Neuro 9

Question 1

Describe acute intermittent porphyria

Answer 1

♣ Deficiency of Porphobilinogen (PBG) deaminase
• THINK: Acute intermittent = guys hollering “damn” (deam-inase) intermittently at A CUTE pretty big girl (PBG) walking by
♣ Symptoms – 5 P’s
• Painful abdomen, Port wine colored urine (due to increase PGB), Polyneuropathy, Psychological disturbances, Precipitated by drugs (CYP450 inducers), alcohol, and starvation

Question 2

What do Anti-Hu antibodies suggest

Answer 2

Paraneoplastic syndrome

Question 3

What sx usually precede Guillain-Barre?

Answer 3

Acute infection, GI or URI

Question 4

What will you see in CSF of Guillain Barre

Answer 4

Elevated protein with few or no cells

Question 5

Clinical presentation of Guillain Barre

Answer 5

♣ Ascending symmetrical paralysis over days to weeks
♣ Absent/depressed deep tendon reflexes
♣ Paresthesias
♣ Autonomic dysfuction (e.g tachycardia, urinary retention, and arrhythmias) occur in 70% of patients
♣ Respiratory compromise
♣ Neuropathic pain is common

Question 6

Presentation of transverse myelitis

Answer 6

• Area of inflammatory demyelination in the spinal cord
• Motor and sensory loss below the level of the lesion with bowel and bladder dysfuntion
• Initially flaccid paralysis followed by spastic paralysis with hyperreflexia
• Patients may report a band of tingling or pain around the torso at the level of the lesion

Question 7

Tx of Guillain Barre

Answer 7

Immunoglobulin or plasmaphoresis

Question 8

Tx of Myasthenic crisis

Answer 8

Plasmapheresis or IVIg, plus corticosteroids

Question 9

What cancer is associated with Lambert Eaton

Answer 9

Small cell lung carcinoma

Question 10

If you see EBV DNA in CSF of AMS HIV patient, what should you think?

Answer 10

CNS lymphoma

Question 11

What other childhood disorder is associated with absence seizures?

Answer 11

ADHD

Question 12

CO poisoning affects what part of the brain?

Answer 12

Bilateral basal ganglia

Question 13

What will you see on EMG in neuropathy

Answer 13

Rapidly firing fibers with decreased recruitment

there are less muscle fibers so remaining ones must fire more

Question 14

What will you see on EMG in myopathy

Answer 14

Early recruitment

there are same amount of muscles but they are weaker

Question 15

What is PRES

Answer 15

• Posterior reversible encephalopathy syndrome (PRES)
♣ Headache
♣ Altered consciousness (acute confusional state)
♣ Visual disturbances (cortical visual loss – blindness with preserved pupillary reactivity)
♣ Seizures

Question 16

ALS causes damage to which part of the spinal cord

Answer 16

Anterior motor horn (LMN) and corticospinal tract (UMN)

Question 17

Syringomyelia causes damage to what part of the spinal cord

Answer 17

• Anterior white commissure (loss of pain and temp)
• Anterior horn (LMN)
• Lateral horn of hypothalamospinal tract (Horners)

Question 18

Damage to non-dominant parietal lobe causes what?

Answer 18

Hemispatial neglect

Question 19

Damage to what causes Erb-Duchenne palsy

Answer 19

Superior trunk (C5-C6)

Question 20

Muscles affected in carpal tunnel

Answer 20

LOAF

• Lumbricals 1-2
• Opponens pollicis brevis
• APB (abductor pollicis brevis)
• FPB (flexor pollicus brevis)

Question 21

Brain vascular supply of upper extremities

Answer 21

MCA

Question 22

Brain vascular supply of lower extremities

Answer 22

ACA

Question 23

Foot drop is caused by damage to what?

Answer 23

Damage to common peroneal nerve (does dorsiflexion)

Question 24

Describe what Cerebral Palsy is

Answer 24

o Is a static (non-progressive) central motor dysfunction
o Can be acquired or genetic
o Risk factors:
♣ Hypoxic ischemic insult to brain in perinatal period, prematurity, low birth weight, chorioamnionitis, prenatal viral infections, prenatal strokes

Question 25

3 types of cerebral palsy

Answer 25

♣ Hemiparetic • Weakness and spasticity seen on one side of the body ♣ Diparetic • Spasticity in all four limbs, but affecting the legs much more than the arms • Often of normal intelligence and less likely to have seizures ♣ Spastic Quadriplegic • All four limbs are affected • Seizures often occur in the first 48 hours of life

Question 26

Clinical features of cerebral palsy

Answer 26

♣ Infants: flaccid weakness, asymmetric limb movements, or seizures ♣ Older children: spasticity, dystonic, developmental delay, drooling

Question 27

What is considered normal IQ

Answer 27

100 with a standard deviation of 15

Question 28

Triad of Tuberous sclerosis

Answer 28

♣ Triad: Angiofibromas, mental retardation, seizures | ♣ Other symptoms: Ash-leaf spots, hamartomas of CNS and skin, angiomyolipoma

Question 29

Neuro finding associated with Neurofibromatosis type 1

Answer 29

Optic nerve glioma

Question 30

Neuro finding associated with Neurofibromatosis type 1

Answer 30

Acoustic schwanomma

Question 31

Will you see miosis or mydriasis in 3rd nerve palsy?

