of the mammillary bodies

**Seen in alcoholics + is associated w/ confabulations
Retrograde amnesia
**Inability to remember things that occured BEFORE a CNS insult
Substance Dependence
**Maladaptive pattern of substance use defined as 3+ of the following signs in a year:
=tolerance
=withdrawl
=substance taken in larger amounts or over longer time than desired
=persistent desire or attempts to cut down
=significant energy spent obtaining/using/recovering from substance
Continued...
=Important social, occupational, or recreational activities reduced because of substance abuse
=continued use in spite of knowing the problems it causes
Substance Abuse
**Defined as 1+ of the following:
=recurrent use resulting in failure to fulfill major obligations at work, school or home
=recurrent use in physically hazardous situations
=recurrent substance-related legal problems
=continued use in spite of persistent problems caused by use
Alcohol INTOXICATION
=Disinhibition
=Emotional lability
=Slurred speech
=Ataxia
=Coma or blackouts
What can be used as an indicator of alcohol use?
=Serum gamma-glutamyltransferase (GGT)
Alcohol WITHDRAWL
=Tremor
=Tachycardia + HTN
=Nausea
=Seizures and delirium tremors (DTs)
=Agitation and hallucinations
Opiod INTOXICATION
=CNS depression
=n/v
=Constipation
=PUPILLARY CONSTRICTION
=Seizures (OD is life-threatening)
Opiod WITHDRAWL
=anxiety, insomnia
=anorexia
=sweating + "piloerection"
=DILATED PUPILS
=fever, nausea, stomach cramps + diarrhea (flu-like symptoms)
=yawning
Amphetamine INTOXICATION
=psychomotor agitation --> prolonged wakefulness and attention
=impaired judgement
=PUPILLARY DILATION
=HTN, tachycardia, arrhythmias
=delusions + hallucinations
Amphetamine WITHDRAWL
=Post-use "crash" including depression, lethargy, hypersomnolence, headache, stomach cramps, and hunger
Cocaine INTOXICATION
=Euphoria
=Psychomotor agitation + impaired judement
=Tachycardia and HTN
=PUPILLARY DILATION
=Hallucinations, paranoid ideations

**Sudden cardiac death!
Cocaine WITHDRAWL
=Post-use "crash" including SEVERE depression and suicidality, hypersolmnolence, fatigue, SEVERE psychological craving
PCP Intoxication
=Belligerence, impulsiveness
=Psychomotor agitation
=Vertical and horizonal nysagmus
=Tachycardia
=Ataxia
=HOMICIDALITY
PCP Withdrawl
=Recurrence of intoxication symptoms due to reabsorption in GI tract --> sudden onset of severe, random, homicidal violence
LSD
=marked anxiety or depression
=delusions, visual hallucinations, flashbacks
=PUPILLARY DILATION
Marijuana Intoxication
=Euphoria
=Anxiety and paranoid delusions + hallucinations
=Perception of slowed time
=Social withdrawl
=Increased appetitie
=Dry mouth
Barbiturate INTOXICATION
=VERY LOW safety margin --> respiratory depression
Barbiturate WITHDRAWL
=Anxiety
=Seizures
=Delirium

**Life-threatening CV collapse
Benzodiazepine INTOXICATION
=Greater saftey margin but still causes minor respiratory depression

=amnesia
=ataxia
=somnolence

**Addictive effects w/ ALCOHOL
Benzo WITHDRAWL
=rebound anxiety, seizures, tremor, insomnia
Caffeine
Restlessness, insomnia, increased diuresis, muscle twitching, cardiac arrhythmias
Caffeine WITHDRAWL
=headache, lethargy, depression, weight gain
Nicotine INTOXICATION
=Restlessness, insomnia, anxiety, arrhythmias
WITHDRAWL
=Irritability
=Headache
=Anxiety
=Weight gain
=Craving
Alcoholism
=Physiologic tolerance + dependence w/ symptoms of withdrawl when intake is interrupted

