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Consists of neurons whose cell bodies are in the motor cortex (precentral sulcus).
This system of neurons runs its fibers through the white matter of the brain, around the
ventricles, and the fibers cross in the medulla.
On the motor cortex, these fibers are separated into what’s called the Motor Homunculus
Motor Homunculus
Normal pressure Hydrocephalus affect the face area of internal capsule which is close to
the ventricles
o Internal capsule received blood from branches of Middle cerebral artery
In the internal capsule region, the face arm and leg fibers are very close together, and if
someone has weakness equally in all of these areas, you can pinpoint that to the internal
capsule (lacunar infarction), or to the brainstem. Face, arm & leg are equally involved
Pons lesions will affect the cerebellum Ataxia in addition to weakness of face, arm & leg
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The left hemisphere is involved mostly with language
The right hemisphere is involved with spatial relationships.
Cortical phenomenon:
o Weakness in the face & arm > leg
o Aphasia
Upper motor neuron: Starts from the cerebral motor cortex cross at the end of the medulla
Anterior horn cells. They synapse with the cells of the anterior horn.
o Upper neuron lesion will produce weakness of the limb & hyperreflexia (deep tendon
reflexes) + Babinski sign.
Lower motor neuron goes from anterior horn cells out to the neuromuscular junction.
A lower neuron lesion will produce weakness of the limb & lack of reflexes. (eg could be
due to herniated disc in the back, or entrapment).
Weakness also maybe due to Neuro-Muscular junction problems or due atrophy of muscles
itself.
Sensory Pathways
Posterior column - involved with vibration & proprioception - cross in the medulla (MLF)
Lateral Spinothalamic tract - pain & temperature (pinprick) – cross on the same level of
spinal cord (within 2-3 levels)
These two systems project to the thalamus Internal Capsule postcentral gyrus
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Brown-Sequard - Hemisection of the spinal cord ------------------------------------------------------ *** TQ
Cranial Nerves
Cavernous sinus - III, IV, V1, VI and sympathetic innervation to carotid ---------------------------- *** TQ
o Tumors (Cranio-Pharyngeoma) of the cavernous sinus affect these nerves.
o Talusa Hunt Syndrome (Inflammation of the cavernous sinus) also affect these nerves
Cerebellum - (important in coordination) right hemisphere controls right side, and vice versa, due to
double crossover of fibers.
Visual System
Right visual field projects to the left part of the retina
In general, the left occipital lobe and the left retina are involved with seeing things on the right
side of the face and vice versa.
A stroke affecting the left occipital lobe will cause loss of vision in the right side of the face.
Pituitary tumor around the optic chiasm will cause a bitemporal hemianopsia
Retinal problems - will have islets of visual problems - not broken up as neatly as neural
problems.
Macular vision is the central area of vision -------------------------------------------------- *** TQ
o It is the most important part of vision and it projects to the macular region at the tip of the
occipital lobe
Generalized Hypoperfusion Syndrome
o Due to dropping of the BP
o area that’s furthest away from the circulation, ie (area between Vertebral a. & PCA
will be affected first “Watershed infarction” (eg - Lt. or Rt. occipital area)
Macular visual loss & Transcortical Aphasia)
o OR area between ACA & MCA will be affected first “Watershed infarction”
Proximal arm/shoulder motor loss: Think of motor homunculus - Face and arm are
affected by middle cerebral artery, leg involved with anterior cerebral artery & the
watershed area in the cortex supplies the shoulder area (causes “man in the barrel”
syndrome)
Occlusion of L posterior cerebral a. - occipital tip is spared - i.e. macular vission sparing
Visual Exam (Funds)
o Macula - paling of disc seen with demyelinating disorders
o Macular vision loss (Macular Scottoma) can occur in migraine
o Swelling seen with tumors (papilledema)
o Visual loss can be with swelling of the disc or not.
o If there is disc edema & patient > 60 y.o. Ischemia of the optic nerve (Painless)
o If there is disc edema & patient in 20’s y.o. Demylination of the optic nerve (MS)
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o If there is No disc edema Central retinal Artery occlusion (CRAO): 25 % due to Carotid
emboli, 25 % due to Lupus (or other arteritis such as temporal)
Temporal Artritis (Painfull) infarction of optic nerve loss of vision
o If bilateral disc edema without visual loss Papil edema (Painless)
Sympathetic innervation of eye - causes dilation - three neurons are involved:
I neuron - posterior hypothalamus upper spinal cord (C8-T2 level)
2 neuron - upper spinal cord (C8-T2 level) - across apex of lung to internal carotid a.
3 neuron - Superior cervical ganglion
pupillary motor fibers
o Horner’s syndrome - pupil is abnormally constricted with ptosis
Use Cocaine then hydroxyamphetamine/cocaine to test pupil: cause release of
Epinephrine (normally would make it dilate)
Introduce drug into pupil to determine which of the three neurons has been
affected.
If lesion is 1st or 2nd order - dilation will occur with medications
If lesion is 3rd order - no dilation will occur with medications
This test is to defirenciate whether the lesion from brain stem or Apex lung TB or
neck lesion 1,2 D - 3 ND ----------------------------------------------------------- *** TQ
Pupils should be equally reactive to light and accommodation
Accommodation: Light in one eye cause constriction of the other eye’s pupil
Afferent pupillary defect visual acuity defect because it involves the optic nerve directly
o Normally the pupils dilate right away and then rebound a little bit.
o If there is a problem with communication between retina and PSNS and SNS, then this is
called an afferent pupillary defect.
o Marcus Gunn pupil Afferent pupillary defect (don’t get constriction of pupils if you
shine the light) - caused by Demyelination of optic nerve
Medial Longitudinal fasciculus
o Connects head & neck motion to eye movements (ie connects vestibular nucleus
(parapontine reticular formation - PPRF to III & VI nuclei)
o Necessary to track objects with eyes movements (Congrugate together - Movements of
one eye is in senchrony with the other eye)
o If there is dis-Congrugate movements then it is a lesion at the brain stem level ***TQ
o To test this clinically by Ice water caloric test:
Test the unresponsive patient: Squirt ice water into ear eyes will gradually move
over to that side, followed by a rapid corrective nystagmus to opposite side.
