Neuro 9 Flashcards

1
Q

Describe acute intermittent porphyria

A

♣ 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

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2
Q

What do Anti-Hu antibodies suggest

A

Paraneoplastic syndrome

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3
Q

What sx usually precede Guillain-Barre?

A

Acute infection, GI or URI

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4
Q

What will you see in CSF of Guillain Barre

A

Elevated protein with few or no cells

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5
Q

Clinical presentation of Guillain Barre

A

♣ 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

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6
Q

Presentation of transverse myelitis

A
  • 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
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7
Q

Tx of Guillain Barre

A

Immunoglobulin or plasmaphoresis

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8
Q

Tx of Myasthenic crisis

A

Plasmapheresis or IVIg, plus corticosteroids

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9
Q

What cancer is associated with Lambert Eaton

A

Small cell lung carcinoma

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10
Q

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

A

CNS lymphoma

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11
Q

What other childhood disorder is associated with absence seizures?

A

ADHD

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12
Q

CO poisoning affects what part of the brain?

A

Bilateral basal ganglia

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13
Q

What will you see on EMG in neuropathy

A

Rapidly firing fibers with decreased recruitment

there are less muscle fibers so remaining ones must fire more

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14
Q

What will you see on EMG in myopathy

A

Early recruitment

there are same amount of muscles but they are weaker

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15
Q

What is PRES

A

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

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16
Q

ALS causes damage to which part of the spinal cord

A

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

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17
Q

Syringomyelia causes damage to what part of the spinal cord

A
  • Anterior white commissure (loss of pain and temp)
  • Anterior horn (LMN)
  • Lateral horn of hypothalamospinal tract (Horners)
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18
Q

Damage to non-dominant parietal lobe causes what?

A

Hemispatial neglect

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19
Q

Damage to what causes Erb-Duchenne palsy

A

Superior trunk (C5-C6)

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20
Q

Muscles affected in carpal tunnel

A

LOAF

  • Lumbricals 1-2
  • Opponens pollicis brevis
  • APB (abductor pollicis brevis)
  • FPB (flexor pollicus brevis)
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21
Q

Brain vascular supply of upper extremities

A

MCA

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22
Q

Brain vascular supply of lower extremities

A

ACA

23
Q

Foot drop is caused by damage to what?

A

Damage to common peroneal nerve (does dorsiflexion)

24
Q

Describe what Cerebral Palsy is

A

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

25
Q

3 types of cerebral palsy

A

♣ 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

26
Q

Clinical features of cerebral palsy

A

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

27
Q

What is considered normal IQ

A

100 with a standard deviation of 15

28
Q

Triad of Tuberous sclerosis

A

♣ Triad: Angiofibromas, mental retardation, seizures

♣ Other symptoms: Ash-leaf spots, hamartomas of CNS and skin, angiomyolipoma

29
Q

Neuro finding associated with Neurofibromatosis type 1

A

Optic nerve glioma

30
Q

Neuro finding associated with Neurofibromatosis type 1

A

Acoustic schwanomma

31
Q

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

A

Mydriasis

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

32
Q

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

A

Phenytoin, Phosphenytoin, Valproic acid

33
Q

Tx of venous sinus thrombosis

A

Anticoagulation

34
Q

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

A
  • 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)
35
Q

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

A
  • 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
36
Q

Parts of the spinal cord affected + sx of ALS

A

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

37
Q

Blood supply of medial and lateral midbrain

A

PCA

38
Q

Describe acute intermittent porphyria

A

♣ 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

39
Q

Damage to PPRF causes what?

A

Eyes look away from side of the lesion

40
Q

Pronator drift is a sign of damage to what?

A

Corticospinal tract (pyramidal tract)

41
Q

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

A

Retinal artery occlusion

42
Q

Describe path of corticospinal tract

A

♣ 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

43
Q

Describe Tuberous sclerosis presentation

A

♣ Triad = Angiofibromas, mental retardation, seizures

♣ Other symptoms: Ash-leaf spots, hamartomas of CNS and skin, angiomyolipoma

44
Q

Describe Sturge Weber syndrome

A

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

45
Q

Triad of Wernicke

A

Confusion, ataxia, ophthalmaplegia

46
Q

Presentation of CJD

A

Rapid onset dementia + ataxia + startle myoclonus

Periodic sharp waves on EEG

47
Q

What function is PPRF and Edinger Westphal nucleus involved in?

A
PPRF = voluntary eye movement
EWN = parasympathetic pupillary reflex
48
Q

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

A

Loss of smell

49
Q

Location of damage / presentation of Friedrich ataxia

A

♣ 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

50
Q

Describe Meniere disease

A

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

51
Q

Paraneoplastic cerebellar degeneration is usually associated with what cancers?

A

Gynecologic or small cell lung cancer

52
Q

Tx of Guillain Barre

A

IV Ig or plasmapheresis

53
Q

Tx of essential tremor

A

Beta blockers
Primidone
Self-medicated via alcohol

54
Q

Apraxia is caused by damage where?

A

Cortex (posterior parietal)