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Neurophysiology
Making The Connections
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Embryology of the Brain
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In the first trimester…• Notochord visible by three weeks• Brain fully formed by 8 weeks• Brain is active early with movements,
especially reflexes• Swallowing is an intrauterine reflex• Brain is active in formation of amniotic fluid
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Amniotic Fluid• 80% is a filtrate of mom’s plasma
– Fetus SUBTRACTS by swallowing the fluid,– Fetus must absorb and digest the fluid
• 20% is added by the fetus– Fetus then urinates the additional fluid into the
sac
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Polyhydramnios• Neuromuscular disease
– Autonomic dysfunction– Muscle disease
• GI obstruction
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Oligohydramnios• Renal agenesis • Urinary outlet obstruction
• Potter’s syndrome
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Spinal Cord• Develops from the notochord• Goes down as far as L-1 or L-2• Ends as the Conus Medullaris
• Nerves come off to the sides as the cauda equina
• Filum terminalis: anchors the tip of the conus medullaris to base of the spinal canal
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Vertebral Arches• Fuse ventral to dorsal• Fusion begins at the cervical level and
proceeds bidirectionally• If child born prematurely, a hole can be
still present at either end
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Upper vertebral arch defects
• Anencephaly• Encephalocele• Encephalomeningocele• encephalomeningomyelocele
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Lower vertebral arch defects• Spina Bifida Occulta• Spina Bifida Aperta• Meningocele• Meningomyelocele
– Arnold Chiari Malformation– Syringomyelia
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Now you need some CSF
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CSF• A filtrate of plasma• Made by the Choroid Plexus in each
ventricle• Requires vitamin A• Requires carbonic anhydrase
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How CSF differs from plasma• Less HCO3• More CL• Lower pH 7.34• Up to 25 WBCs normal in first month of life
only; after that, only up to 3 WBCs normal
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CSF Flow• Lateral ventricles > foramen of Munro > 3rd
ventricle > aqueduct of Sylvius > 4th
ventricle > foramina of Lushka and Magendie > subarachnoid layer > spinal canal > dural sinuses > back into plasma
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Vomiting Centers• Chemotactic Trigger Zone: located on the
floor of the 4th ventricle– Responds to any increase in ICP– Uses dopamine
• Area Postrema: located on the blood side of blood:brain barrier– Responds to offensive smells or particles– Uses dopamine
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Hydrocephalus • Noncommunicating: due to an obstruction• Communicating: overproduction of CSF
• Applies pressure on the brain
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Communicating Hydrocephalus
• Newborns: mainly in premature newborns– Intraventricular hemorrhage
• Children: due to inflammation– meningitis
• Adults: overingestion of vitamin A– Pseudotumor Cerebri
• Elderly: due to brain atrophy– Normal Pressure Hydrocephalus
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Normal Pressure Hydrocephalus ( NPH)
• ventricles expands as the brain atrophies• Enlarged ventricles then compress the
long midline fibers that go to the bladder and legs
• Dementia• Incontinence• ataxia
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To treat NPH…
PLACE A VP SHUNT
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Noncommunicating Hydrocephalus
• Due to some form of obstruction• In newborns
– Aqueductal stenosis– Dandy-Walker cyst
• In children: meningitis, especially TB• In adults: cancer• In elderly: cancer
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The role of CSF• To add cushion for the brain• Shock absorption
• Head Injury– Coup lesions– Contracoup lesions
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Embryology of the brain
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How to organize Neurophysiology
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Visual Cortex• Remember that everything is REVERSED• Temporal fibers see the nasal visual field• Nasal fibers see the temporal visual field• Light must hit the retina by 3 months of
age or the child is blind for life• You must verify that a child has a RED
reflex on eye exam at birth
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Abnormalities of the Eyes• Anisocoria: unequal pupil size• Amblyopia: difference in visual acuity• Strabismus: misalignment of the eyes• Stigmatism: corneal defect• Myopia: nearsightedness• Hyperopia: farsightedness• Presbyopia: loss of accomodation seen
with aging
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Visual field deficits
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White Reflex• Cataracts: opacification of the lens
– Does not allow light to hit the retina– Must be removed– Increased incidence with high glucose or
galactose ( sorbitol or galactitol accumulates)
– Idiopathic: 90%– Diabetes or galactosemia– Rubella
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White Reflex• Retinoblastoma (rare)
– Rb gene– Cancer – High association with Ewing’s sarcoma
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Monocular blindness• Newborns: cataracts or retinoblastoma• Children: optic nerve gliomas
– Neurofibromatosis– MEN III
• Adults: embolic phenomena– TIA– Acute retinal artery occlusion– Acute retinal vein occlusion
• Elderly: macular degeneration
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Optic Chiasm Lesions• Loss of nasal fibers bilaterally• Bitemporal hemianopsia
• Pituitary tumors: 90%– Pituitary sits just beneath the chiasm
• Pineal tumors– Pineal gland sits just lateral to the chiasm
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Visual field deficits
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Optic Tract Lesions• Lesion of IPSILATERAL temporal fibers
and CONTRALATERAL nasal fibers• Homonymous Hemianopsia
• Mcc: cancers and tumors
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Visual field deficits
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Quadranopsia• Only way to get such a lesion is back in
the calcerine fissure• Pie in the sky deficit• Make sure you reverse BOTH words
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What unique information does each cortex contain?