This tests the vestibular connections between the III and VI nerve.
• Identifies lesions of MLF (if only one eye moves & the other eye stays in
midline you know there is a lesion). (very potent stimulus - can agitate
brain dead patients) mnemonic: cold opposite warm same (“COWS”)
o Also can be tested by Doll’s eyes maneuver (move the head to one side then the other
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STROKE
Definition
Stroke = Acute disruption of blood flow to the brain leading to focal neurologic deficits
TIA (transient ischemic attack) = Acute disruption of blood flow to the brain leading to focal
neurologic deficits lasting less than 24 hours (usually 3 – 10 minutes)
Usually the emboli from the heart or internal carotid artery (must do surgery if stenosis of
internal carotid artery > 70%)
TIA of left internal carotid artery left eye vision loss (due to occlusion of left Ophthalmic
Artery) + right face weakness + slert of speech, then weakness of right side of the body (due to
occlusion of left Middle cerebral artey)
Rx: Heparin then surgery if occlusion > 70%
Rx of TIA of Vertebrobasilar system: No surgery, Cumudine
Crushendo TIA: multiple TIA’s that will lead to Stroke if not treated (Emergency admission to
hospital)
Dissection of Internal Carotid Artery (due to trauma) clot goes to inside the artery
Horner Syndrome ------------------------------------------------------------------------------- ***TQ
o Rx: Heparin or Cumodine to prevent clotting
Epidemiology
700,000 stroke per year (Ischemic Strokes - 80%: 1/2 - small vessels & 1/2 - large vessels)
Leading cause of disability in USA
Incidence increases with age
African Americans > Hispanics > Whites
Stroke subtypes
Ischemic (large & small vessels clots)
Hemorrhagic (Aneurysm rupture or Arterio-Venous malformation ruptures)
Superior Sagital Sinus thrombosis (Rare)
o Usually happens in people who have hyper-coagulative states (post partum and Multiple myloma)
o Blood will not be able to drain into the sagital sinus infarction of the top of the brain
bilateral hemispheric infarctions
o Rx: Anti coagulants
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Stroke Syndromes
Hemorrhagic Stroke
Due to Aneurysm rupture or Arterio-Venous malformation ruptures
o Aneurysm location mostly occur in the anterior circulation: anterior communicating artery 30%,
anterior, middle cerebral artery 30% & posterior cerebral artery 30% (least in basilar artery <
15%)
Most patients with Aneurysm ruptures dies or if survive will have permanent neurological
defects
Rx if survive:
• Platelets
• Keep the person perfused (Hydration)
• Prevent spasms of surrounding blood vessels (motopine)
The most important to remember (Worst headache in my life: Emergency) --------*** TQ
• CT scan
• Lumber puncture
• MRA
o Arterio-Venous malformation
May present with bleedings or seizures
May be gradual developments of neurological deficits
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Risk factors of hemorrhagic stroke
Hypertension
Cigarette smoking
Alcohol use
⇓ Physical activity
High cholesterol (Hyperlipidemia)
Atrial fibrillation
Transient ischemic attacks
Carotid artery dissection - with trauma get blood in lumen and wall of artery can prduce the emboli. Put
them on thinners
Bruit in Carotid Artery indicates stenosis. If its asymptomatic don’t do anything for it. (some
physicians will perform surgery even if asymptomatic when stenosis > 70%: I prefer surgery)
Subclavian Steal syndrome - Narrowing of artery proximal to origin of vertebnral artery. May have TIA
of the vertebral artery when exercising (The arm steal blood from vertebral artery).
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EPILEPSY
Seizures
Electrical discharge in CNS (due to irritation of cortex).
Different areas of brain produce different symptoms
Types of Seizures
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Complex Partial (focal) seizure
Affect the prefrontal area (Temporal lobe > Frontal lobe)
o Lowest seizure threshold high seizure incidence is found in hippocampus (short term
memory) because want it to fire easily. So any lesion or inflammation in temporal or
frontal lobes around hippocampus can cause seizures.
Staring is seen but will perform complex motor activities during the seizure (ex. groom selves)
Stereotypic behavior—repeated behavior ex. Can wipe of pants walk to the window and walk
back.
Patients are never violent during these seizures
Aura - occurs in complex seizures and migraine and is a sensation (smell) of something to
come. This will occur wherever the lesion is.
There is post ictal confusion - patient doesn’t remember anything after
Todd’s paralysis - transient, occurs as weakness after focal discharge in brain. Ex. arm after
the motor movement is patient with seizures will be weak after. Have to rule out infarction.
Rx
o Carbamazepine (Tegretol), or Dilatin (Phenytoin) ------------------------------------- *** TQ
o Oxcarbamazepine (Trileptal): a new form of carbamazepine, or Gabapentin (Neurontin)
o Lobectomy if refractory
Status Eplapticus
Life threatening (Emergency) due to failure to recover from SZ before another one begins.
If SZ continues > 1 hour and if not stopped the brain will fry (Hypoxia brain damage).
Cause: Non-compliant patient with med or due to Metabolic derangements, infection, trauma
or misdiagnosis of the seizure type.
Rx:
o ABC: Check airway and check heart if working blood gases O2 intubation
o Blood for Glucose, Electrolytes, CBC (Infection), BUN & ECG monitoring
o Thiamine - 100 mg. I.V. then 1 amp. (40 ml) 50% Dextrose and then Saline
Remember: Always give Thiamine before Dextrose b/c a patient with alcohol will
develop Wernicke Korsakoff Encephalopathy (Thiamine Deficiency) ----------- ***TQ
Also, don’t give Nitrous Oxide anesthesia for Vit B12 deficiency patients severe
subacute combined degeneration (Post. Coll. & CS tract) quadraplegia ------ ***TQ
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o Drugs
Benzodiazepines (Ativan - 0.1 mg/kg I.V. < 2mg/min) to disrupt seizure Then
give Dilantin (cant give more than 50mg.min or get QT elongation and give full
dose, can precipitate in dextrose solution so give saline) or Phosfenatoine (quicker
acting Dilantin)
If seizures don’t stop give Phenobarbitol (will have to intubate since it will cause
stop breathing when given after Benzodiazepines).
