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Neurosurgery Board Prep > SANS CNS Questions review > Flashcards

SANS CNS Questions review Flashcards

1
Q

Branches of the meningohypophyseal trunk

A

Inferior hypophyseal arteries
Dorsal meningeal artery
Medial tentorial artery of Bernasconi-Cassinari

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2
Q

Loss of heterozygosity 1p/19q is characteristic of

A

Oligodendroglioma

WHO grade 2

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3
Q

Most commonly seen genetic mutation in gemistocytic and fibrillary astrocytomas an mixed oligoastrocytomas

A

TP53 mutations

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4
Q

Loss of heterozygosity of 10q is most frequently seen in

A 
Primary and secondary GBM
Other frequent genetic abnormalities:
-EGFR amplification
-p16(INK4a) deletion
-PTEN mutations
-TP53 mutations (more common with secondary GBM)
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5
Q

Primary treatment for posterior fossa JPA

A

Gross total resection

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6
Q

Primary treatment for suprasellar JPA

A

Chemo and/or radiation
(Chemo: carboplatinum and vincristine)

Suprasellar JPAs cannot be excised due to resulting visual, hypothalamic, and endocrine morbidity

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7
Q

Pineal mass
Elevated alpha-fetoproteins

Three most likely dx?

A

Endodermal sinus tumor
Embryonal carcinoma
Immature teratoma

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8
Q

Pineal mass
Elevated beta-hCG

Two most likely dx?

A

Choriocarcinoma

Mixed germ cell tumors

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9
Q

Best treatment for germinoma?

A

Intensity-modulated radiation therapy

  • Pure germinomas are commonly highly radiosensitive
  • Often treated without surgical rsxn
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10
Q

4wk old presents s/p seizure. CTH demonstrates calcified subependymal nodules and physical exam demonstrated hypopigmented macules. Dx? Next step?

A

Tuberous sclerosis
-Echocardiography to screen for rhabdomyomas

Epilepsy beginning in infancy as infantile spasms is a common presentation of TS

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11
Q
  • Autosomal dominant
  • Facial angiofibromata
  • Shagreen patches
  • Ungual fibromas
  • Retinal astrocytomas
  • Renal cysts
  • Renal angiomyolipomas
  • Pulmonary lymphangioleiomyomatosis
  • Hamartomatous rectal polyps
A

Tuberous Sclerosis

-Chromosomes 9 and 16

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12
Q

Dynamic T2-weighted MR imaging is performed to distinguish tumefactive demyelination versus glioma. What radiographic feature is most useful in establishing the likely diagnosis?

A

Demonstration of veins coursing through the lesion

-No angiogenesis exists in TDL

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13
Q

Manifestations (sodium level) of a classic triphasic response following transsphenoidal resection of a large craniopharyngioma are?

A

1) Hypernatremia
2) Normo or hyponatremia
3) Hypernatremia

1) Initial symptomatic DI s/p surgery (24hrs)
2) Inappropriate vasopressin secretion (4-5 days)
3) DI (up to 2 wks later)

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14
Q

75yo F with a fourth ventricular mass.

Histopathology shows nests of small blue cells amid a pink fibrillary matrix

Dx? MRI characteristics?

A

Subependymoma

-Iso/hypo intense on T1 and ABSENT contrast enhancement

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15
Q

A 32 year-old male has intractable seizures localized to the left language-dominant supplementary motor area. What neurological deficits will most likely result following surgical resection?

A
  • Temporary mutism
  • Contralateral paresis
  • Neglect

Transient - resolve within days - weeks s/p surgery

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16
Q

Anomia and finger agnosis are characteristic of which syndrome?
Location of lesion/damage?

A

Gerstmann’s syndrome

-Dominant parietal damage

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17
Q

Alexia without agraphia

Most likely location of lesion/damage?

A

Posterior corpus callosum damage

-Disconnection syndrome

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18
Q

Lesion in CPA

  • Hypointense on T1
  • iso/hyperintense on T2
  • Restricted diffusion on DWI
  • minimal/no contrast enhancement
A

Dermoid cyst

-Epidermoid cyst is similar but usually hyperintense on T1 due to cholesterol components

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19
Q

Mitochondrial disorder that causes slowly progressive ptosis and ophthalmoplegia

A

Kearns-Sayre syndrome

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20
Q

55yo p/w

  • Severe HA
  • Acute vision loss
  • B/L ptosis
  • Complete ophthalmoplegia of both eyes

Most likely dx?

A

Pituitary apoplexy

-Involvement of optic apparatus, possible cavernous sinus involvement (ophthalmoplegia and ptosis)

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21
Q

ETV is performed through which structure?

A

Tuber cinereum

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22
Q

What do the three standard peaks in a typical MRI spect measure?

  • NAA
  • Creatine
  • Choline
A
  • NAA: Neuronal viability
  • Creratine: Internal marker or reference
  • Choline: marker of cellular turnover (involved in synthesis of phospholipids)

Other markers:

  • Glutamine/glutamate: Astrocyte markers (may be altered in setting of neuronal damage)
  • Myoinositol: Astrocyte marker elevated in Alzheimer disease
  • Lipids and lactate: elevated in inflammatory processes and necrotic tissue
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23
Q

75yo M is dx with GBM.

-Which genetic abnormality would MOST likely be found in this tumor?

A

EGFR

Primary GBM account for vast majority of cases in the elderly
-Amplification and overexpression of EGFR gene has been reported in majority of primary GBM

vs. TP53 mutation in secondary GBM

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24
Q

Parkinson’s triangle boundaries in the cavernous sinus (infratrochlear triangle)

A
  • Trochlear nerve
  • Ophthalmic division V1 of the trigeminal nerve

The oculomotor nerve is located above

Maxillary division V3 is located below

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25
Q

Prolactin level that determines prolactinomas vs not

A

Prolactinomas: > 150 ng/ml

<150 ng/ml (unlikely)

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26
Q

Most likely complication after pituitary adenoma radiosurgery?

A

Anterior pituitary insufficiency

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27
Q

Maximum radiation dose to the optic apparatus

A

10-12 Gy

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28
Q

Most likely complication after vestibular schwannoma radiosurgery

A

Hearing loss

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29
Q

47yo who presents with confusion, agitation, fatigue, fever, hyponatremia, hypoglycemia, and hypotension s/p rsxn of R frontal met 3 weeks ago. Most likely diagnosis?