**Continued drinking despite medial and social contraindications + life disruptions
Treatment:
**Disulfiram
=conditions the patient to abstain from alcohol use

**Supportive treatment of other systemic manifestations

**AA and other peer support groups
What are Delirium Tremors?
**Life-threatening alchol withdrawl syndrorme that peaks 2-5 days after last drink
In order of appearance, what are the symptoms?
1) Autonomimc system hyperactivity
=tachycardia, tremors, anxiety

2) Psychotic Symptoms
=hallucinations, delusions

3) Confusion
Treatment?
=benzodiazepines
Complications of Alcoholism
1) Alcoholic hepatitis + Cirrhosis
2) Pancreatitis
3) Dilated cardiomyopathy
4) Peripheral neuropathy
5) Cerebellar degeneration
6) Wernicke-Korsakoff Syndrome
7) Testicular atrophy and hyperestrinism
8) Mallory-Weiss Syndrome
Alcholic Cirrhosis
**Long-term alcohol use leads to MICRONODULAR CIRRHOSIS w/ accompanying symptoms.
What are these symptoms?
=Jaundice
=Hypoalbuminemia
=Coagulation factor deficiencies
=Portal HTN
Complications:
=Peripheral edema + ascites
=Encephalopathy
=Neurologic manifestations (i.e. asterixis)
=Esophageal varices
What causes Wernicke-Korsakoff Syndrome?
=Vitamin B1 (thiamine) deficiency

**Associated w/ periventricular hemorrhage/necrosis esp. in mamillary bodies

**Common in malnourished alcoholics
Presenation:
**Presents w/ TRIAD of:
=confusion
=opthalmoplegia
=ataxia
May progress into:
=memory loss
=confabulation
=personality change (i.e. Korsakoff's psychosis--irreversible)
Treatment:
IV Vitamin B1 (=thiamine)
What is Mallory-Weiss Syndrome?
**Longitudinal lacerations at the gastroesophageal junction caused by excessive vomiting.

**Associated with PAIN in contrast to esophageal varices!
Heroin Addiction
**500,000 US addicts

**Heroin is a Schedule I drug = NOT prescribable

**Look for TRACK MARKS--i.e. needle sticks in veins
Users are at risk for:
1) Hepatitis
2) Abscesses
3) OD
4) Hemorrhoids
5) AIDS
6) RIGHT SIDED ENDOCARDITIS
What drugs can competitively INHIBIT opiods?
=Naloxone
=Naltrexone
What drug can be used for heroin detoxification OR long-term maintenance?
Methadone
=long-acting oral opiate
What is Delirium?
**Rapid decrease in attention span and level of arousal
=disorganized thinking
=hallucinations, illusions, + misperceptions
=disturbance in sleep-wake cycle
=cognitive dysfunction
What is the KEY to diagnosis?
=waxing and waning level of consiousness

**Develops RAPIDLY
Causes?
**Most COMMON psychiatric illness on medical and surgical floors
=often reversible
=check for drugs w/ ANTICHOLINERGIC EFFECTS

**i.e. often due to either SUBSTANCE USE or MEDICAL ILLNESS
What is Dementia?
**Gradual decrease in cognition
=memory deficits
=aphasia, apraxia, agnosia
=loss of abstract thought
=behavioral/personality changes
=impaired judgement
What is the KEY to Diagnosis?
**RULE OUT DELIRIUM
=patient is alert--no change in level of consiousness
=more gradual onset
=in ELDERLY patients, often depression presents like dementia

**Commonly irreversible.
What classifies a Major Depressive Episode?
**Characterized by at least 5 of the Following for 2 weeks INCLUDING either depressed mood or anhedonia:

="SIG E CAPS"
SIG E CAPS
1) Sleep Disturbance
2) Loss of Interest (=anhedonia)
3) Guilt/feelings of worthlessness
4) Loss of Energy
5) Loss of Concentration
6) Change in Appetite/Weight
7) Psychomotor retardation or agitation
8) Suicidal Ideations
9) Depressed Mood
Lifetime Prevalence of a Major Depressive Episode
**5-12% Male

**10-20% Female
What is Major Depressive DISORDER?
=reccurent --> requires 2 or more episodes w/ a symptom-free interval of 2 months
What is Dysthymia?
=milder form of depression lasting at least 2 years
Sleep Patterns of Depressed Patients
1) Decreased SLOW WAVE sleep

2) DECREASED REM latency

3) Early morning awakening
=important screening question!
What are the Risk Factors for Suicide Completion?
**"SAD PERSONS"
=Sex --> MALE
=Age --> TEEN or ELDERLY
=Depression
=Previous attempt
=Ethanol or drug use
=Loss of rational thinking
=Sickness --> medical illness OR 3+ prescription medications
=Organized plan
=No spouse (=divorced/widow/single--esp. if childless)
=Social support lacking
Electroconvulsive Therapy
**Treatment option for MDD refractory to other treatment
=painless and produces a seizure
Major Adverse Effects?
=disorientation
=anterograde/retrograde amnesia
What is a MANIC Episode?
=distinct period of abnormally and persistently elevated, expansive, or irritable mood lasting at least 1 week

**During the mood disturbance, 3+ of the following are present

="DIG FAST"
DIG FAST
=Distractibility
=Irresponsibility--> seeks pleasure w/o regard for consequences
=Grandiosity--> inflated self-esteem
=Flight of Ideas--racing thoughts
=Increased in goal-directed ACTIVITY/psychomotor AGITATION
=Decreased need for sleep
=Talkativeness or pressured speech
What is a Hypomanic Episode
**Like a manic episode EXCEPT the mood disturbance is NOT severe enough to cause marked impairment in social/occupational functioning OR to necessitate hospitalization

=there are no psychotic features
What is Bipolar Disorder
**6 Separate Criteria Exist for Bipolar Disorders w/ Combinations of:
1) Manic = Bipolar I
2) Hypomanic = Bipolar II
3) Depressed Episodes

**1 Manic OR Hypomanic episode defines bipolar disorder
What is the DOC for treating Bipolar Disorder?
Lithium
Cyclothymic Disorder
=a milder form of bipolar disorder lasting at least 2 years
Malingering Disorder
=Patient cocnsiously fakes or claims to have a disorder in order to attain SECONDARY GAIN (i.e. avoiding work, obtaining drugs)
What is a Factitious Disorder?
**Patient consiously creates symptoms in order to assume a "sick role" and to get medical attention
Munchausen's Syndrome
**Extreme form of factitious disorder
=chronic history of multiple hosptial admissions and willingness to recieve invasive procedures
Munchausen's by Proxy
=seen when illness in a child is caused by the parent

**Motivation is unconcious
=often times parent feels good about playing the "concerned parent" role

**IS CHILD ABUSE and MUST be reported!
Somatoform Disorders
**Both illness production AND motivation are UNCONSIOUS desires

**More common in women.

**Several types:
=Conversion
=Somatiform Pain Disorder
=Hypochondriasis
=Somatization Disorder
=Body Dysmorphic Disorder
=Pseudocyesis
Conversion
**Motor or sensory symptoms (i.e. paralysis, or pseudoseizure) that suggest neurologic or physical disorder BUT physical exam and tests are negative
=often follows an ACUTE STRESSOR
=patient may be unconcerned about symptoms
Somatoform Pain Disorder
=prolonged pain that is not explained completely by illness
Hypochondriasis
=Preoccupation with and fear of having a serious illness in spite of medical reassurance
Somatization Disorder
=variety of complaints in multiple organ systems with NO identifiable underlying pathology
Body Dysmorphic Disorder
=Preoccupation w/ minor or imagined physical flaws--patients often seen cosmetic surgery
Pseudocyesis
=false belief of being pregnant associated w/ objective physical signs of pregnancy
GAIN--Can be PRIMARY, SECONDARY, or TERTIARY.
**Primary:
=what the symptom does for the patient's internal psychic economy