Pediatric Seizures
Neonatal SZ
1st day of life - due to hypoxia or hypoglycemia
5-7 days - due to Hypo-Ca or narcotic withdrawal
Later - due to infections or Metabolic derangement
Rx:
o fix the cause
o Pyrodoxine & Phenobarbitol
Prognosis is poor if sustained seizures or abnormal neurological symptoms are present
Febrile SZ
Occur between 6 months & 5 y
Duration < 15 minutes
Febrile SZ will reoccur
93% will never lead to epilepsy (7% may lead to epilepsy 25 y)
Occur when a child has a sudden increase in temperature
Will have a diagnosable infection
If you never saw this patient before have to also think that it could be meningitis (H. Flu)
o Meningitis can produce a SZ so might need a lumbar puncture.
o In meningitis every minute is important to prevent long-term damage.
o If there is a family Hx of febrile SZ than might not have to do lumbar puncture.
Rx: if > 3 febrile SZ Phenobarbitol &/or Valium (suppository)
Atypical Febrile SZ
Duration < 15 minutes
Focal, not generalized
Not with temperature swings
Occur with clusters
Abnormal Neuro Exam
Need full evaluation
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TRAUMA
Rx:
Monitor intracranial pressure (ICP)
o If ⇑ ICP use Hyperventilation, Manitol (osmotic diuretics)
Surgery if Hematoma &/or open Fractures
Subdural Hematoma
Surface or Bridging vessels torn from cerebrum
Altered mental status
Focal signs
Headache
Epidural Hematoma
Tear in Middle Meningial Artery
Brief post traumatic loss of consciousness
Lucid interval
Ipsilateral papillary dilation
Contralateral hemiparesis
Perinatal Trauma
Types: Premature or Term
Intraventricular Hemorrhage in Term infants can occur if there is difficulties during birth
Premature Intraventricular - Periventricular hemorrhage
o If birth weight less 1500 gm there is a 40% chance that there will be bleeding into &/or
around their cerebral ventricles ------------------------------------------------------------ *** TQ
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o SS: floppy with no muscle tones, agitated,
⇓ HR, ⇓ BP, ⇓ Hct, ⇓ To, ⇓ Respiratory Rate Respiratory arrest
Signs of ⇑ ICP with decerebration
o Grades ---------------------------------------------------------------------------------------- *** TQ
Grade I: Intraventricular Hemorrhage just under ependymal ligning
Grade II: Intraventricular Hemorrhage without dilation of ventricles
Grade III: Intraventricular Hemorrhage with dilation of ventricles
Grade IV: Intraventricular Hemorrhage with dilation of ventricles &
Intracerebral Hemorrhage (around the ventricles)
o Dx: Ultrasound on anterior fontanelle
o Rx: Supportive care and it may go away, keep child inside longer
Symptoms
o Loss of motor function & pain-temperature sensation below the level of the lesion
o Preserved posterior column function (position sense and vibration sense)
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Brown Sequard (Hemisection of spinal cord) ------------------------------------------------------------- *** TQ
Symptoms:
o Ipsilateral loss of motor function & proprioceptive sensory function
o Contralateral loss of pain-temperature sensation
Symptoms:
o Loss of motor, pain & To on both side
o * Mnemonic: MUD = Motor > Sensory; Upper > Lower; & Distal > Proximal
Bilateral arm weakness > lower limb weakness with paresis more dense distally
than proximally
Variable sensory impairment (pain & temperature sensory loss > propioceptive
sensory loss)
o Bladder dysfunction
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COMA
Definitions
o Sleep - a state of non-pathological decreased mental status from which the patient can be
readily aroused to full consciousness
o Coma - refers to a state of markedly depressed consciousness such that the patient is
poorly responsive, or unresponsive, to vigorous physical stimuli
o Stupor - refers to a state of severely impaired arousal with some purposiveful
responsiveness to vigorous stimuli; however, the stuporous patient drifts immediately into
unconsciousness when vigorous stimulation ceases
o Obtundation - refers to a lesser state of decreased arousal with some purposiveful
responsiveness to touch or verbal stimuli
o Lethargy - refers to a state in which arousal, though diminished, is maintained
spontaneously or with repeated light stimuli; however, the lethargic patient often drifts
slowly into unconsciousness if left alone
o Confusion - refers to a state of adequate arousal, but with clinical evidence of cognitive
dysfunction
Sudden onset of Coma can be due to: (Vascular or Traumatic)
o Cardiac Hypoxia Encephalopathy
o Subarachnoid hemorrhage
o Basilar (brainstem) artery occlusion
o Pontine hemorrhage
o Bilateral Cerebral infarction
o Brain Trauma
Coma that developing minutes to hours can be due to: (Metabolic - Toxic)
o Metabolic – Hypoxic, Hypoglycemia, DKA, Renal, Hepatic
o Infection
o Toxic
o Hypertensive crises Hypertensive Encephalopathy
o Reyes syndrome
Focal deficits followed by Coma Most likely due to Herniation
o Herniation can follow Brain Trauma, Hemorrhagic Stroke or Bleeding from Tumor
Tonsilar Herniation Medulla compression stop breathing Death
Uncal Herniation III nerve compression pupil’s dilation
Central (Thalamic) Herniation: Rare
*** It is often possible to differentiate metabolic coma from brainstem coma by methodically
evaluating brainstem function
3 useful clinical indicators of brainstem dysfunction that can help differentiate Brainstem
coma from Metabolic coma:
o Abnormal breathing patterns
o Abnormal pupillary responses
o Abnormal eye movements
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Diagram showing the change in pupil size and reactivity - depending on the site of pathology
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Summary of some key clinical features of coma
The combination of deep coma (even with total flaccidity or abnormal posturing) + briskly