Answer 31

Mydriasis Parasympathetic (constrictors) travel with CNIII, so if you knock these out you get dilation

Question 32

Pt has a seizure and you want to give prophylactic meds until you figure out what is going on, what do you give?

Answer 32

Phenytoin, Phosphenytoin, Valproic acid

Question 33

Tx of venous sinus thrombosis

Answer 33

Anticoagulation

Question 34

What are the 4 midline columns in the brainstem and presentation of damage to each

Answer 34

* Motor pathway (corticospinal tract) – Contralateral weakness * Medial lemniscus – loss contralateral proprioception/vibration * Medial longitudinal fasciculus – Ipsilateral intranuclear ophthalmoplegia * Motor nucleus and nerve – Ipsilateral CN motor loss (3, 4, 6, 12 – midline motor divide into 12)

Question 35

What are the 4 lateral (side) columns in the brainstem and presentation of damage to each

Answer 35

* Spinocerebellar pathway – Ipsilateral ataxia * Spinothalamic – Contralateral pain/temp sensation loss * Sensory nucleus of CN 5 – Ipsilateral pain/temp loss in face * Sympathetic pathway – Ipsilateral Horner’s syndrome

Question 36

Parts of the spinal cord affected + sx of ALS

Answer 36

Affects anterior horn (LMN) and corticospinal tract (UMN)

Question 37

Blood supply of medial and lateral midbrain

Answer 37

PCA

Question 38

Describe acute intermittent porphyria

Answer 38

♣ Deficiency of Porphobilinogen (PBG) deaminase • THINK: Acute intermittent = guys hollering “damn” (deam-inase) intermittently at A CUTE pretty big girl (PBG) walking by ♣ Symptoms – 5 P’s • Painful abdomen, Port wine colored urine (due to increase PGB), Polyneuropathy, Psychological disturbances, Precipitated by drugs (CYP450 inducers), alcohol, and starvation

Question 39

Damage to PPRF causes what?

Answer 39

Eyes look away from side of the lesion

Question 40

Pronator drift is a sign of damage to what?

Answer 40

Corticospinal tract (pyramidal tract)

Question 41

Diagnose: sudden onset painless monocular vision loss with cherry red spot

Answer 41

Retinal artery occlusion

Question 42

Describe path of corticospinal tract

Answer 42

♣ UMN from motor cortex descend to pyramid in the medulla ♣ Axons cross to contralateral side in the caudal medulla and descend via lateral cortico-spinal tract ♣ Enter the spinal cord at lateral white column and travel to the anterior grey horn ♣ Synapse to LMN (alpha motor neuron) which then exits via ventral spinal root to innervate muscle

Question 43

Describe Tuberous sclerosis presentation

Answer 43

♣ Triad = Angiofibromas, mental retardation, seizures | ♣ Other symptoms: Ash-leaf spots, hamartomas of CNS and skin, angiomyolipoma

Question 44

Describe Sturge Weber syndrome

Answer 44

♣ Port-wine stain of the face in CN V1/V2 distribution ♣ Seizures/epilepsy ♣ Intellectual disability ♣ Glaucoma

Question 45

Triad of Wernicke

Answer 45

Confusion, ataxia, ophthalmaplegia

Question 46

Presentation of CJD

Answer 46

Rapid onset dementia + ataxia + startle myoclonus Periodic sharp waves on EEG

Question 47

What function is PPRF and Edinger Westphal nucleus involved in?

Answer 47

``` PPRF = voluntary eye movement EWN = parasympathetic pupillary reflex ```

Question 48

What is one of the earliest and most unique sx of Parkinsons

Answer 48

Loss of smell

Question 49

Location of damage / presentation of Friedrich ataxia

Answer 49

♣ Degeneration of cerebellum and spinal cord • Ataxia, muscle weakness, loss of vibratory sense and proprioception, diabetes mellitus, hypertrophic cardiomyopathy, slurred speech, dysphagia • Presents in childood with kyphoscoliosis ♣ THINK: Friedreich is fratastic (frataxin): he’s your favorite frat brother, always staggering and falling but has a sweet, big heart

Question 50

Describe Meniere disease

Answer 50

♣ Increased pressure and volume of endolymph ♣ Features: • Episodic vertigo with nausea and vomiting • Ear fullness/pain • Unilateral sensorineural hearing loss • Tinnitus

Question 51

Paraneoplastic cerebellar degeneration is usually associated with what cancers?

Answer 51

Gynecologic or small cell lung cancer

Question 52

Tx of Guillain Barre

Answer 52

IV Ig or plasmapheresis

Question 53

Tx of essential tremor

Answer 53

Beta blockers Primidone Self-medicated via alcohol

Question 54

Apraxia is caused by damage where?

Answer 54

Cortex (posterior parietal)