A

Steroid withdrawal syndrome/Addisonian crisis/Adrenal insufficiency

  • AMS
  • Fevers
  • Hyponatremia
  • Hypoglycemia
  • Hyperkalemia
  • Hypotension
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30
Q

Most likely vessel to be injured during a transsphenoidal?

A

Sphenopalatine artery

  • Terminal branch of maxillary artery
  • 1-10% chance of injury
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31
Q

Patient presents with new onset of right facial droop and associated with horizontal diplopia. MRI demonstrates a 4th ventricular lesion. Which anatomy structure is affected causing the diplopia?

A

Abducens nucleus

  • At the level of the facial colliculus, where facial fibers loop around the abducens nucleus
  • Lesion at the abducens nucleus will result in ipsilateral conjugate gaze palsy
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32
Q

Patient presents with bilaterally preserved abduction without conjugate adduction of the contralateral eye but preserved convergence. What structure is affected?

A

MLF

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33
Q

Pt presents with rotatory diplopia that is compensated by tilting the head to the right. What structure is affected?

A

(Left) trochlear nucleus
-Disease at this level (at the midbrain) would result in rotatory diplopia compensated with head tilt away from affected side

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34
Q

Sudden onset ear pain, facial paralysis hearing loss, and vertigo. No mass lesion seen on MRI. Dx?

A

Zoster Oticus / Ramsay-Hunt syndrome

-MRI may demonstrate a linear enhancement in the IAC with extension into the tympanic segment of the facial nerve

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35
Q

What is the primary oncogenetic function of Merlin?

A

Tumor suppressor
-Suppressive role of the Ras-Raf-Mek system

Merlin is an NF2 gene transcription product

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36
Q

During awake crani, which tumor location displays a significantly increased risk of intraop seizure?

A

Frontal

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37
Q

What is the approximate risk of cognitive decline with stereotactic radiosurgery (SRS) plus whole brain radiation therapy (WBRT) for the treatment of metastatic disease

A

50%

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38
Q

Disorder with distinctive EEG pattern of periodic bursts of stereotype slow-wave and sharp-wave complexes that occur in 3 to 15 second intervals

A

Subacute sclerosing panencephalitis

-Progressive post measles panencephalitis

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39
Q

Opening the choroidal fissure in the atrium will lead to

A

Quadrigeminal cistern

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40
Q

What tumor histology is associated with highest risk of cerebral hemorrhage in patients receiving therapeutic anticoagulation?

A
  • Glioma (4 fold higher)

- Mets: no significant increase

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41
Q

What clinical factor portends a favorable local control rate after undergoing stereotactic radiosurgery?

A

Tumor treatment volume

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42
Q

Histopathology:

  • Uniformly cellular
  • Numerous, ectatic or compressed, thin walled branching vessels with gaping sinusoidal spaces (Staghorn configuration)
A

Hemangiopericytoma

  • Aggressive CNS tumor
  • Invade locally and metastasize
  • High rate of recurrence
  • 20-50% metastasize to lungs, liver, or bone
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43
Q

Post-operative management of GBM

A

Stupp protocol

  • Adjuvant chemotherapy TMZ
  • and fractionated radiotherapy ~60Gy
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44
Q

18yo girl presents with progressive HAs. Pt discovered to have SEGA. Best treatment option?

A

Everolimus

  • mTOR inhibitor
  • mTOR pathway has ben implicated
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45
Q

Which laboratory measurement is most specific for Cushing’s syndrome?

A

24hr urinary free cortisol

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46
Q

The addition of which chemotherapeutic agent to radiation therapy has been shown to increase overall survival in patients with newly diagnosed anaplastic oligodendroglioma?

A

PCV

  • Procarbazine
  • Lomustine (CCNU)
  • Vincristine
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47
Q

Paragangliomas of the jugular foramen derive from what cell type?

A

Chromaffin cells of neuro-ectodermal origin

  • Location: Jugular fossa, tympanic cavity, carotid body, larynx, and aorticopulm arch
  • Sporadic, but 25% familial inheritance pattern
  • about 3% of cranial base paragangliomas secrete catecholamines
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48
Q

What intracranial lesion is derived from epithelial cells of ectodermal origin from the stomodeum

A

Rathke’s pouch

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49
Q

What mass lesion do these cell types give rise to?

  • Meningoepithelial cells:
  • Cerebellar stem cells:
  • Germ cells taht migrated aberrantly
A
  • Meningoepithelial cells: Meningioma
  • Cerebellar stem cells: Medulloblastoma
  • Germ cells that migrated aberrantly: Germinoma
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50
Q

Best management strategy for patient with esthesioneuroblastoma without evidence of metastatic disease?

A

Surgical rsxn then conformal radiation tx to tumor bed

- plus chemo if evidence of metastases

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51
Q

What extent of surgical resection of low grade gliomas contributes to a survival benefit?

A

75-100%

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52
Q

During awake crani, which tumor location displays a significantly increased risk of intraop seizure?

A

Frontal

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53
Q

What is the approximate risk of cognitive decline with stereotactic radiosurgery (SRS) plus whole brain radiation therapy (WBRT) for the treatment of metastatic disease

A

50%

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54
Q

Disorder with distinctive EEG pattern of periodic bursts of stereotype slow-wave and sharp-wave complexes that occur in 3 to 15 second intervals

A

Subacute sclerosing panencephalitis

-Progressive post measles panencephalitis

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55
Q

Central American patient p/w new onset seizures. MRI demonstrates multiple rim-enhancing cysts throughout the brain. Most likely dx?

A

Taenia solium

  • Neurocysticercosis
  • Southwest US, Central and South America
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56
Q

What tumor histology is associated with highest risk of cerebral hemorrhage in patients receiving therapeutic anticoagulation?

A
  • Glioma (4 fold higher)

- Mets: no significant increase

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57
Q

What clinical factor portends a favorable local control rate after undergoing stereotactic radiosurgery?