reactive pupils almost always means a non-brainstem metabolic coma
The combination of deep coma + spontaneous eye movements (or conjugate deviation of the
eyes on oculovestibular reflex caloric testing) almost always means a non-brainstem metabolic
coma
The combination of coma + brisk pupillary reflexes + poor/absent eye movements strongly
suggests a sedative drug overdose
A comfortable-looking patient, who has a normal breathing pattern +/- yawning, suggests a
metabolic coma
An uncomfortable-looking patient + fixed mid-position/large pupil + absent eye movements +
abnormal breathing pattern +/- coughing/swallowing/hiccuping motions suggests brainstem
coma (primary or secondary) and the probable need for intubation and further aggressive
management
The combination of DEEPENING coma + unilateral DILATING pupil (or an unilateral
dilated pupil not due to mydriatics or eye disease) suggests a rapidly expanding supratentorial
process and secondary transtentorial herniation until proved otherwise
The DILATING pupil is usually on the side of the supratentorial pathology, while the
hemiplegia is usually contra-lateral (occasionally ipsilateral - suggests Kernohan's syndrome)
=> therefore use the dilating pupil as the primary guide when deciding on the likely side of
supatentorial pathology
VERTIGO / DIZZINESS
The most important initial determination that needs to be made in a patient with acute dizziness is to
determine whether the patient's dizziness represents True Vertigo - rather than being due to some other
specific/non-specific syndrome causing dizziness eg. non-specific lightheadeness or near-syncope/pre-
syncope or dysequilibrium syndrome
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Vertigo
Sensation of disorientation in space with a sensation of motion where no motion exists, and the
sensation may be swaying, tilting or rotational in character:
o Mild vertigo may produce a feeling that the earth is moving or tilting slightly, or it may
produce a vague sensation of motion-sickness, or a feeling that one is being pulled to the
side; while severe vertigo may produce a distinct whirling sensation with prominent
autonomic symptoms of nausea and vomiting and sweating and pallor
o Vertigo is due to a mismatch of two-or-more of the vestibular, visual and proprioceptive
inputs to the vestibular system and thus any unilateral lesion of the vestibular apparatus
/ pathways may produce Vertigo
Must differentiate between acute peripheral vertigo (due to unilateral peripheral Vestibular disease)
from acute central vertigo (due to Cerebellar or Brainstem disease) ------------------------------- *** TQ
Central Vertigo
Vestibular nucleus is involved
o NOT the inner ear connections
o Commonly get Brainstem signs as well because the nucleus is near the MLF and other
cranial nerves involved in vision
Can get Cerebellar symptoms
Central Vertigo patient will complain more about Balance problems & Nystagmus --------------- ***TQ
Peripheral Vertigo patient will complain more about Spinning ---------------------------------------- ***TQ
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Central vs. Peripheral Vertigo
Can distinguish by nystagmus
o Central vertigo - nystagmus usually worse towards the side of lesion
Ex: right brain stem lesion, eyes jerk more when look to the right
o Peripheral vertigo - nystagmus usually worse (+2) when looking towards the good ear
Alcohol
Mainly affect cerebellum (vermus) ataxia
Also affect the MD nucleus of Thalamus problem in formations of new memories
o Must give Thiamine before Dextrose
Wernicke’s encephalopathy ------------------------------------------------------------------ ***TQ
o Seen in chronic alcoholics
o Ataxia—affects vermis of cerebellum
More trouble walking than with extremities
o Intranuclear ophthalmic lesions
Strabismus of eyes (eyes look in different directions)
Confusion
Nystagmus
Korsakoff’s Amnesia ---------------------------------------------------------------------------- ***TQ
o Also seen in chronic alcoholics
o Anterograde amnesia—loss of memory for things after amnesia started
o Can not form new memories
o Continues last for years
o Confabulations - if ask what have been doing lately they will make up stories because they
can’t remember
Withdrawal seizures
o If heavy drinker who suddenly stops
Auditory hallucinations with clear sensorium
Delorium tremens=really bad shakes
• ~15% mortality
• Tremendous adrenergic rush
• High blood pressure, cardiac problems, heart attacks
• Rx: give them a sedative and then gradually withdraw them from that
o Typically Librium (Benzodiazepine)
Arsenic
o Get loss of muscle and weight
Lead poisoning
o In children who eat paint
o ⇑ ICP, Coma & Seizures
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Reyes syndrome ---------------------------------------------------------------------------------- ***TQ
o Metabolic derangement post infection
o Upper respiratory infection that leads to liver derangement
o Vomiting, lethargy, liver function test decline (⇑ liver enzymes), sleepiness, ⇑ ICP
eventually they herniated
Hyperthyroidism -------------------------------------------------------------------------------- ***TQ
o Exopthalmos (bulging of eyes)
o Myopathy
o Worsens myasthenia gravis
o Hypocalcaemia periodic paralysis
o Atrial fibrillation
o Attention tremors
o Psychiatric disorders (Anxiety)
Hypothyroidism ---------------------------------------------------------------------------------- ***TQ
o Muscle problems
o Peripheral Neuropathy
o Ataxia
o Diploplia
o Depression/Psychosis & Dementia
Vit. B12 deficiency
o Don’t give Nitrous Oxide anesthesia for Vit B12 deficiency patients severe subacute
combined degeneration (affect Posterior Columns & Cortico-Spinal tracts) loss of
vibration with quadraplegia -------------------------------------------------------------- ***TQ
Diabetes
o Nerve infarcts (III CN & other)
o More prone to strokes
