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A 4 year old girl who was born prematurely presents with difficulty walking. She also has spastic gait, chorea, athetosis, & increased motor tone. What is your diagnosis?
- Cerebral Palsy.
- Delayed motor skills.
- Motor defecit, unknown etiology.
- More common in premies (due to periventricular hemorrhage affecting white matter).
- Corticospinal tract affected = altered leg movement.
- Spastic gait: clumsy & stiff.
- Chorea: abrupt, jerky movements of limbs.
- Athetosis: slow, withering, continuous movements.
A 19 year old woman presents with decreasing performance in college & an abnormal eye exam. She has been clumsy with her hands, trips while walking, has decreased strength, resting tremors, & Kayser-Fleischer Rings.
Clinical presentations: tremor, clumsiness, rigidity, speech slurring, drooling, uncontrollable grinning, dyskinesia, personality changes, chronic hepatitis, portal hypertension, & abnormal liver function tests.
- Wilson's Disease: hepatolenticular degeneration.
- Autosomoal recessive disease.
- Leads to accumulation of copper in the blood.
- Copper stones are deposited in the liver, brain, & eyes.
- Hepatitis is generally the presenting symptom = may lead to cirrhosis.
- Basal ganglia affected.
A ten year old boy is seen by a neurologist because of progressive difficulty walking & diminution of vision. The patients has a right foot deformity (pes cavus). He presented to a cardiologist with hypertrophic cardiomyopathy. He has an uncle that died of cardiac complications. He also presents with diabetes mellitus, ataxia, nystagmus, dysarthia, areflexia, positive Babinski sign, & decreasedsensations in the lower limbs.
- Friedeich's Ataxia.
- Autosomal-recessive inheritance.
- Three tracts degenerate: pyramidal, dorsal, & spinocerebellar.
- Cardimyopathy, skeletal abnormalities, & optic atrophy.
- Increased incidence of diabetes mellitus.
- Ataxia with progressive involvement of all the extremities.
A 54 year old man was brought to the emergency room after 1 week of confusion & lethargy. He has a history of chronic alcohol abuse & poor eating habits. He is lethargic, not oriented, & has endgaze nystagmus. Labs show a thiamine deficiency.
- Wernicke-Korsakoff Syndrome: cause by B1 deficiency.
- Frequently seen in alcoholics & severely malnourished patients.
- Triad of acute mental confusion, opthalmoplegia, & gait ataxia = CN VI.
- Korsakoff's Pyschosis: anterograde & retrograde amnesia, confabulation.
A 21-year-old man presents to the emergency room after loosing consciousness 30 minutes ago. He has sustained rhythmic tonic-clonic contractions of his extremities since then with eye rolling, tongue bitting, & urinary incontince. He has not regained consciousness at any point. He has a known seizure disorder but has been non-complaint with his medications.
- Status Epilepticus = neurological emergency.
- Prolonged seizure activity can cause permanent brain damage.
- In children, prolonged fever may also cause SE.
- In adults it is often caused by strokes or withdrawal of anticonvulsants.
A 16-year-old bot presents with sudden loss of consciousness & muscle hypertonia, followed by rhythmic movements of the limbs with upward rolling of the eyes, tongue biting, & urinary incontince; he is in a state of confusion & lethargy.
- Grand Mal Seizure.
- Stereotpic rhythmic movements.
- Urinary incontince.
- Prolonged post-ictal confusion.
- May occur with no aura or warning.
A 16-year-old boy is brought to the physician by his mother, who reports two recent episodes during which she observed her son to be "out of it." He initially complained of an unpleasant smell, suddenly stopped talking & stared straight ahead, after which the patient was confused & sleepy. When he was an infant he had herpes encephalitis.
- Complex Parital Seizures.
- Involves loss of consciousness & frequently follows an auditory or olfactory aura.
- Cerebral scar tissue may be focus of seizure activity.
- Can still make basic verbal responses or follow simple motor commands.
- Absence Seizure: staring into space with finger fumbling, lip smacking, & blinking.
- Children 3 - 13 yo.
- Petite mal seizures.
- No aura.
- Febrile Seizure: due to high fever.
- Older than 6 months, younger than 5 yo.
- Parkonson's Disease.
- Loss of dopaminergic neurons in the basal ganglia.
- Onset between 45-65 yo.
- Resting tremor: asymmetric.
- Bradykinesia: microgrpahia, decreased facial expression, decreased blink rate, soft speech.
- Huntington's Disease.
- Autosomal dominant.
- 35-45 yo.
- Parkinsonian features, & eventually akinesia.
- Gilles de la Tourette's Syndrome.
- Multiple motor & vocal tics.
- Wallenberg Syndrome (Lateral Medullary Infarct or PICA Syndrome).
- Brain stem infarction of the lateral medulla.
- Contralateral loss in pain & body temp from body.
- Ipsilateral loss of pain & temp from face.
- Ipsilateral dysphagia, hoarseness, diminished gag reflex.
- Vertigo, diploplia, nystagmus, vomitting.
- Ipsilateral Horner's syndrome.
AND NOW, AMERIPRESS NOTES!
What are the 4 main components of the brainstem?
- 1. Cranial nerve nuclei.
- 2. Long tracts.
- 3. Cerebellar circuitry.
- 4. Reticular formation.
- 1. Corticospinal tract: voluntary movement.
- 2. Corticobulbar tract: voluntary motor for cranial nerves
- 3. Dorsal column: vibration, light touch, conscious proprioception.