A

Tumor treatment volume

How well did you know this?
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58
Q

Histopathology:

  • Uniformly cellular
  • Numerous, ectatic or compressed, thin walled branching vessels with gaping sinusoidal spaces (Staghorn configuration)
A

Hemangiopericytoma

  • Aggressive CNS tumor
  • Invade locally and metastasize
  • High rate of recurrence
  • 20-50% metastasize to lungs, liver, or bone
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59
Q

Post-operative management of GBM

A

Stupp protocol

  • Adjuvant chemotherapy TMZ
  • and fractionated radiotherapy ~60Gy
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60
Q

18yo girl presents with progressive HAs. Pt discovered to have SEGA. Best treatment option?

A

Everolimus

  • mTOR inhibitor
  • mTOR pathway has ben implicated
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61
Q

Which laboratory measurement is most specific for Cushing’s syndrome?

A

24hr urinary free cortisol

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62
Q

MOA of clopidogrel

A

Clopidogrel = Plavix

-Inhibits adenosine diphosphate (ADP) chemoreceptor, P2Y

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63
Q

Heparin bolus and activated clotting time in endovascular treatment

A
  • Initial 100u/Kg of heparin bolus

- Maintenance of activated clotting time 2x patient’s baseline

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64
Q

Pretreatment before endovascular stenting

A

ASA81
Clopidogrel 75mg
-10 days prior to procedure

If emergent:
Load with
-600mg of clopidogrel
-50u/Kg of heparin bolus (following deployment of first coil)

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65
Q

MOA of aminocaproic acid

A

Inhibitor of plasminogen

  • (plasminogen activates plasmin to break down thrombin and activate fibrinolysis)
  • ACA is an antifibrinolytic that helps stabilize clotting
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66
Q

67yo M who p/w worsening parasthesias of the ipsilateral face and contralateral extremity. The symptoms are provoked with head turning. Where is the most likely site of vascular compression in this patient?

A

Dominant vertebral artery

  • Bow Hunter syndrome: Vertebrobasilar insufficiency from intermittent compression
  • MC dominant vert compression at C1/2 with head rotation
  • Secondary to mechanical narrowing, bone spurs, tumors fibrous bands, infection, or trauma
  • Symptoms develop when head is turned to the contralateral side
  • Usually isolated posterior circulation with absent/hypoplastic PComms
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67
Q

77-year-old man with history of poorly controlled hypertension awakes with bilateral vision loss. Examination reveals no light perception in both eyes, normal pupillary responses without relative afferent defect, no gaze palsy and normal optic discs. He is slightly confused and language is fluent. However, during your examination, he becomes globally aphasic with gaze deviation to the left and tonic stiffening of his left arm for 45 seconds. Most likely Dx?

A

PRES (Posterior reversible encephalopathy syndrome)

  • Subacute painless b/l vision loss
  • HA
  • AMS
  • Aphasia
  • Seizures
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68
Q

Pathophysiology of PRES

A

-Increased vascular permeability of the posterior circulation

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69
Q

Syndrome:

  • Simultanagnosia
  • Optic ataxia
  • Ocular apraxia
A

Balint syndrome

  • seen in bi-parietal lesions
  • Neurodegenerative disorders (Alzheimer’s and posterior cortical atrophy)
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70
Q

A 43-year old patient experienced a visual field defect and hemisensory loss after clipping of a PCA aneurysm. What is the most likely site of injury?

A

Lateral posterior choroidal artery

Posterior choroidal artery infarcts affect

  • LGN
  • Pulvinar
  • Posterior thalamus
  • Hippocampus and parahippocampal gyrus

Resulting in: VF defects, hemisensory disturbances, psych dysfunctions, abnormal movements, rarely hemiparesis and eye movement disorders

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71
Q

Occlusion of which cerebral artery can cause spastic paraparesis and contralateral sensory loss along with urinary incontinence and gait apraxia?

A

Recurrent artery of Heubner

  • Anteromedial section of the curate neucleus
  • Anteroinferior section of interncal capsule
  • parts of putamen and septal nuclei
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72
Q

Most common site for hypertensive hemorrhage

A

Putamen/BG - 50%
Thalamus/pons - 10-15%
Cerebellum - 10%

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73
Q

CHADS VASC score and anticoagulation recs for stroke prevention

A

CHADS2 score 0 or 1: antiplatelet
CHADS2 score >= 2: Warfarin/AC

Congestive heart failure (1)
Hypertension (1)
Age 75 or older (1)
Diabetes (1)
S2 - Prev stroke or TIA (2)
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74
Q

Symptoms of AICA occlusion

A

Lateral pontine syndrome

  • Contralateral hemibody loss of pain and temp (lateral spinothalamic tract)
  • Ipsilateral upper and lower facial paralysis (facial nucleus/nerve)
  • Ipsilateral facial loss of pain and temp (spinal trigeminal nucleus and tracT)
  • Nystagmus
  • N/V/Vertigo (vestibular nuclei)
  • Ipsilateral hearing loss (cochlear nuclei)
  • Ipsilateral limb and gait ataxia (inferior cerebellar peduncle)
  • Ipsilateral Horner’s (descending sympathetic tract)
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75
Q

CNIII palsy from aneurysm compression vs diabetic neuropathy.

A
  • Diabetic neuropathy: pupil sparing CNIII palsy
  • Aneurysmal compression: affects external fibers first
  • Control of ocular muscles via central fibers within nerve (vascular issues/DM)
  • Miosis controlled by parasympathetic fibers via external fibers (affected by compression)
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76
Q

Most common aneurysm to cause CNIII palsy

A

Pcomm anerurysm

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77
Q

Most common location of cranial dAVF

A 
Transverse sinus (63%) 
-at Trans-sig junction
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78
Q

What is the most significant risk factor for normal perfusion pressure breakthrough after AVM resection?

A

Large size

  • characterized by post-op swelling or hemorrhage
  • d/t loss of autoregulation in vessels of surrounding barin parenchyma
  • Staged embolization and premedication with propranolol 20mg PO QID 3d before surgery can decrease risk
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79
Q

Mutation of which gene has been linked to cerebral amyloid angiopathy?

A

Presenilin (PS)

  • amyloid precursor protein
  • Amyloid angiopathy occurs due to amyloid AB deposits in cortical capillaries
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80
Q

KRIT-1 gene mutations are related to…

A

familial cerebral cavernous malformations

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81
Q

IV tPA should be administered within what time frame after symptom onset?

A

4.5hrs
tPA dosing: 0.9mg/kg (max dose 90mg)

IA tPA if onset <6hrs

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82
Q

MRCLEAN trial

-What did it show?