**Secondary:
-what the symptom gets the patient (sympathy, attention)

**Tertiary
=what the caretaken gets--i.e. like an MD on an interesting case
Pain Disorder
**Reccurent periods of intense fear and discomfort peaking in 10 minutes w/ 4 of the following--PANICS:
1) Palpitations
2) Paresthesias
3) Abdominal distress
4) Nausea
5) Intense fear of dying or losing control and lIght-headedness
7) Chest pain, Chills, Choking, disConnectedness
8) Sweating, shaking, SOB
How is this panic described?
=Described in context of occurence--i.e. panic disorder w/ agoraphobia
Specific Phobia
**Fear that is excessive or unreasonable
=cued by the presence or anticipation of a specific object or situation
=exposure provokes anxiety response

**Person recognizes that the fear is excessive (=INSIGHT)
=fear intereferes w/ normal routine
Treatment?
Include systematic desensitization.
Examples:
1) Gamophobia
=fear of marriage

2) Algophobia
=fear of pain

3) Acrophobia
=fear of heights

4) Agoraphobia
=fear of open places
Post-Traumatic Stress Disorder
**Person experienced or witnessed event that involved ACUTAL or THREATENED death or serious injury
=response involves fear, helplessness, or horror
=traumatic event is persientely reexperienced as nightmares or flashbacks + person avoids stimuli associated w/ the trauma
How LONG does the disturbance last?
>1 month

=causes severe distress and social/occupational impairment

**PTSD can follow ACUTE stress disorder which lasts 2-4 weeks
Adjustment Disorder
**Emotional symptoms (anxiety, depression) causing impairment following an identifiable psychosocial stressor--> i.e. divorce or moving

**lasts < 6 months
Generalized Anxiety Disorder
**Uncontrollable anxiety for at least 6 months that is unrelated to a specific person, situation or event

**Causes sleep disturbances, fatigue, and difficulty concentrating
Autistic Disorder
**Patients have severe communication problems and problems forming relationships
=characterized by repetitive behavior, unusual abilities (=savants) + usually below-normal intelligence
Treatment?
=Increase communication and social skills
Asperger Disorder
**Milder form of autism involving problems w/ social relationships + repetitive behavior

**Children are of normal intelligence and LACK social or cognitive deficiets
Rett Disorder
**X-linked disorder seen ONLY in girls
=affected males die in utero

**Characterized by loss of development + MR appearing at age 4
=stereotyped HAND-WRINGING
ADHD
**Limited attention span and hyperactivity

**Children are emotionally labile, impulsive, and prone to accidents

**Normal intelligence
Treatment?
Methylphenidate (=Ritalin)
Conduct Disorder
**Continued behavior violating social norms

**At age >18 y.o., it is diagnosed as ANTISOCIAL PERSONALITY DISORDER
Oppositional Defiant Disorder
**Child is noncompliant in the absence of criminality
Tourette's Syndrome
**Motor and vocal tics and involuntary profantiy

**Onset <18 y.o.
Treatment?
Haloperidol
Separation Anxiety Disorder
**Fear of loss of attachment figure leadingg to factitious physical complaints to avoid going back to school

**Common onset age 7-8
Anorexia Nervosa
**Abnormal eating habits (i.e. excessive dieting)
**Body image distortion
**Increased exercise

**Seen primarily in adolscecnt girls + commonly coexists w/ depression
PE:
1) Severe weight loss
2) Amenorrhea
3) Anemia
4) Electrolyte disturbances
Bulimia Nervosa
**Binge-eating followed by self-induced vomiting or use of laxatives