Ischemic strokes
• Especially if have hypertension
Acute intermittent porphyria ------------------------------------------------------------------ ***TQ
o Metabolic problem with an enzyme involved in heme metabolism
o Inherited disorder
o Triad of symptoms
GI
Psychiatric
Neurological
o Rx: high carb. diet then Indural prevents them from having the problem
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NEURO – ONCOLOGY
Metastatic Spread to Brain
o Most common are breast and lung cancer ----------------------------------------------- ***TQ
o Hypernephroma (kidney) and melanoma (skin) can also spread to brain
o Prostate cancer rarely metastasizes to brain
Metastasizes to spinal cord & Posterior fossa via venous plexus (Batson plexus)
Metastatic Spread to Spinal Cord
o Lung and breast cancer
o Prostate cancer especially to lumbar area
o Lymphomas & Leukemia
Spinal cord compression
Acute - can give radiation even before scan because lymphoma is most common
and they are very radio sensitive (if cause unknown)
Paraneoplastic Syndrome
o Cerebellar degeneration could be malignant
o Small cell cancer in the lungs produces Eaton Lambert Syndrome -------------- ***TQ
Small cell cancer can induce limbic encephalitis (rare)
Antibodies against the presynaptic release of acetylcholine
Myasthenia gravis is antibodies against the post-synaptic neuromuscular junction
Eaton Lumbert is antibodies against the pre-synaptic release of Acetylcholine
• Have muscle weakness
o Eaton Lumbert gets better with exercise b/c more release of
acetylcholine
o Myasthenia Gravis gets worse with exercise
o Eaton Lumbert does NOT usually involve the face
o Myasthenia gravis does usually involve the face
• Eaton Lumbert get dyploplia
o Sensory neuropathies
Lose sensation in legs unexplainably
Can come from cancer (from somewhere else)
Dementia
Obstoclonus/Myoclonus ----------------------------------------------------------- ***TQ
• Commonly seen with neuroblastoma
• Myoclonus - jerking movements of any part of body
• Obstoclonus - jerking movements of the eyes
o Progressive Multifocal Leukoencephalopathy ------------------------------------------- ***TQ
Due to papovo virus when immunosuppressant
Can also get DIC (disseminated intravascular coagulation) emboli infarction
o Shingles from herpes zoster
Pain Vesicular rash
Common in immunosuppressed who get cancer
Leukemia & lymphomas
o Leukemia can affect the CNS in that:
If affecting platelets get bleeding
If affecting WBCs get infections (ie. meningitis)
Leukemic cells can get into the CNS Carcinomitis meningitis
• Very high protein levels and very low glucose levels in CSF
Lymphoma can also produce spinal cord compression
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1˚ BRAIN TUMORS
What to know
° Posterior fossa tumors
° Spinal cord tumors
POSTERIOR FOSSA TUMORS (MECAG) ----------------------------------------------- ***TQ
° Cereberallar Astrocytoma
Location: In cerebellar hemispheres
Clinical: Ataxia in ipislateral extremities (arm or leg on same side)
Prognosis: Excellent with surgical therapy
Timing: Symptoms takes months to develop
Typical pt: Older child
° Medulla Blastoma
Location: Along the midline of the cerebellum
Prognosis: Good with chemotherapy
Timing: Symptoms develop quickly
Pt: Youngest children
° Ependymoma
Location: Around lining of the 4th ventricle
Timing: Symptoms develop quickly
° Brainstem Glioma
Location: Within post fossa, in the brainstem
Prognosis: Worst prognosis, no treatment
° General Characteristics
th
Can ⇑ ICP b/c of impeded flow through 4 ventricle
Esp. Medulla Blastoma, Ependymoma (b/c they are more midline)
Youngest kid/shortest duration → Oldest kid/longest duration ----------- ***TQ
MB → Ep → CA → BG (MECAG)
Greastest chance of survival → Worst Prognosis --------------------------- ***TQ
CA → MB → Ep → BG
MIDLINE CERBERAL TUMORS
° In 3rd ventricle
Colloid cyst
Obstructs fluid
Choroids Plexus Papilloma
In choroid plexus
Ependymoma
rd
In ependymal lining of 3 ventricle
° Pineal (around 3rd ventricle)
Gliomas
In hypothalamus/thalamus
Clinical: Intense muscle wasting syndrome
Teratomas
Dystreminomas
Pineolma
Clinical: Early sexual development
Neuroectodermoma
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OPTIC PATHWAY TUMORS
OPTIC NERVE TUMORS ----------------------------------------------------------- ***TQ
°
No vision problems: Benign enlargement of optic nerve
Vision problems: Meningioma or Glioma
° OPTIC PATHWAY TUMORS (nerve+chiasm) ----------------------------------- ***TQ
Neurofibromatosis
Type of optic nerve Glioma
Clinical
• Lesions on skin
• Neurofibroma tumors around peripheral nerves in PNS
• Brainstem & Optic nerve gliomas
Rx: Radiotherapy (only after the age of 7 years)
SUPRASELLAR & CHIASMATIC
° Craniopharyngiomas
Diabetes Insipidus develops after surgery
⇓ Vasopressin (compound released from post pituitary)
⇑ Urination
Treatment: Vasopressin
HEMISPHERIC CEREBRAL
° Intraventricular
Ependymomas
Choroid Plexus Papillomas
° Parenchymal
Glioma
Most common cancer in Brain Parynchyma
Has different stages
Oligodendroglioma
Meningioma
Neuroectodermal
° Glioblastoma Multiforme
Stage IV of Glioma
Most malignant
° Glioblastoma Cerebri
Glioma diffuses in neurons
Spreads throughout brain
Very deadly
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EXTRAMEDULLARY INTRADURAL (60%)
Location:
° Outside of spinal cord, but within the dura
Types
°
Menengioma
Occur in middle age females
In thoracic spine
Neurofibromas
Anywhere in spine
Dumbell Neuromas
• Go through dura, then come out
• Dumbell in shape
EXTRAMEDULLARY (25%)
° Location: Outside of spinal cord and the dura
° Types
Herniated disc
Metastasis
Lymphoma
Arteriovenous malformations (can be intra/extra)
Fractures
Dislocations
Abscesses
Note regarding the MRI
° When people have back pain they get an MRI
50% lumbar MRI
50% cervical MRI
Nobody gets thoracic MRI
° Be careful!