A

The MRCLEAN trial showed that in patients with acute ischemic stroke caused by a proximal intracranial occlusion of the anterior circulation, intraarterial treatment performed within 6 hours after stroke onset was effective and safe.

  • (IV tPA and Intra-arterial tx vs IV tPA alone)
  • Absolute difference of 13.5% in rate of functional independence (32.6% vs 19.1%) in favor of intervention
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83
Q

symptomatic patients with carotid artery stenosis (< 50%) should receive…

A

Best medical tx

  • antiplatelet therapy
  • statins
  • tx of htn, DM, diet correction, obesity
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84
Q

In patients who receive medical therapy alone for asymptomatic carotid stenosis of 60-99%, what is the 5-year risk of stroke or death?

A

Asymptomatic stenosis

  • Medical management: 11%
  • Both medical and CEA: 5.1%
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85
Q

Stroke and cardiovascular complications in CEA vs CAS

A

CREST trial

  • Periprocedural rate of stroke was higher with CAS -Rate of MI was higher with CEA
  • CEA and CAS all together had similar rates of periprocedural complications
  • Quality of life analyses among survivors at 1yr indicated stroke had a greater adverse effect than did the MI
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86
Q

According to the International Study of Unruptured Intracranial Aneurysms (ISUIA), what is the 5yr risk of rupture of cavernous carotid artery aneurysms (<7mm) ina patient with h/o prev aneurysmal SAH

A

0% in pts with or without a h/o aneurysmal rupture

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87
Q

What factor significantly impacts the rerupture risk of a previously treated ruptured intracranial aneurysm?

A

Raymond–Roy Occlusion Classification (RROC; also known as the Montreal Scale, Modified Montreal Scale, or the Raymond Montreal Scale)

  • Class 1: complete obliteration
  • Class II: residual neck
  • Class III: residual aneurysm

Class III aneurysms have a higher propensity to remain incompletely occluded and potentially rebleed

CARAT study - rerupture risk

  • Complete occlusion: 1.1%
  • 91-99% occl: 2.9%
  • 70-90% occl: 5.9%
  • <70% occl: 17.6%
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88
Q

Which anatomic feature best predicts risk of intracranial hemorrhage related to dural arteriovenous fistulae (DAVF):

A

-Presence of cortical venous drainage

Borden and Cognard classifications

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89
Q

Borden classification for dAVF

A

1) Venous drainage directly into venous sinus or meningeal veins
2) Venous drainage into dural venous sinus with Cortical venous reflux
3) Venous drainage directly into subarachnoid veins (CVR) only

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90
Q

Cognard classification for dAVF

A

1: confined to sinus, antegrade flow, no CVR
2a: confined to sinus, retrograde flow (reflux) into sinus, no CVR
2b: drains into sinus with reflux into cortical veins. Antegrade flow

2a+b: drains into sinus with reflux into cortical veins. Retrograde flow

3: Drains directly into cortical veins (40% hemorrhage)
4: Drains directly into cortical veins with venous ectasia (65% hemorrhage)
5. Spinal perimedullary venous drainage - progressive myelopathy

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91
Q

5yr cumulative rupture rates according to size and location of unruptured aneurysm

A

<7mm (if h/o prev SAH from diff aneurysm)

  • Cavernous: 0% (0%)
  • Anterior circ: 0% (1.5%)
  • Posterior circ: 2.5% (3.4%)

7-12mm

  • Cav: 0%
  • Ant: 2.6%
  • Post: 14.5%

13-24mm

  • Cav: 3%
  • Ant: 14.5%
  • Post: 18.4%

> 25mm

  • Cav: 6.4%
  • Ant: 40%
  • Post: 50%
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92
Q

Indications for acute SDH evacuation

A

> 10mm or MLS >5mm (regardless of GCS)

If doesn’t meet above criteria, surgery if GCS drops by > 2points from injury to admission and/or pupils are asymmetric or fixed/dilated

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93
Q

Lindegaard ratio

A

< 3: normal
3-6: mild-moderate
> 6: severe

  • Very specific for diagnosis of cerebral vasospasm
  • 60% sensitive
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94
Q

Most common presenting symptoms in newborns with vein of Galen malformations?

A
  • Congestive heart failure (47%)
  • Increased ICPs/hydro (40%)
  • Cranial bruit (25%)
  • Focal neurological deficit (16%)
  • Seizures (11%)
  • Hemorrhage (10%)
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95
Q

Vascular lesion with characteristic “Caput medusa” or sunburst appearance on angiography

A

DVA/venous angiomas

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96
Q

Brain bx shows deposition of beta amyloid protein appearing as birefringent “apple-green” under polarized light when stained with Congo red

A

Cerebral amyloid angiopathy

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97
Q

Most common presentation of a vein of Galen in an adolescent patient?

A

headache or seizure

Older children and adults with vein of Galen malformations usually have low-flow fistulae

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98
Q

Vein of Galen malformation classifications

A

Yasargil and lasjaunias

look it up

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99
Q

Radiographic criteria found in hydrocephalus

A
  • Sella erosion
  • Frontal horn’ percent of brain width is > 50%
  • Temporal horns’ width is more than 2mm
  • AP view shows disproportion of the ventricle size and sulci
  • Bowing of third ventricle
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100
Q

Best management of an ethmoidal dural AVF with cortical venous drainage

A

Surgery

-vs. endovascular embolization: presence of retinal artery supply from the ophthalmic arteries –> Risk to vision

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101
Q

Pt presents with following. Most likely dx?

  • Double vision
  • Conjunctival congestion
  • Decreased visual acuity
  • Retro-orbital bruit
  • Increased intraocular pressure
A

Carotid-cavernous fistula

  • Indication to tx: Proptosis, visual loss, abducens palsy, intractable bruit, severely elevated intraocular pressure, cortical venous filling
  • Best tx: Transvenous embolization via superior ophthalmic vein or petrosal sinus
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102
Q

Most common presentation of an adult patient with moyamoya

A

-Intracranial hemorrhage

Children: recurrent infarcts or TIAs. Also may have fine involuntary movements of the extremities and slowly progressive mental impairment

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103
Q

Classification of moyamoya disease

-Suzuki and Takaku

A
  • Stage 1: Stenosis of carotid artery at suprasellar portion (usually b/l)
  • 2: Moyamoya vessels begin to develop at base of the brain
  • 3: Moyamoya vessels are prominent as major trunks in anterior circulation become severely occluded
  • 4: PCAs occluded, moyamoya vessels begin to diminish and collateral pathways from extracranial circulation develop
  • 5: Moyamoya vessels are diminishing and extracranial circulation progress
  • 6: Moyamoya vessels and the major cerebral arteries completely disappear. The cerebral hemisphere receives blood through the abnormal extracranial-intracranial anastomosis
104
Q

Aside from b/l ICA occlusions, what is a typical angiographic finding in moyamoya disease?