**Body weight is normal
PE:
1) Parotitis
2) Enamel erosion
3) Electrolyte disturbances
4) Alkalosis
5) Dorsal hand calluses from inducing vomiting
Hallucination vs. Illusion vs. Delusion
**Hallucinations:
=perceptions in the ABSENCE of external stimuli

**Illusions
=misinterpretations of ACUTAL external stimuli

**Delusions
=false beliefs not shared w/ other members of the culture/subculture that are FIRMLY maintained despite obvious proof to the contrary
Delusions vs. Loose Association
**Delusion
=disorder in the content of thought (i.e. the actual idea)

**Loose Association:
=disorder in the FORM of the thought (i.e. the way ideas are tied together)
Visual and Auditory Hallucinations
**Common in schizophrenia
Olfactory Hallucinations
**Occur as an aura of a psychomotor epilepsy
Gustatory Hallucinations
RARE
Tactile Hallucinations
i.e. FORMICATION
=sensation of ants crawling on one's skin

**Common in DTs
**Also seen in cocaine abusers (i.e. "cocaine bugs")
HypnaGOgic Hallucinations
Occur while GOing to sleep
Hypnopompic Hallucinations
Occur while WAKING from sleep
Sleep Apnea
**Person stops breathing for at least 10 seconds repeatedly during sleep
Central Sleep Apnea
NO respiratory effort
Obstructive Sleep Apnea
Respiratory effort AGAINST airway obstruction
What is associated w/ sleep apnea?
1) Obesity
2) Loud snoring
3) Systemic/pulmonary HTN
4) Arrhythmias
5) Possibly sudden death

**Individuals may become chronically tired
Treatment:
Weight loss, CPAP, surgery
Narcolepsy
**Disordered regulation of sleep-wake cycles
=may include hypnagoic or hypopompic hallucinations

**Person's episodes start off with REM cleep

**STRONG GENETIC COMPONENT
Cataplexy
Loss of all muscle tone following a strong emotional stimulus

**occurs in some patients w/ narcolepsy
Treatment?
**Stimulants
=i.e. amphetamines
Schizophrenia
**Periods of psychosis and disturbed behavior w/ a decline in functioning lasting > 6 months
Schizophreniform Disorder
1-6 months
Brief Psychotic Disorder
<1 month

=usually stress-related
What are the 5 Subtypes of Schizophrenia?
1) Disorganized
2) Catatonic
3) Paranoid
4) Undifferentiated
5) Residual
Diagnosis requires 2 or more of the following:
1) Delusions
2) Hallucinations
=often auditory
3) Disorganized thought
=i.e. loose associations
4) Disorganized or catatonic behavior

**1-4 are POSITIVE SYMPTOMS...the patient can ALSO have NEGATIVE SYMPTOMS
What are the Negative Symptoms?
=flat affect
=social withdrwal
=lack of motivation
=lack of speech or thought
Etiology and Prevalence
**GENETIC factors outweight environmental factors in the etiology

**1.5% lifetime prevalence
=males = females
=blacks = whites

BUT presents earlier in MEN
Schizoaffective Disorder
**A combination of schizophrenia and a mood disorder.
Personality Trait
**An enduring pattern of percieving, relating to, and thinking about the environment and onself--exhibited in a range of social/personal contacts
Personality DISORDER
**Occurs when these patterns become inflexible and maladaptive--causing impairment in social or occupational functioning

**Person is generally NOT AWARE of the problem

**NOT usually diagnosed in children.
CLUSTER A Personality Disorders
="odd, eccentric"

1) Paranoid
2) Schizoid
3) Schizotypal

**Patients cannot develop meaningful social relationships
**Genetic association w/ schizophrenia
Paranoid
=distrust and suspiciousness
=PROJECTION is main defense mechanism
Schizoid
=Voluntary social withdrawl
=Limited emotional expression
Schizotypal
**Interpersonal awkwardness

