Neurology Amboss

Question 1

Genioglossus
Styloglossus
Hyoglossus

Answer 1

CN 12

1. Protrude tongue
2. draw side of tongue upward to create trough
3. Hyoglosseus retract and depress tongue

Question 2

Closing of the Jaw nerve

Answer 2

CN V3 Motor

Question 3

Jugular Foramen

Answer 3

CN IX X XI, jugular vein

Question 4

Glucose intolerance and malignant otitis externa

Answer 4

A subtype of otitis externa characterized by a necrotizing inflammation of the external auditory canal. Most frequently caused by Pseudomonas aeruginosa. Risk factors are poorly controlled diabetes mellitus and immunosuppression.

Question 5

Otitis media most likely caused by

Answer 5

Strep Pneumonia

and for children externa is H influenzae or pseudomonas for diabetic patients etc.

Question 6

Pus filled mastoid air cells is caused by

Answer 6

infection in middle ear traveling to mastoid air cells

Question 7

Glycogen synthase kinase 3

Answer 7

Activation of glycogen synthase kinase-3 and other protein kinases induces increased phosphorylation of tau which leads to cluster formation and development of tangles.

Prion Beta pleated sheets are rapidly progressing dimentia

Question 8

Helicotrema

Answer 8

located near the apex of cochlea. Lower frequency sound waves vibrate the basilar membrane of the helicotrema, which converts this input into nerve signals that encode auditory information

Question 9

Otitis Media can cause conduction hearing loss by

Answer 9

damaging tympanic membrane

Question 10

Rasagiline Selegilline Side effects

Answer 10

Nausea, headache, confusion, hallucination

Question 11

COMT (catechol-O-methyl transferase)

Answer 11

Blocked by Entacapone, tolcapone
in circulation L dopa —> 3OMD

in brain
dopamine —> 3-MT

Side effects:

dyskinesia, hallucinations, confusion, nausea, orthostatic hypotension, diarrhea

Question 12

Lymphocytic infiltrate of endoneurium

Answer 12

GBS

Question 13

Immune complex deposition of arterial endothelium

Answer 13

Vasculitis and found in Lupus, commonly will have purpura in vasculitis

Question 14

Spirochete protein cross reactivity in the meninges

Answer 14

aseptic meningitis in Lyme disease

Question 15

Osmotically mediated Schwann cell damage

Answer 15

diabetic neuropathy

Question 16

Pituitary adenoma can be secreting what

Answer 16

1. lactotrophs most commonly

2. somatotrophs GH

Question 17

Lateral gaze paralysis and horizontal nystagmus can be found in

Answer 17

Alcoholics

Question 18

Essential tremor

Answer 18

worsens with movement

Question 19

Internal capsule compartments

Answer 19

Determine fibers pass in each parts of the internal capsule :

• Anterior limb → thalamo cortical fibers
• Genu → cortico bulbar fibers
• Posterior limb → cortico-spinal & somatic sensory fibers

Question 20

Ventral thalamus infarct vs Posterior limb of internal capsule

Answer 20

Ventral thalamus is pure sensory but posterior limb motor function is off

Question 21

Deviation of Uvula vs Tongue?

Answer 21

Uvula will move to the side where the lesion is not for CN X and expect also hoarseness

Tongue will deviate to side of lesion is CN 12 damage, or other side if upper neuron cortex damage

Question 22

Spinal acessory nerve

Answer 22

arises from upper cervical spine C1-C5

Question 23

Trisomy 21

Answer 23

1st trimester BHCG up, PAPPA down

2nd trimester BHCG up, inhibin A up, estriol down, AFP down

Question 24

Mutations in the sonic hedgehog signaling pathway cause holoprosencephaly. This may be seen in

Answer 24

trisomy 13 or fetal alcohol alcohol syndrome.

Question 25

Warfarin in pregnancy

Answer 25

warfarin is known to cross the placenta, and its teratogenic effects are well documented, as seen in this neonate: nasal and midface hypoplasia; shortened limbs and digits; calcifications of the vertebral, humeral, and femoral epiphyses (stippled epiphysis); and, as shown by the MRI image, Dandy-Walker syndrome (superior displacement and hypoplasia of the superior vermis).

Unfractioned heparin/low molecular weight heparin is better suited… but is not good for reducing thomboembolic events if there is a mechanical heart valve

Question 26

Surface ectoderm

Answer 26

Epidermis, adenohypohysis (from rathke pouch), lens of eye, epithelial lining of oral cavity, sensory organs of ear, olfactor epithelium, below pectinate line in anal canal, parotid, sweat, mammary gland

Question 27

Prader Willi patient

Answer 27

Obese, intellectual disability hypogonadism

Question 28

Angelman

Answer 28

ataxia, seizures, inappropriate laughter

Question 29

The anterior neuropore closure occurs at approximately

Answer 29

day 24 and the posterior neuropore occurs at 28 days

Question 30

Ions in stereocillia hair

Answer 30

Mechanically gated potassium (K+) channels on the tips of stereocilia open when stretched, causing K+ influx and depolarization, triggering voltage-gated calcium (Ca2+) channels to open. This Ca2+ influx into the hair cell causes release of excitatory neurotransmitters from synaptic vesicles, which diffuse through the perilymph to spiral ganglia neurites.