A

Diffuse hypertrophy of the lenticulostriate arteries

-This produces the “puff of smoke” radiograhic finding

105
Q

Spetzler-Martin grade and associated morbidity

A
  • Grade1: 0%
  • Grade2: 5% minor deficit, 0% major deficit
  • Grade3: 12% minor, 4% major
  • Grade4: 20% minor, 7% major
  • Grade5: 19% minor, 12% major
106
Q

According to NASCET, what is the rate of perioperative permanent disabling stroke and death related to CEA

A
  • Rate of permanently disabling stroke and death: < 2%

- Overall rate of perioperative stroke and death: < 6.5%

107
Q

Mortality rate of malignant MCA infarction with malignant edema managed with intensive non-operative care

A

80% mortality

108
Q

Mortality rate of acute basilar artery occlusion

A

90%

109
Q

Which genetic disorder has been associated with moyamoya disease?

A

Trisomy 21 - Down’s syndrome

-Also associated: sickle cell disease

110
Q

Indication for fusing asymptomatic down’s syndrome child with atlantoaxial instability

A

1cm subluxation

111
Q

Sagittal division of the spinal cord is called?

-Associated findings?

A

-Diastematomyelia/split cord malformation

  • Cutaneous stigmata overlying the spine: nevi, lipomas, dimples, and hemangiomas
  • Prominent patch of hypertrichosis with stiff, dark hairs
  • Orthopedic problems of the feet (clubfoot)
  • Fusion with contralateral adjacent lamina (intersegmental laminar fusion): pathognomonic
  • Type 1: arachnoid and dura are split into 2
  • Type 2: Single dural sac
112
Q

Pt develops severe left shoulder pain after heavy manual labor. 10 days later develops rapidly progressive weakness of his left shoulder girdle muscles and biceps. Normal sensory exam. MRI of C-spine is unremarkable. Most likely dx?

A

Parsonage-Turner syndrome (brachial neuritis, brachial plexopathy, or neuralgic amyotrophy)

  • Autoimmune disorder triggered by a stressor
  • Painful prodrome followed by weakness in affected extremity
113
Q

For which type of lumbar fusion is the use of BMP (bone morphogenetic protein) FDA approved?

A

Anterior lumbar interbody fusion procedures

-Use of BMP was associated with fusion rates and clinical outcomes at least as good as autograft

114
Q

ASIA injury grading

A
  • ASIA A: complete
  • B: incomplete, sensory function is spared without motor function except preservation of sacral segments
  • C: Motor function preserved, more than half of key muscles with strength < 3/5
  • D: Motor function preserved, more than half of key muscles with strengh > 3/5
  • E: Normal strength
115
Q

When placing bicortical sacral pedicle screws, which structure is at most risk with a laterally placed screw that perforates the anterior cortex?

Inferiorly placed screw?

A

Laterally placed screw: L5 nerve root
-L5 nerve drapes over the sacrum

Inferiorly placed screw: S1

116
Q

Rate of spinal cord re-tethering after surgical untethering in pts with myelomeningocele

A

15-30%

  • Repeat untetering procedures are most successful at alleviating back pain (80% of pts report improvement)
  • Motor loss and bladder dysfunction improve in ~50%
117
Q

Most common complication in an ACDF?

A

Dysphagia (18%)

Others

  • Hoarseness (4.9%)
  • New neurologic deficit (2%)
  • Recurrent laryngeal nerve injuries (1-2%)
  • Esophageal injury, Horner’s, vertebral artery injury, thoracic duct injury (1%)
118
Q

Child with back pain. Imaging findings:

  • three consecutive wedged vertebrae
  • Irregular vertebral endplates
  • Apparent loss of disc height
  • Thoracic kyphosis that does not correct with supine positioning

Dx?

A

Scheuermann’s disease

  • Spinal disorder of children featuring aseptic necrosis of the vertebral body ring apophysis (development of vertebral endplate and ring apophysis is altered)
  • Affects lumbar or thoracic spine
  • Managed conservatively
  • Surgery considered for painful kyphosis
119
Q

Chiari malformation types and features

A

Chiari 0
-Syrinx without cerebellar tonsillar or brain stem descent

Chiari 1

  • Most common
  • Peg-like cerebellar tonsils displaced through foramen magnum
  • Syringomyelia commonly associated (65%)

Chiari 1.5
-Caudal descent of cerebellar tonsils and brain stem

Chiari 2

  • Displacement of the medulla, fourth ventricle, and cerebellar vermis through FM
  • Usually associated with lumbosacral myelomeningocele
  • Hydrocephalus commonly associated (70%)

Chiari 3

  • similar to chiari 2
  • with an occipital and/or high cervical encephalocele

Chiari 4

  • Severe cerebellar hypoplasia without displacement of cerebellum through foramen magnum
  • “Pigeon breast” deformity is a characteristic finding

Chiari 5

  • Absent cerebellum
  • Herniation of the occipital lobe through the FM
120
Q

-Flowing calcification or ossification of at least four contiguous vertebral bodies
-preservation of disc height
-Absence of sclerosis or fusion of SI joints
Dx?

A

DISH (Diffuse idiopathic skeletal hyperostosis)

vs. Ankylosing spondylitis
- Syndesmophytes and calcification of the annulus fibrosis and nucleus pulposus in AS

121
Q

Brown-Sequard presentation

A
  • Ipsilateral motor paralysis
  • Ipsilateral loss of proprioception and vibratory sensation
  • Contralateral loss of pain and temperature sensation inferior to lesion
122
Q

What factors predict nonunion in dens fractures

A
  • Comminution of the dens
  • Displacement >5mm
  • Advancing age
  • Posterior displaced fractures
  • Pts with neurologic deficits
123
Q

What is the most common adverse event following laminoplasty for cervical spondylotic myelopathy

A

Loss of cervical range of motion (30-50%)

124
Q

Vertebral artery injury during C1-2 transarticular screw placement is most likely to result when the screw is misplaced in which direction?