**Odd beliefs or magical thinking

**Eccentric appearance
CLUSTER B Personality Disorders
="dramatic, erratic"

**Genetic association w/ MOOD DISORDERS + SUBSTANCE ABUSE

1) Antisocial
2) Borderline
3) Histrionic
4) Narcissistic
Antisocial
**Disregard for and violation of rights of others
**Criminality

**Males > Females
=Called a "conduct disorder" if the person is <18
Borderline
**Unstable mood and interpersonal relationships

**Impulsiveness

**Sense of emptiness

**FEMALES > Males
Histrionnic
**Excessive emotionality
**Attention seeking
**Sexuallly provocative
Narcissistic
**Gradiosity
**Sense of entitlement

**May react to critism w/ rage

**May demand "top" physician/best healthcare
CLUSTER C Personality Disorders
**Anxious or fearful

**Genetic association w/ ANXIETY disorders

1) Avoidant
2) OCD
3) Dependent
Avoidant
**Sensitve to rejection
**Socially inhibited and timid
**Feelings of inadequacy
Obsessive-Compulsive
Preoccupation w/ order, perfectionism, and control
Dependent
**Submissive and clinging
=excessive need to be taken care of
=low self-confidence
What is Potter's Syndrome?
**Bilateral renal agenesis --> oligohydraminos --> limb deformities, facial deformities, pulmonary hypoplasia

**Babies w/ Potter's can't PEE in utero!
Cause?
**Malformation of the ureteric bud
Horseshoe Kidney
**Inferior poles of both kidneys fuse
=as they ascend from the pelvis during fetal development, horseshoe kidneys get TRAPPED under the inferior mesenteric artery --> and THUS remain low in the abdomen
CASTS in the Urine:
**Presence of casts indicates that hematuria/pyuria is of RENAL origin
What do RBC Casts Signal?
1) Glomerular inflammation
=nephritic syndromes

2) Ischemia

3) Malignant HTN

4) BLADDER CANCER
What do WBC Casts Signal?
1) Tubulointerstitial disease

2) Acute pyelonephritis

3) Glomerular disorders

4) ACUTE CYSTITIS
What do Granular Casts Signal?
ACUTE TUBULAR NECROSIS
What do Hylanine Casts Signal?
**NONSPECIFIC
NephrItic Syndrome
**Hematuria, HTN, oliguria, azotemia

**Think "I" for INFLAMMATION
What can cause NephrItic Syndrome?
1) Acute Poststreptococcal glomerulonephritis
2) Rapidly Progressive (=Cresenteric) Glomerulonephritis
3) Goodpasture's Syndrome
4) Membranoproliferative Glomerulonephritis
5) IgA Nephropathy
6) Alport's Syndrome
Acute Poststreptococcal Glomerulonephritis
**Most frequently seen in CHILDREN

**Presents w/ peripheral and periorbital edema

**Resolves spontaneously
LM Findings?
**Glomerulil enlarged and HYPERCELLULAR

**Neutrophils

**"Lumpy-Bumpy"
EM?
**Subepithelial humps
Immunofluorescence? (IF)
GRANULAR pattern
Rapidly Progressive (Cresenteric) Glomerulonephritis
**Rapid course to renal failure from one of many causes
LM and IF
CRESENCT-MOON SHAPE
Goodpasture's Syndrome
**Type II Hypersensitivity

**Can cause HEMOPTYSIS and hematuria
IF
**Linear pattern

**Anti-GBM antibodies
Membranoproliferative Glomerulonephritis
**Slowly progresses to renal failure
EM
Subendothelial humps

="tram-track" appearance
IgA Nephropathy
**"Berger's Disease"

**Mild disease--often postinfectious
IG and EM
Mesangial deposits of IgA
Alport's Syndrome
**Collagen IV Mutation
=can cause nerve deafness and ocular disorders