Rare herniated discs can occur in that region
Women can have a Benign Menengioma in the thoracic (a benign tumor that
can easily be removed and cure all symptoms)
Men can have AVM that can present as a tumor
° Thoracic spinal cord symptoms ------------------------------------------------------ ***TQ
⇑ Reflexes in Legs, ⇓ reflexes in arms
OTHER SPINAL CORD LESIONS
CONUS MEDULLARIS
° L2 on adults (lower on children)
° Area of origination for all L2 and lower nerves: pelvic, genital, lower extremities
° 1/2 inch long → tiny but controls a lot, tumors here are very BAD
° Lesion in this (L2-L3) level causes
Saddle anesthesia (in between the legs)
L2, 3, 4: knee jerk lost
S1: ankle jerk lost
Sex and urinary bladder dysfunction
⇓ Anal sphincter tone
CAUDA EQUINA
° Fibers below conus medullaris
° Lesion causes (similar to “pinched nerve”)
Unilateral symptoms
Variable deep tendon reflexes
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SPINAL DISC HERNIATIONS
Most common: L5-S1
°
Next most common:
° L4-5
Rare: ** Central herniation of disc at L1-2, L2-3 levels will effect Conus
°
Medullaris
VASCULAR VS. NEUROGENIC CLAUDICATION ----------------------------------- ***TQ
° Vascular Claudication
Pain after periods of activity (It’s like having angina pectorus in you legs)
Atherosclerosis of the legs, not enough blood getting to legs
The more you use the muscle, the more it hurts
Clinical (Pain with Exercise)
Pain ⇓ when relaxing, and ⇑ during activity
⇓ Pulses in feet, and medial malleosis
Hair growth in feet area
Treatment: bypass surgery to bring blood to legs
° Neurogenic Claudication
Due to Lumbar Stenosis
Clinical (Pain with Standing)
Bulging discs
Arthritis
Osteocytes
Tight region between L3, 4, 5 (cauda equina region)
Pain (Positive Line or Curb Sign)
• ⇑ w/Standing or extending the neck (closes spaces where nerves
exit)
• ⇓ w/Sitting down or flexing the head forward
========================================================================
CNS INFECTIONS
Meningitis
ACUTE ASEPTIC
o Nonbacterial Meningitis, or inflammation of the subarachnoid space, generally viral
usually self-limited and results in a lymphocytic pleocytosis
o Clinically, less fulminant than bacterial meningitis
o Causes:
Viral: Echovirus, nonparalytic poliovirus & coxsackievirus, etc.
Low pathogen Bacteria: Staph Albus, Lyme, Listeria, syphilis, Q-fever
Non inflammatory causes:
• Subarachnoid hemorrhage
• SLE & RA
• Carcinomatous meningitis
o Aseptic Meningitis: ⇑ lymphocytes, ⇑ protein, normal glucose ---------------- TQ
o Carcinomatous Meningitis: ⇑ lymphocytes, ⇑⇑ protein, ⇓⇓ glucose ----------- TQ
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ACUTE SEPTIC / BACTERIAL
o Life threatening (Neurological emergency)
o Pathology: Bacterial inflaming edema & destruction of the cerebral cortex seizures,
confusion or focal neurological signs.
o CSF: ⇑ poly (Not lymphocytes), ⇑ protein, ⇓ glucose
o Other CSF tests:
Gram Stain: (not accurate test): 10% false negative, 30% false negative
Counter immuno electrophoresis (CIE) is very accurate:
• 90% -accuracy with H. flu, B. strep & meningococcus
• 50% accuracy with pneumococcus
Limulus Lipate / (horseshoe crab test): 90% accuracy for Gm-ve bacteria
India Ink: Cryptococcus
Acid-East: TB
Anaerobic: Cultures (specially base skull fracture & Alcoholics)
CHRONIC MENINGITIS
CSF: ⇑ lymphocytes, ⇑ protein, Normal or ⇓ glucose
TB Meningitis
• Pt gets lung TB Brain
• Thick exudate bathes the base of the brain
• Cranial nerve (esp. CN7 & CN6) signs develop & Dementia
Syphilitic meningitis
• Produces dementia
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Cryptococcus
• Occurs mostly in HIV pts (prophylaxis taken)
• India Ink
Coccydium mycosis
• Sewankeen Valley fever
• Common in south-west US
Fungus
• Symptoms: cold, headache, fever, cough, myalgia, apathy, confusion
BRAIN ABCESSES
Most common place: Frontal lobes (b/c they have the most surface area and mass) then temporal,
parietal, occipital
Symptoms: headaches, neurological signs, fever
Most common cause: anaerobic infection
o Other causes: Trauma, neurosurgical manipulation, infection of nearby sinus, blood spread
from heart or lungs
Dx & Rx: LP if safe, drainage & Antibiotics (Pen. or Amp. & Chloromphynicol)
ENCEPHALITIS
Encephalitis vs. Meningeal Encephalitis
o Encephalitis: refers to brain infection, like Herpes Simplex Encephalitis (HSE)
o Menengial Encephalitis: refers to inflammation of meniges
HERPES SIMPLEX ENCEPHALITIS (HSE) --------------------------------------------------------- *** TQ
o Most common brain infection
o Presents with focal symptoms & targets mainly Neonatal
HSE type II at birth
• 60 % mortality if fetus gets Herpes Simplex Encephalitis
• Cause vesicular rash, conjunctivitis, jaundice, bleeding, seizures & coma
• A woman with genital herpes MUST give birth via c-section!