A

Caudal/inferior

125
Q

Upper and lower motor neuron dysfunction with progression. Most likely dx?

A

ALS

  • Most common neurodegenerative disease of the motor neuron system
  • Loss of anterior horn cells as well as corticospinal tract neurons
126
Q

Most common cause of perioperative visual loss in patients undergoing spinal fusion?

A

Anterior and/or posterior ischemic optic neuropathy (89% of cases)

127
Q

Where does artery of Adamkiewicz arise from and what does it supply?

A
  • Commonly arises at T10 on the left side but may arise anywhere from T7-L4
  • Supplies the anterior 2/3 of spinal cord
128
Q

What is a contraindication to performing a cervical laminoplasty

A

Cervical kyphosis

129
Q

What characteristic imaging finding on MRI would best differentiate neuromyelitis optica from multiple sclerosis

A

Longitudinally-extensive spinal cord lesion
-Most consistent with neuromyelitis optica (NMO)/Devic disease

NMO/Devic disease

  • Bilateral optic neuritis
  • Longitudinally-extensive transverse myelitis
  • Antibodies to aquaporin 4 water channel = NMO antibodies
130
Q

Which characteristics of a pedicle screw increases pullout strength

A
  • Increasing the interthread distance
  • Decreasing minor-major diameter ration
  • Increasing the angle (triangulation)
  • Expanding the screw tip
  • Increasing screw length and diameter
  • Bicortical purchase
131
Q

Rule of Spence

  • What is it?
  • Measurement
  • Integrity of which structure does it assess
A

Rule of Spence

  • Sum of overhang of C1 lateral masses over C2 (both left and right)
  • Equal to or greater than 7mm = disruption of transverse ligament
  • Assesses integrity of transverse ligament
132
Q

Occipital condyle fracture types

A

Type 1: non-displaced linear condyle fracture

2: Extension of basilar skull fx
3: Condyle fracture with alar ligament avulsion

133
Q

Measurements to evaluate atlanto-occipital dislocation

A

BAI-BDI (basion-axial-interval - basion-dental-interval)

BAI: distance from basion to line drawn along posterior wall of C2 vertebral body.

  • Distance of > +12mm: Type 1 injury (anterior injury)
  • Distance of > -4mm: Type 3 injury (posterior injury)

BDI: Basion dental interval > 12mm is abnormal = consistent with a Type 2 Atlanto-occipital dislocation (longitudinal injury)

134
Q

Atlanto-occpital dislocation types

A

Traynelis has classified injuries to 3 majory types + others

Type 1: Anterior
Type 2: Longitudinal
Type 3: Posterior
Other types: lateral, rotational, multidirectional

135
Q

For how long are EMG studies normal following nerve injury

A

2 weeks

136
Q

Nerve damage after GSW, best management?

A

Monitor for 3-6months.

  • Surgical exploration and possible graft repair if no recovery
  • 70% of these injuries recover without surgical intervention

vs. sharp laceration of nerve = immediate surgical intervention

137
Q

Patient with multiple stab wounds to upper extremity. Pt has isolated deficits in finger extension and ulnar wrist extension. Most likely level of injury?

A

PIN (Posterior interosseous nerve)

  • Isolated motor (No sensory component)
  • Extensor carpi ulnaris and finger extensors
138
Q

what supplies sensation to base of dorsal thumb, index, and middle fingers, and anatomy snuffbox?

A

Superficial radial sensory nerve (radial nerve)

139
Q

What nerve provides sensation to the medial forearm?

A

Medial antebrachial cutaneous nerve
-Arises from the medial cord

(Lateral antebrachial cutaneous nerve arises from musculocutaneous nerve)

140
Q

Young girl with multiple nodular areas running along the cervical nerve roots and brachial plexus on MRI. Examinations of her eyes also shows iris hamartomas. Dx?

A

NF1 (has to have 2 or more of following)

  • Iris hamartomas = Lisch nodules (need 2 or more)
  • 2 or more neurofibromas of any type or one plexiform neurofibroma
  • Freckling in axillary or inguinal region
  • Optic glioma
  • Characteristic osseous lesion (sphenoid wing dysplasia or thinning of long bone cortex)
  • First degree relative with NF1
  • Mutations/deletions of neurofibromin gene (tumor suppresor/negatively regulates ras oncogene)
  • Chromosome 17
  • Autosomal dominant
141
Q

Examination of which movement is most helpful in distinguishing an L4 radiculopathy from a femoral neuropathy?

A

Hip flexion

  • L4 radiculopathy: Affects knee extension. Not hip flexion
  • Femoral neuropathy: Affects both KE and HF (Except if neuropathy occurs at level of inguinal ligament, HF may be spared and mimic L4 radiculopathy more closely)
142
Q

What is the nerve that is compressed in tarsal tunnel syndrome?

A

Tibial nerve

  • Compression of tibial nerve by lancinate ligament on medial surface of the ankle, posterior to the medial malleolus
  • Results in plantar foot pain (spares the heel)
  • Intrinsic muscles of the foot can be affected in severe cases
143
Q

Pain in lateral heel, lateral ankle, and lateral foot occurs from which nerve compression?

A

Sural nerve

144
Q

Pain along the anteromedial leg from the knee to the ankle occurs from which nerve compression?

A

Saphenous nerve

145
Q

Pain along the dorsum of the foot occurs from which nerve compression?

A

Superficial peroneal nerve

146
Q

Pain along the dorsal webspace between first and second toes occurs from which nerve compression?

A

Deep peroneal nerve

147
Q

One of the best ways to ascertain that ulnar nerve compression is distal (at the wrist) and not proximal (at elbow)

A

Test ulnar sensation on the dorsal surface of the hand
-Dorsal sensory branch of the ulnar nerve leaves the ulnar nerve before the wrist

Wrist compression: Guyon’s canal syndrome

  • Does not affect dorsal ulnar sensation
  • Does affect palmar and terminal digital sensation
148
Q

Which nerve derives its motor component primarily from the inferior trunk and its sensory component predominantly from the lateral cord?