**SPLIT BASEMENT MEMBRANE
NephrOtic Syndrome
**Massive proteinuria, hypoalbuminemia, peripheral + periorbital edema, hyperlipidemia

**"O" = prOteinuria
Causes:
1) Membranous Glomerulonephritis
2) Minimal Change Disease
3) Focal Segmental Glomerular Sclerosis
4) Diabetic Nephropathy
5) SLE
Membranous Glomerulonephritis
**Common cause of ADULT nephrotic syndrome
LM
**Diffuse capillary and basement membrane thickening
IF
GRANULAR pattern
EM
"spike and dome" appearance
Minimal Change Disease
**"Lipoid Nephrosis"

=most common cause of childhood nephrotic syndrome

**Responds well to STEROIDS
LM
NORMAL glomeruli
EM
FOOT PROCESS EFFACEMENT

color image 93
Focal Segmental Glomerular Sclerosis
**Causes a more severe disease in HIV patients
LM
Segmental sclerosis and hyalinosis
Diabetic Nephropathy--LM
**Kimmelstiel-Wilson "wire loop" lesions

**Basement membrane thickening

color image 95
SLE
**There are actually 5 patterns of renal involvement

**In MEMBRANOUS GLOMERULONEPHRITIS
=wire-loop lesion w/ subendothelial deposits
Kidney Stones
**Can lead to severe complications such as:
=hydronephrosis
=pyelonephritis

**4 Major Types:
=calcium
=ammonium magnesium phosphate
=uric acid
=cystine
Which are Radiopaque vs. Radiolucent?
RADIOPAQUE:
=Calcium
=Ammonium magnesium phosphate (struvite)

Radiolucent:
=Uric acid
=Cystine
Calcium
**MOST COMMON kidney stones (75-85%)

**Can be composed of calcium oxalate, calcium phosphate or both

COLOR IMAGE 97
What could cause these calcium stones?
**Conditions that cause HYPERCALCEMIA:
=cancer
=increased PTH
=increased vitamin D
=milk-alkali syndrome

**TEND TO RECUR!
Ammonium Magnesium Phosphate
**STRUVITE STONES**

**2nd most common kidney stone
=caused by INFECTION w/ UREASE-POSITIVE BUGS

**Can form STAGHORN CALCULI that can be a nidus for UTIs
What are urease-positive bugs?
=Proteus
=Staph
=Klebsiella
Uric Acid
**Strong association w/ HYPERURICEMIA (i.e. GOUT)

**Often seen as a result of diseases w/ increased cell turnover such as:
=leukemia
=myeloproliferative disorders
Cystine
**Most often secondary to cystinuria
Renal Cell Carcinoma--Epidemiology:
**Most common renal malignancy

**Most common in MEN ages 50-70

**Increased incidence w/ SMOKING and OBESITY
Genetic Origin?
**Associated w/ von Hippel-Lindau AND gene deletion in chromosome 3
Where does it originate and where does it go?
**Originates in renal tubule cells --> polygonal clear cells

**Invades IVC and spreads hematogenously
Presentation
=hematuria
=palpable mass
=secondary polycythemia
=flank pain
=fever
=weight loss
Is it associated w/ paraneoplastic syndromes?
**YES**

=ectopic EPO
=ACTH
=PTHrP

COLOR IMAGE 98
Wilm's Tumor
**Most common renal malignancy of early childhood (age 2-4)

**Caused by a deletion of tumor suppression gene WT1 on chromosome 11
Can be part of the WAGR Complex:
1) Wilm's Tumor
2) Aniridia
3) Genitourinary malformation
4) Mental-motor retardation
Presenation
**Presents w/ a huge, palpable flank mass, hemihypertrophy
Transitional Cell Carcinoma
**Most common tumor of the URINARY TRACT SYSTEM--> can occur in:
=renal calyces
=renal pelvis
=ureters
=bladder
What symptom is especially suggestive of bladder cancer?
Painless hematuria
Associated with:
**Associated w/ problems in your "Pee SAC:"