Adult form of HSE
• AKA Limbic Encephalitis (b/c it affects the limbic system)
o S & S ------------------------------------------------------------------------------------------ *** TQ
Before it enters the brain: Genital herpes, bells palsy, vesicular rash
Once it enters the brain: Bizarre behavior initially, then high fever, lethargy,
Seizures, Confusion Coma Death
CSF: ⇑ Polys, ⇑ Protein & RBCs
o Target & Dx
Frontal region & Temporal lobes (maybe b/c it enters through sinus)
MRI shows temporal lobe gets inflamed & necrotic
Encephalogram shows spike activity in temporal lobes
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o Rx ----------------------------------------------------------------------------------------------- *** TQ
Treatment must be started at earliest suspicion
Pts with meningitis start treatment with a Acyclovir (IV) while waiting for results
o Oat-cell Cancer
Paraneoplastic syndrome --------------------------------------------------------- *** TQ
Symptoms resemble Herpes Simplex Encephalitis (Limbic Encephalitis)
SYPHYLIS
o Great Mimicker (along with HIV) b/c there is a great variety of possible symptoms
o Sx & Dx
May be Asymptomatic, just involve genitals & joints
CSF for VDRL even if blood VDRL is negative
CSF: ⇑ lymphocytes, ⇑ protein, Normal glucose
Syphilis meningitis with cranial nerve palsy & epilepsy
Inflammation of arteries & Strokes
General Pareisis: Dementia w/Upper motor neuron lesions
Argyle-Robinson pupils ---------------------------------------------------------- *** TQ
• Accommodate (far to near), but don’t react to light
• Prostitute Pupils: with syphilis would Accommodate, but Not React
Optic Atrophy with blindness
Diffness
Dementia
Tabes-Dorsalis: ⇓ vibration, ⇓ proprioception, ataxia (+ Romberg sign) &
+ Babinskis ---------------------------------------------------- *** TQ
Anterior spinal syndrome: infarct damage to corticospinal tract ⇓ pain
HIV
o Neurological Symptoms
Dementia
• Deficits in attention & concentration
• Due to demyelination between frontal lobes & the rest of the brain
Myelopathy
• Due to demyelination of spinal cord
Myopathy
• The muscle are affected proximal weakness
Neuropathy
• Can affect axons or myelin around nerves
• Can affect the blood supply Infarction of nerves
Opportunistic infections .
• Toxoplasmosis meningitis
• Cryptococcal meningitis
Neoplasms
• Lymphoma
• Leukemic infiltration
• Plasmocytomas
• Karposi’s sarcoma (which can go to brain)
Vascular
• Ischemic or hemmoragic vascular abnormalities
• Due to arteritis & maybe associate with syphilis
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Parasites
o TOXOPLASMOSIS
Pigeon exposure
Common in HIV
Sx & Dx
• MRI or CT scan w/contrast
o Ring Enhancing Lesions (Hole with surrounding inflammation)
• CSF: ⇑ Lymphocytes (Eosinophylia), ⇑ Protein & ⇓ glucose
o Remember: ⇑ Lymphocytes in Viruses, Fungus & Parasites
⇑ PMN in Bacteria
o CYSTICEROSIS
Tinea Solium (uncooked pork)
Most common parasitic infection of the brain world wide
Ova in GI travel to blood as oncoshperes travel to brain lodge into small
blood vessels live for 1-2 years w/out problems they die the area where
they died becomes inflamed damage occurs corresponding to that area
S & S (depend on location)
• Ex. bugger dies in Right Cerebellar Hemisphere Ipisilateral ataxia
• Ex. Lenticulostriate arteries on the left contralateral paralysis
• Can spill out into 3rd ventricle (must be scooped out during surgery)
Rx: Steroids, Prasiquantal, Albendazole, etc
Viruses
o HERPES ZOSTER - SHINGLES
Occur in 50-70 y.o.
S&S
• Severe Dermatome Pain BEFORE Rash ----------------------------- *** TQ
• Usually in thorax dermatomes, but could be a cranial nerve, too
• Papullo-vesecular rash followed by resolution of the rash
• Severe post herpatic pain may continue for long time
Rx
• Steroids
• Acyclovir or Fanvir
• Pain management (Antidepressants & Narcotics if needed)
o CYTOMEGALOVIRUS (CMV)
Causes multifocal lesions in the brain
Occurs in immunosurpressed pts (Iike cancer or HIV pts)
o PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY (PML)
Papovavirus
Occurs in immunosurpressed pts (Iike cancer or HIV pts)
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DEMYELINATION OF CNS & PNS
I. CENTERAL NS DEMYELINATION
° Primary Idiopathic disease
Multiple Sclerosis **
Most common cause of Central NS Demyelination
Devic’s Disease
Variant of MS
Transverse myelitis: inflammation of one segment of the sp cord
Optic Neuritis: inflammation of optic nerve
Schilder’s Disease
Adrenoleukodytrophy
X-linked Recessive
“lorenzo’s oil” disease
Balo’s Disease
Variant of Schilder’s disease, but with more cysts
° Infections or vaccines which illicit demyelination
Papova virus
Cause progressive multifocal leukoencephalopathy in Immuno-
compromised pts?
MMR (measles/mumps/rubella) & Epstein Barr & Varicella
Cause demeylination
Rubeola virus
Causes SSPE (Subacute Sclerosis Pan Encephalitis)
Very similar to Jacob-Krutzfield disease (Mad Cow Disease)
° Nutritional
Rapid correction of hyponitremia with saline --------------------------- ***TQ
Causes CPM (Central Pontine Meylinolysis)
Pons becomes demyelinated
Toxins in home-made wines
Marchiafava Bignami syndrome
° Inherited
Causes everything (neuropathies, myopathies)
° Toxic/Metabolic
Radiation
Melts myelin
Alcoholism
Hurts myelin (esp. in vermis area of cerebellum)
B12 deficiency
CO poisoning
Hypoxia (⇓ O2)
Mehothrexate (chemotherapy agent)
° MULTIPLE SCLEROSIS ------------------------------------------------------------- ***TQ
Diagnosis
** There must be two different lesions separated in time and space
Optic neuritis or one episode of Transverse Myelitis ≠ MS
• Neuro exam
• MRI (see areas of demyelination in spinal cord &/or brain)
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Myelin basic protein in CSF
• Because it is being broken down
Oligoclonoal band in CSF
• Evidence of antibody reaction against protein
⇓ speed of Evoke Response
• ⇓ Visual ER: Measures how fast electrical activity goes from retina
Optic n.