A

Median nerve

  • Motor input mostly from medial cord (C8-T1 nerve roots, inferior trunk)
  • Sensory input from the lateral cord (C6 nerve root)
149
Q

Pt presents with slowly progressive pain and weakness in right hand and forearm. Reveals weakness of wrist extension with radial deviation, finger extension, and thumb extension. Deltoid, bicep, tricep, and brachioradialis strength is normal. Sensory exam is normal. Dx?/What nerve is affected? most likely where?

A

Posterior interosseous neuropathy
-PIN/distal radial nerve
-Sensation is preserved
-Compression: arcade of Frohse (most likely)
Other sites: fibrous muscle bands (brachialis, brachioradialis, supinator, ECRB), leash of Henry (set of vascular branches)

150
Q

Patient presents with severe atrophy and weakness of thenar, hypothenar, lumbrical, and interossei muscles. Also has hypesthesia in medial aspect of forearm and hand. Sensation in median nerve distribtuino is spared. Most likely dx?

A

Thoracic outlet syndrome

  • Gilliatt-Sumner hand: severe atrophy and weakness of thenar, hypothenar, lumbrical, and interossei muscles accompanied by hypesthesia
  • Entrapment of lower trunk of brachial plexus

Sites of compression:

  • Interscalenic triangle (formed by anterior and middle scalene muscles and first rib)
  • Soft tissue abnormalities (ervical band/sibson’s fascia)
  • Congenital anatomic variants (cervical rib or hypertrophic C7 TP)

Tx: Surgical decompression

151
Q

L5 radiculopathy vs peroneal neuropathy

A

L5:
-Isolated weakness of DF and ankle INversion

Peroneal:
-Isolated weakness of DF and ankle Eversion

152
Q

Sensory and motor function in preganglionic injury (high thoracic)?
On EMG?

A

Preganglionic injury occurs between DRG and spinal cord (at spinal rootlets)

  • Sensory loss in arm but normal SSEP (preserved DRG connection distally)
  • Motor loss and abnormal motor nerve conduction studies
153
Q

Anterior interosseous nerve innervates which muscles

A

AIN is a branch of the median nerve and innervates

  • Flexor pollicis longus
  • Flexor digitorum profundus 1 and 2
  • Pronator quadratus muscles

Entrapment –> problems flexing distal thumb and index finger

154
Q

What nerves arise from the posterior cord of the brachial plexus?

A

Axillary and Radial nerve

155
Q

Pt c/o pain along left medial knee and electrical shocks that shoot down the anteromedial left leg to the ankle.

Which nerve is affected? Most likely site?

A 
Saphenous nerve (sensory terminus of the femoral nerve)
-Adductor canal (MC site of entrapment). Saphenous nerve arises within the adductor canal in distal thigh
156
Q

Femoral nerve injury. Where is the injury in each?

  • Weakness includes hip flexion
  • Weakness of knee extension (not HF)
  • NO weakness, but sensory loss in anteromedial distal leg
A
  • HF weakness: Intraabdominal level
  • KE weakness: Just distal to inguinal ligament
  • Sensory loss only (saphenous nerve injury): Distal thigh or below
157
Q

Which nerve roots form the three trunks?

  • Upper
  • Middle
  • Lower
A
  • Upper: C5, C6
  • Middle: C7
  • Lower: C8, T1
158
Q

Which muscles are supplied by the musculocutaneous nerve?

A

MCN -arises from lateral cord of brachial plexus

  • Coracobrachialis
  • Biceps
  • Brachialis

-Sensory to lateral forearm (lateral antebrachial cutaneous nerve)

159
Q

Obturator nerve function

A

Obturator nerve arises from L2-4 nerve roots within the lumbosacral plexus

  • Sensory to hip joint and superomedial thigh
  • Motor to thigh adductors
160
Q

Which nerves provide innervation to arm supinators

A

Radial and MCN

  • Elbow straight: Supination by supinator
  • Elbow bend: Supination by supinator and biceps
161
Q

Function of suprascapular nerve

A

Shoulder abduction and external shoulder rotation

Supraspinatus and infraspinatus (respectively)

162
Q

Function of sural nerve?

A

Pure sensory nerve, supplies lateral ankle and foot sensation
-Arises from the medial and lateral sural cutaneous branches of the tibial and peroneal nerves (respectively)

163
Q

Best spinal level for electrode placement to achieve best stimulation coverage for chronic medically-refractory neuropathic pain of right leg and foot

A

T11 - for lower extremity and foot

T9 - for low back and lower extremity coverage

164
Q

Which artery makes pathological contact with trigeminal nerve in trigeminal neuralgia

A

Superior cerebellar artery - Trigeminal neuralgia

AICA - hemifacial spasm
PICA - glossopharyngeal neuralgia

165
Q

Type 1 vs Type 2 Complex regional pain syndrome (CRPS)

A

CRPS
-Posttraumatic neruopathic pain

Type 1: occurs without evidence of injury to a major peripheral nerve
Type 2: (causalgia). Occurs with injury to a specific nerve. Changes in involved limb may extend beyond the anatomic limits of that nerve

Symptoms: Allodynia, mechanical hyperalgesia, color changes, temperature, pseudomotor abnromalities, and motor disorders
Tx: Physical therapy, early use of involved limb

166
Q

Pt with debilitating paroxysmal, lancinating pain involving the base of the tongue and tonsillar region on the left side. Imaging is normal. Dx?

A

Glossopharyngeal neuralgia
-PICA is the most commonly implcated vessel

If caused by elongated styloid process: Eagle syndrome

167
Q

Geniculate neuralgia is associated with which nerve?

A

Nervus intermedius

168
Q

A patient with chronic lower extremity pain treated successfully for 6 years with an intrathecal morphine pump now presents to the Emergency Department several hours after getting a routine pump refill. He is lethargic, has pinpoint pupils, and has a diminished respiratory rate. The patients exam normalizes after naloxone administration. What is the most appropriate subsequent step to determine the cause of the patient’s symptoms?