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Frontal Lobe ( Precentral Gyri)
• CST ( motor fibers) originates from here• Unique information:
– Personality– Abstract reasoning
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Frontal Lobe Lesions• Atonic seizures• Dimentias
– Alzhiemer’s– Pick’s disease
• Schizophrenia: loss of asymmetry• Frontal lobotomies
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Temporal Lobe• Hearing• Balance • Hallucinations ( controlled by serotonin)
• Posterior temporal lobe: Wernicke’s area
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Temporal Lobe Lesions• Temporal lobe seizures• Schizophrenia• Dementias • Drugs
– SSRI– Amphetamines
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Amphetamines• Taken up presynaptically; cause release of
catecholamines• Clue: vertical nystagmus
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Amphetamines• Used in ADD
– Methylphenidate– Pemoline– Adderal– dexadrine
• OTC for weight loss– dexatrim
• Cause hallucinations– LSD– PCP– ECSTACY
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SSRI’s• Fluoxetine• Paroxetine• Luvoxetine• Sertraline• Nefazadone• Trazadone
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Parietal Lobes• Dominant lobe: long term memory; all the
things you learned since kindergarten– left side is dominant in 90% of right-handed
and left-handed people• Nondominant lobe: apraxia and
hemineglect– Right side is nondominant in 90% of right-
handed and left-handed people
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THALAMI• Epithalamus• Thalamus• Hypothalamus• Subthalamic Nucleus
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Epithalamus• The ONLY nucleus with NO known
function
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Thalamus• ALL SENSORY information in and out of
the brain MUST stop here• ALL information about the ARMS stay
LATERAL• ALL information about the LEGS stay
MEDIAL
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Thalamic Infarct• ALL sensory information from the body is
lost, but motor information is intact
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Hypothalamus• Controls hunger
– Hunger center: lateral– Satiety center: medial
• Controls menstrual cycle• Controls temperature
– Anterior: cools– Posterior: warms
• Controls stress response
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Stress Response• Parasympathetic discharge always first• Sympathetic discharge always second
• Stress ulcers• Curling’s ulcers• Cushing’s ulcers• IBS
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Acetomenophen• Works at the level of the hypothalamus• First, it cools the body ( stimulates anterior
hypothalamus) then it resists fever (blocks posterior hypothalamus)
• Oxidizes the liver (toxicity)– Treat with n-acetylcystiene ( reducing agent);
the four hour level is the most important factor
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Subthalamic Nucleus• Final relay station for coordinating fine
motor movements• Lesion: Ballismus and Hemiballismus
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Internal Capsule
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Substantia Nigra• Responsible for INITIATING movements• Uses DOPAMINE for neurotransmitter• Receives inhibitory signals from basal
ganglia via ACH or GABA
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Parkinson’s Disease• Loss of DOPAMINE fibers from substantia nigra
to striatum (caudate and putamen)• Unable to initiate activities
• Mask like facies• Bradykinesia• Shuffling gait• Fenestrating gait• Pill rolling tremor• Autonomic dysfunction: Shy Dragger syndrome
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Parkinson’s Disease, cont• Treatment: L-dopa/ carbidopa
– Bromocryptine– Amantadine– selegyline
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Movement disorder in middle-aged people
• Huntington’s disease– 90%– Autosomal dominant– Trinucleotide repeats– Caudate nucleus
involved– Anticipation– Decreased GABA
fibers– Treat with DA blockers
• Wilson’s disease– < 10%– Autosomal recessive– Ceruloplasmin def– Copper excess– Lenticular nucleus
involved– Kayser-Fleischer rings– Liver involvement– Treat with
penicillamine
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Internal Capsule• ALL MOTOR fibers going in and out of the
brain goes through here• Blood supply comes from the
lenticulostriate arteries ( smallest arteries in the brain)
• Lacunar hemorrhages: due to HTN– Causes significant MOTOR deficits
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Reticular Activating System (RAS)
• Maintain FOCUS on one item at a time• Requires NE and Serotonin• C-AMP second messenger• Has a refractory period first thing in the
morning
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Attention Deficit Disorder• ADD or ADHD• RAS not working• Poor attention and focus• Restlessness• Unable to sit long enough to complete a
task• Tx: methylphenidate; pemoline; dexadrine;
adderal
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Internal Capsule
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Midbrain
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Corticospinal Tract• Responsible for fine motor activity• Has to inhibit extension so that smooth