• ⇓ Auditory ER: speed from tympanic membrane → auditory
cortex
• ⇓ Somatosensory ER: speed from skin → somatosensory cortex
Prognosis, variable: some pts…
Have only two episodes their whole lives
Keep getting progressively worse
Cause (most probable!!!)
Viral presentation, maybe Corona Virus
Body reacts to virus
Body cross reacts the antibody response to CENTRAL myelin
Geographic
More common in temper climate, less in tropical
Maybe viral exposure in EARLY LIFE causes MS b/c
• Moving at 17 from Costa Rica to NY → ⇓ risk
• Moving at 17 from NY to Costa Rica → ⇑ risk
========================================================================
Muscle Weakness
Weakness in proximal part of the leg or the shoulder. That typically refers to Myopathy.
Exceptions such as Guillian Barre & Werdnig-Hoffman neuropathies can sometimes give
proximal weakness.
In general: ---------------------------------------------------------------------------------------------- *** TQ
o Myopathy gives Proximal Weakness
o Neuropathy gives Distal Weakness
Muscular Dystrophies
Limb-Girdle Dystrophy
o Progressive weakness of a limb-girdle
o Inherited
VonFuch’s Disorder
o Affects eye muscles: (Occulopharyngeal) Dystrophy
o Effects eye muscles and the pharynx giving you problems with swallowing.
Paramyotonia congenital
o Affects face forms & hands
Schwartz
o Cranial myotonia in infants
o Abnormalities of the skeletal systems
Congenital myotonia
o Children of mothers with myotonic dystrophy can have respiratory failure and
myotonic weakness at birth.
Inflammatory Myopathies .
o Refers to inflammation of muscles
o Polymyositis & Dermatomyositis
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Poymyositis --------------------------------------------------------------------------------------------------- *** TQ
o Weakness
o No family history
o > 30 years onset
o ⇑ CPK
o Don’t have rashes or any nerve problems
o No other causes of muscle inflammation from alcohol, drugs, or thyroid that would cause
inflammation
o Autoimmune disorder - altered cellular immune response. Lymphocytes are
synthesized against muscle cells and cause inflammation
o Extra ocular muscles are spared
o Rx: Steroids
Polymyalgia Rheumatica
o Muscle inflammation
o A lot of women (greater than 55) with aches and pains
o Much more common disorder where steroids are also helpful
o May be associated with temporal arteritis (inflammation of the arteries and can get infarcts
of the optic nerve-ischemic optic neuropathy) Increased sedimentation rate
Carnitine Deficiency
o Carnitine carries long chain fatty acids inside the mitochondria.
o This deficiency leads to all kinds of muscle & liver problems.
o Can look like Reyes syndrome with increased intracranial pressure, in addition to swelling
of the brain and liver problems
o These are typically inherited
Mitochondrial Encephalopathy
o Problems with mitochondria in the brain
o Brain cells have ragged red fibers in mitochondria
o Cytochrome oxidase complex 4 defect
NERVE DISORDERS
Nerve Disorders
Neuronapathy: disease of the Nerve cell body
Axonapathy: disease of the Axon
Myelinopathy: disease of the Myelin
o Most important myelinopathy is Guillian-Barre
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Axonapathy
o Most common type of neuronapathy
o Occur in patients with diabetes & uremia
Axons start dying because of other metabolic disease
o Glove & Stocking sensory & motor loss: and they can have early symmetrical loss of the
ankle jerk and moderate slowing of the nerve conduction velocity. If the myelin is lost
nerve conduction is slowed
Neuronopathy
o anything that hits the nerve cell body (anterior horn cell)
o Werdnig-Hoffman & ALS - syndromes are involved in neuronopathy
How to difference between Myasthenic crisis & Cholinergic crisis? -------------------------------- *** TQ
Cholinergic Crisis
o ⇑⇑ Ach
o Parasympathetic system is involved because Ach is the neurotransmitter of this system
o Slow heart rate
o Abdominal cramping (due to ⇑ blood flows to the intestine)
Myasthenic Crisis
o ⇓⇓ Ach
o Weakness
If the patient takes too much Mestinon or Tensilon ⇑⇑ Ach cholinergic crisis
If they have ⇓⇓ Ach myasthenic crisis
EMG
Insertional activity: When the patient is relaxed & not moving there should be no muscle activity
o If there is a negative deflection it is called a positive sharp wave
o If there is a positive deflection it is called fibrillations
o Fibrillations & positive sharp waves are signs of nerve problems (Not muscle problems)
o . (polimysitis: characterestc EMG pattern)
Motor unit potentials:
o LMN disease large motor unit potentials
o Myopathic disease small motor unit potentials
Typical EMG patterns in Neuropathy
o Large motor unit
o Fibrillation is in positive sharp waves
o Increased interference pattern
Typical EMG patterns in Myopathy
o No spontaneous activity or small motor unit potential
o Normal insertional activity
EMG pattern of Pyomyositis ---------------------------------------------------------------------- *** TQ
o Inflammation of the muscle but the muscle is also dying
o Combination of both Myopathy & Neuropathy patterns
o Muscular dystrophy with pinched nerve in the back give the same pattern as Pyomyositis
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PEDIATRIC NEUROLOGY
The difference between Ataxic Telagiectasia & Von Hippel Landau ------------------------------ *** TQ
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o Fredrick’s Ataxia --------------------------------------------------------------------------- *** TQ
Onset before 20
Autosomal Recessive > Dominant
Ataxia
Nydtagmus
Hammer toe
Babinski
Kyphoscoliosis
Cardiac dysfunction
In-coordination of hands
Speech difficulty
Progressive course Death after about 15 years
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