- 4. Anterolateral pathway: pain sensation from the body.
- Reticulospinal tract.
- Vestibulospinal tract.
- Tectospinal tract.
3 cerebellar peduncles: back of brainstem.
Cerebellum influences UMN's.
- 1. Inferior: sensation from body.
- 2. Middle: cerebellar hemisphere to cerebellar hemisphere.
- 3. Superior: information leaving cerebellum to go up to cerebrum.
What are the 3 main functions of the Reticular Formation?
- 1. Pain modulation.
- 2. Arousal: upper midbrain.
- 3. Posture & locomotion.
What is generally the cause of Locked-In Syndrome?
- A major infarct in the ventral pons, via a thrombosis in the basilar artery.
- Absent motor function = patient is unable to move anything but eyes.
- Intact sensation & cognition = patient is aware & able to feel.
Ataxia: uncoordinated gait, clumsiness.
Which part of the reticular formation deals with consciousness?
The rostral part
- Caudal = reflexes & autonomic motor function.
- *hiccups case study*
Diziness & nausea are associated with which regions of ischemia?
CN VIII or cerebellum.
Diploplia (double vision) & blurry vision are associated with what region of ishemia?
Eye movement pathways.
An infarct in which area of the brain results in incoordination?
Unsteady gait = cerebellum & corticospinal tract.
An infarct in which area of the brain would affect CN's III & IV?
- Pupillary dialation (CN III).
- Flexor posturing = stroke posture.
Trigeminal nerve signs, bilateral Babinski, generalized weakness, facial tingling, upper/lower vision loss, & extensor posture are all associated with an infarct in which area of the brain?
Hiccups are associated with which cranial nerve?
Medulla = CN X: nausea, vomiting, respiratory arrest, autonomic instability, & hiccups.
Medulla = CN VIII: vertigo, nystagmus.
Damage to posterior columns is associated with which 2 arteries?
- 1. Vertebral A.
- 2. Basilar A.
Name 2 a/k/a's for the Cerebellum.
Cerebellum: plans & coordinates movements.
- 1. Cerebellovestibular system.
- 2. Vestibulocerebellar system.
Name 2 a/k/a's for the lateral cerebellum.
- 1. Neocerebellum.
- 2. Cerebrocerebellum.
- Lateral cerebellum: motor planning "feed forward."
- Cerebellum tells UMN's to fire in the correct order.
- Cognitive function.
Name 2 a/k/a's for the intermediate cerebellum.
- 1. Archicerebellum.
- 2. Paleocerebellum.
- Intermediate cerebellum: monitors ongoing movements.
- "Feed back."
What is the a/k/a for the vermis?
Spinocerebellum = trunk control.
What is an a/k/a for the flocculonodular lobe?
True or false: the spinocerebellar tract is responsible for conscious proprioception.
False: spinocerebellar = unconscious proprioception.
Basal ganglia problems are associated with what type of tremors?
- Dysmetria: intention, or action tremor.
- Action tremor (intention tremor): cerebellar problem.
Charcot's triad is a sign of which pathology?
Charcot's triad: nystagmus, scanning speech, intention tremor = MS in cerebellum.
Which nucleus is affected by Wilson's Disease?
The lentiform nucleus.
I AM BURNT OUT.
HERE'S THE REVIEW FROM THE BACK OF AMERIPRESS.
- Uncoordinated wavering movement.
- Ipsilateral to lesion.
- Bilateral lesion of thalamus.
- Upper brainstem reticular formation.
- Bilateral regions of cerebral cortex.
- Lateral medulla.
- Ipsilateral ataxia.
- Ipsilateral decreased facial pain & temperature sense.
- Contralateral decreased body pain & temp sense.
- Ipsilateral Horner's Syndrome: ptosis, miosis, anhidrosis.
- Hoarseness or dysphagia.
- Ipsilateral decreased taste.
Medial medullary syndrome:
- Contralateral arm/leg weakness.
- Contralateral decreased position & vibration sense.
- Ipsilateral tongue weakness.
- Females more.
- Steptococcus group B exposure.
- Exhibits choreiform movement.
- Large proximal motions.
- Rotary, flinging movements.
- Fastest movement of disorders.
- Sudden rapid muscular jerk.
- Neurodegenerative disorder.
- Memory loss.
- Motor aphasia: can't speak or write.
- In frontal lobes (dominant).
- Next to motor strip = face, mouth, hands.
- Decreased fluency.
- Patient can understand but not talk.
- Phrase length less than 5 words.
- Naming difficulties.
- Repetition is impaired.
- Comprehension is intact.
- Reading aloud & writing are difficult.
Wernicke's Aphasia: patient on episode of house.
- Comprehension is impaired.
- Can talk but not understand.
- Does not respond to questions.
- Spontaneous speech is normal but full of errors.
- Similar words in sound of function used = cable instead of table.
- Speech errors: paraphasic (similar word), phonemic (word that rhymes), neologism (makes up new word).
- Naming is imparied.
- Repetition is impaired.
- Anosognosia = unaware.
- May display angry or paranoid behavior.
- Both Wernicke's & Broca's are affected.
- Impaired fluency.
- Impaired comprehension.
- Impaired repetition.
- Lesion in arcuate fasciculus.
- Poor repetition.
Alexia & Agraphia:
Impaires reading & writing.
- Can't follow command.
- Sloppily dressed.
- Stooped posture.