1) Phenacetin
2) Smoking
3) Aniline dyes
4) Cyclophosphamide

COLOR IMAGE 90
Pyelonephritis
Can be ACUTE or CHRONIC
Acute Pyelonephritis
**Affects cortex with relative SPARING of the glomeruli/vessels

**White cells casts in urine are pathognomonic

**Presents w/ fever, CVA tenderness

COLOR IMAGE 89A
Chronic Pyelonephritis
**Coarse, asymmetric corticomedullary scarring

**Tubules can contain eosinophilic casts = "thyroidization of the kidney"

COLOR IMAGE 89B
What is diffuse cortical necrosis?
**Acute generalized infarction of cortices of both kidneys

**Likely due to a combination of vasospasm AND DIC
What is it associated with?
1) Obstetric catastrophes
=i.e. abruptio placentae

2) Septic Shock
Acute Tubular Necrosis
**MOST common cause of acute renal failure
=reversible BUT fatal if left untreated
=death most often occurs during the initial oliguric phase

**Recovery in 2-3 weeks
What is it associated with?
1) Renal ischemia
=i.e. shock

2) Crush injury
=myoglobulinuria

3) Toxins
What is Renal Papillary Necrosis associated with?
1) Diabetes Mellitus
2) Acute pyelonephritis
3) Chronic phenacetin use
Acute Renal Failure
**Abrupt decline in renal function with INCREASED creatinine and INCREASED BUN over a period of several days

1) Prerenal azotemia
2) Intrinsic renal
3) Postrenal
Prerenal Azotemia
**Decreased RBF (i.e. hypotension) --> DECREASED GFR

=Na and water retained by the kidney
Intrinsic Renal
**Generally due to acute tubular necrosis OR ischemia/toxins

**Patchy necrosis leads to debris obstructing the tubule and fluid backflow across necrotic tubule --> DECREASED GFR

**Urine has epithelial/granular casts
Postrenal
**Outflow obstruction
=stones
=BPH
=neoplasia

**Develops only with bilateral obstruction
Consequences of Renal Failure
**Failure to make urine and excrete nitrogenous wastes

**2 Forms of Renal Failure:
1) Acute Renal Failure
=often due to hypoxia
2) Chronic Renal Failure
=due to HTN and diabetes
Uremia
**The actual clinical syndrome marked by INCREASED BUN and CREATININE and associated symptoms.
SO, consequences of renal failure:
1) Anemia
=failure of erythropoietin production
2) Renal osteodystrophy
=failure of active vitamin D production
3) Hyperkalemia
=can lead to cardiac arrythmias
4) Metabolic acidosis
=due to decreased acid secretion and decreased generation of HCO3-
Continued...
5) Uremic encephalopathy
6) Sodium and H20 excess --> CHF and pulmonary edema
7) Chronic pyelonephritis
8) HTN
Low Serum Sodium
=disorientation
=stupor
=coma
High Serum Sodium
**Neurologic
=irritability
=delirium
=coma
Low serum Chlorine
Secondary to metabolic alkalosis
High serum chlorine
Secondary to non-anion gap acidosis
Low serum Potassium
**U waves on ECG

**Flattened T-waves

**Arrhythmias

**Paralysis
HIGH serum potassium
**Peaked T-waves

**Arrhythmias
Low serum Calcium
**Tetany

**Neuromuscular irritability
High serum calcium
**Delirium

**Renal stones

**Abdominal pain
Low serum magnesium
**Neuromuscular irritability

**Arrhythmias
High serum magnesium
**Delirium

**Decreased DTR's

**Cardiopulmonary arrest
Low serum phosphate
**Low-mineral ion product = causes bone loss
High serum phosphate
**High-mineral product = causes metastatic calcification

**Renal stones