flexion can occur
• Spasticity• Babinski• Hyperreflexia• Clonus
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Corticospinal Tract, cont• Fibers originate from the frontal lobes, the
precentral gyri• Fibers descend through the internal
capsule and CROSS at the medullary pyramids
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CST Pathology• Atonic seizures: depolarization goes
across the frontal cortex• B-12 deficiency• ALS
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Midbrain
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Increased Intracranial Pressure
• First sign: papilledema• First symptom: headache
• Second sign: esotropia (CN VI paralysis)• Second symptom: diplopia or blurred
vision
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Increased Intracranial Pressure
• First sign of herniation: (CN III paralysis) loss of pupillary reflex; anisocoria
• Herniation is down to level just above the red nucleus
• CST and Corticorubral pathways are both compressed
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Midbrain
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If Herniation Continues…• Second sign of herniation:
DECORTICATE posturing• Compression has ocurred to below CN III
but above the red nucleus• Red nucleus still makes the upper
extremities flex while the legs extend• UNTIL…
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The Final Push• Herniation goes beyond the red nucleus• CST and Corticorubral and rubrospinal
tracts are all lost• All extremities will extend by default• Medulla is pushed through the foramen
magnum.
• DECEREBRATE posturing
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Midbrain
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Dorsal Columns• Vibratory sensation• Two-point discrimination• Position sense• Conscious proprioception• The only sensory pathway with four
synapses
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Dorsal Columns, cont• Fasciculus: made up of a few fibers• Tractus: more fibers than a fasciculus
• Gracilis: carries leg fibers; located MEDIALLY
• Cuneatus: carries arm fibers; located laterally
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Dorsal Columns, cont• FIRST SYNAPSE: dorsal root ganglion• Forms fasciculus gracilis, then tractus
gracilis ( lower extremities)• Forms fasciculus cuneatus, then tractus
cuneatus ( upper extremities)• SECOND SYNAPSE: nucleus gracilis and
nucleus cuneatus in MEDULLA
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Dorsal Columns, cont• THIRD SYNAPSE: THALAMUS• FOURTH SYNAPSE: parietal lobes
( postcentral gyri)
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Dorsal Column Pathology• Syphilis• Vitamin B-12 Def• Brown-Sequard
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Spinothalamic Tract• Pain and Temperature• The only pathway that CROSSES in the
spinal cord• Fibers enter the spinal cord, ascend two
levels, then cross to opposite side via the anterior white commissure
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Spinothalamic Tract• FIRST SYNAPSE: dorsal root ganglion• SECOND SYNAPSE: thalamus• THIRD SYNAPSE: parietal lobes
( postcentral gyri)
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Spinothalamic Tract Pathology
• Syringomyelia
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Spinocerebellar Pathway• The only pathway in the spinal cord that
crosses twice ( equivalent to ipsilateral)• Responsible for depth perception• Signs of damage:
– INTENTION TREMOR– DYSMETRIA or PRONATOR DRIFT– DYSDIODOKINESIS– ROMBERG SIGN
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Spinocerebellar Pathway, cont
• This pathway does NOT reach the cortex• Unconscious proprioception• FIRST SYNAPSE: dorsal root ganglion• SECOND SYNAPSE: thalamus• THIRD SYNAPSE: cerebellum
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Spinocerebellar Pathway Pathology
• Alcohol attacks the vermis (midline) of the cerebellum while other diseases attack the hemispheres
• Fredrieck’s Ataxia• Ataxia Telangiectasia• adrenoleukodystrophy
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PONS• Responsible for responding to the
environment • Contains the PNEUMOTACTIC and
APNEUSTIC center• CNS area most sensitive to osmotic shifts
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Pons – Pathology• Locked-in Syndrome• Central Pontine Demyelinolysis
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Medulla• Controls ALL basic functions
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Make sure you know the cranial nerves !
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How Do I Figure Out Any Lesion?
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You know it’s a spinal cord lesion when…
• Pain and temperature loss is opposite to all other deficits
• Level of the lesion is two dermatomes above where pain and temperature loss begins and on the opposite side
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You know it’s a CNS lesion when…
• UMN signs on one side of the body ( upper and lower extremities)
• The lesion is on the opposite side of the brain
• Use the cranial nerves to locate the level of the lesion
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The most important organ!!!
• The Brain
•The End The End The End