Question 31

Presbycusis

Answer 31

Age-related degeneration of the organ of Corti (or presbycusis) is a type of sensorineural hearing loss that is part of the normal aging process. It is the most common cause of sensorineural hearing loss in the elderly and manifests with gradually worsening high-frequency hearing loss. The cause of presbycusis is degeneration of the proximal hair cells of the organ of Corti (depicted in the drawing), which lie in the proximal portion of the basilar membrane (farthest from the helicotrema or apex and closest to the ossicles and oval window). This is the thinnest and least compliant portion of the basilar membrane and is responsible for sensing high-frequency sounds.

Question 32

Osteogenesis Imperfecta

Answer 32

decreased production of collagen type 1 bone (skin tendon, dentin, fascia, cornea)

Brittle bone disease, COL1A1, COL1A2, AD, multiple fractures bone deformities, blue sclerae, translucent connective tissue over chordial veins, tooth abnormalities, conductive hearing loss (OSSICLE damage)

Glycosylation—glycosylation of pro-α-chain hydroxylysine residues and formation of procollagen via hydrogen and disulfide bonds (triple helix of 3 collagen α chains).

LENS 4 letters type 4 collagen

type 1 CORNY
Type 2 HUMOURS (vitrious)

Question 33

Menkes disease

Answer 33

Cross-linking—reinforcement of many staggered tropocollagen molecules by covalent lysine-hydroxylysine cross-linkage (by copper-containing lysyl oxidase) to make collagen fibrils. Problems with cross-linking 􏰂Menkes disease.

X-linked recessive connective tissue disease caused by impaired copper absorption and transport due to defective Menkes protein (ATP7A, vs ATP7B in Wilson disease). Low copper levels (vs high levels in Wilson disease). Leads to􏰄activity of lysyl oxidase (copper is a necessary cofactor)􏰂 defective collagen. Results in brittle, “kinky” hair, growth retardation, hypotonia,􏰃risk of cerebral aneurysms.

MENKE IS IN THE HEAD

hypotonia…. dead vibes… cerebral aneurysms, HAIR fucked, growth fucked like a dumbo

Question 34

is found in patients with Marfan syndrome, a connective tissue disorder that results from a genetic defect in fibrillin. Patients with Marfan syndrome do not have an increased risk for fractures.

Answer 34

Arachnodactyly

Question 35

Damage to the vestibulocochlear nerve (CN VIII)

Answer 35

would result in sensorineural hearing loss and potentially equilibrium disturbances. However, patients with cranial nerve VIII lesions have hearing loss in the high-frequency range and exhibit problems with speech discrimination.

Question 36

Damage to the cochlea

Answer 36

result in sensorineural hearing loss, but would not result in vertigo. Presbycusis, age-related sensorineural hearing loss from hair cell degeneration, is usually symmetric and tends to affect high-frequency sound perception.

Question 37

Ménière disease

Answer 37

idiopathic disorder that usually affects people between the ages of 30 and 60 and involves episodes of vertigo that typically last for hours. Nystagmus is present during the vertiginous attacks, but would not be seen on exam of this patient who is not acutely symptomatic at presentation. Patients also have fluctuating difficulty hearing that often progresses to permanent, low-frequency sensorineural hearing loss in both ears. Aural fullness and tinnitus are also associated.

Question 38

Craniopharyngioma

Answer 38

Rathke pouch, most common non glial brain tumor in children, mimodal age distribution,
arise from epithelial nest

Question 39

Tryptophan is the precursor for

Answer 39

Serotonin

Question 40

Anti Jo1 antibodies

Answer 40

histidyl tRNA synthetase and most commonly found in patients with idiopathic inflammatory myopathies, specifically polymyositis. Weakness in proximal muscles

GBS remember is antibodies is myelin and is ascending paralysis

Question 41

Lambert Eaton Myasthenic syndrome

Answer 41

antibodies against calcium channels in presynaptic nerve terminal.

Proximal muscle weakness, decreased deep tendon reflexes, post tetanic potentiation (that is why it improves with movement)

Question 42

Gamma motor neurons

Answer 42

Small diameter motor neurons that innervate the intrafusal muscle fiber within a muscle spindle. The motor neurons maintain intrafusal fiber tension during extrafusal muscle fiber contraction

Question 43

Extrafusal fibers

Answer 43

constitute bulk of the contracting muscle belly. They produce the muscle tension

Question 44

Golgi Tendon organs

Answer 44

they initiate inverse stretch reflex, in which muscle contraction is inhibited during excessive stretch. These receptors are found in the tendon near the muscle tendon junction. When a weight lifter drops weight the muscle contraction is inhibited by this reflex.

Question 45

Metabolic acidosis by TCA>

Answer 45

Yes, and overuse of diuretics causes metabolic alkalosis