A

Aspirate the pump reservoir

  • To measure the amount of drug in the reservoir
  • Patient most likely had a “pocket fill” with drug injected into the pocket surrounding the pump –> large subcutaneous opioid injection
169
Q

AED most commonly associated with agranulocytosis

A

Carbamazepine

-Odds ratio of 11%

170
Q

MOA of buprenorphine. When should pt stop taking buprenorphine prior to major surgery

A

Buprenorphine (Suboxone)

  • partial mu opioid receptor agonist and antagonist at the kappa opioid receptor
  • Binds m-opioid receptor 1000 times higher affinity than morphine

-Stop 3days prior to surgery

171
Q

MOA of gabapentin

A

-Binds to voltage-gated calcium channels, and is considered to be a membrane stabilizer

172
Q

MOA of baclofen

A

GABA-b receptor agonist

173
Q

Ketamine MOA

A

NMDA receptor agonist

174
Q

MOA of Lidocaine/ -caine’s

A

binds to voltage gated sodium channels in nerve cells, prevents depolarization, –> prevents transmission of a pain response

175
Q

Fentanyl MOA

A

binds the mu, delta, and/or kappa (as well as other) opioid receptors in the nervous system

176
Q

Herpes zoster outbreak resulting in ear pain is known as…

A

Ramsay-Hunt syndrome

-zoster outbreak in the geniculate ganglion

177
Q

Anesthetic agent that causes increase in cerebral blood flow

A

Ketamine

-Increases both CBF and CMRO2 (Cerebral metabolic rate of oxygen)

178
Q

Radiological marker of dural laceration in pediatric skull fx’s

A

Diastasis of edges of fracture > 4mm

-Skull fx with dural laceration and brain contusion can lead to development of a traumatic meningoencephalocele (‘growing skull fracture’/ ‘leptomeningeal cyst’)

Tx: Dural repair and cranioplasty

179
Q

Most common type of infection after head injury?

A

UTI

180
Q

Most appropriate maintenance rate for IV fluid in a child?

A

4ml/kg/hr for first 10kg

2ml/kg/hr for second 10kg

1ml/kg/hr for additional weight

181
Q

6month old infant p/w enlarging head circumference, full anterior fontanelle, splitting of the cranial sutures and developmental delay. On imaging pt found to have hydrocephalus and absence of the cerebellar vermis. Most likely Dx?

A

Dandy-Walker malformation

182
Q

What cranial suture is the most common site of premature fusion in non-syndromic craniosynostosis?

A

Sagittal synostosis (40-60%)

  • Coronal synostosis (20-30%)
  • Metopic synostosis (<20%)
  • Lambdoid synostosis (Rare)
183
Q

DDx of macrocephaly in infants

A
  • Benign macrocrania (MC)
  • Chronic subdural hematoma (possibly due to NAT)
  • Hydrocephalus
184
Q

A pt underoing EDAS for moyamoya. What is the most common cause of new perioperative neurological deficit?

A

Cerebral ischemia

  • Due to baseline ischemia and lack of cerebral autoregulatory reserve
  • Increased metabolic demand during perioperative period (hyperthermia, pain)
  • Perioperative hypocarbia, hypoxia, and hypotension
185
Q

Blood volume in pediatric patients

A
  • Premature infant: 90-100cc/kg
  • Full term infant: 80cc/kg
  • 1-12month baby: 75cc/kg
  • Child over 1yr: 70cc/kg
186
Q

Vitamin deficiency associated with craniosynostosis?

A

Vitamin D deficiency/Rickets

187
Q

Characteristics of propofol infusion syndrome?

A

PIS: Occurs in pts undergoing prolonged high-dose propofol therapy (4-5 mg/kg/hr for 48hrs)

  • rhabdomyolysis (high CK, myoglobinuria)
  • Renal failure (oligo/anuria, elevated creatinine, hyperkalemia)
  • Bradyarrhythmias, ventricular arrhythmias
  • Cardiac failure
  • Lipemia (high TG)
  • Hepatomegaly/fatty liver (elevated transaminases)
  • Metabolic acidosis
188
Q

Vitamin deficiency associated with craniosynostosis?

A

Vitamin D deficiency/Rickets

189
Q

Tx for baclofen overdose

A

Physostigmine

  • Counteracts the respiratory depressant effects of intrathecal baclofen.
  • Side effects: Bradycardia, increased respiratory secretions
190
Q

Three most common DDx for bump on skull in childhood?

A
  • Dermoid cyst
  • Ossifying cephalohematoma
  • Eosinophilic granuloma
191
Q

Which suture is affected?

  • Trigonocephaly
  • Scaphocephaly
  • Brachycephaly
  • Plagiocephaly
  • Turricephaly
A
  • Trigonocephaly: Metopic suture (triangle head)
  • Scaphocephaly: Sagittal suture (boat head)
  • Brachycephaly: B/L coronal sutures (short head)
  • Plagiocephaly: Unilateral coronal suture (harlequin appearance)
  • Turricephaly/oxycephaly: Coronal suture + another suture
192
Q

Embryonic development days:

  • Development of the notochord
  • Primary neurulation (Fusion of neural tube)
  • Anterior neuropore closure
  • Posterior neuropore closure
  • Secondary neurulation
A
  • Development of the notochord: 16
  • Primary neurulation (Fusion of neural tube): 22
  • Anterior neuropore closure: 24
  • Posterior neuropore closure: 27
  • Secondary neurulation: 28-32
193
Q

Chances of being seizure free for at least one year with the following AED treatment schedules

  • First
  • Second
  • Third
A

First: 50.4%
Second: 10.7%
Third: 2.7%

194
Q

VNS results. Likelyhood of seizure reduction

A

~50% reduction of seizures in approximately 30% of patients`

195
Q

2month old with hemorrhagic posterior fossa mass involving both cerebellar hemispheres and vermis. Genetic analysis of biopsy reveals SMARCB1/INI tumor suppressor gene deletion in chromosome 22. Most likely dx?

A

Atypical teratoid/rhabdoid tumor (AT/RT)

  • Loss of SMARCB1/INI tumor suppressor gene on chromosome 22
  • exclusive genetic marker to ATRT
196
Q

Intersegmental laminar fusion in a 6yo pt. Dx?

A

Diastematomyelia (split cord syndrome)

-Pathognomonic

197
Q

Most likely possible side effect of corpus callosotomy for epilepsy?

A

Disconnection syndrome

-inability to name objects, but able to recognize them

198
Q

Mitochondrial disorder that causes slowly progressive ptosis and ophthalmoplegia

A

Kearns-Sayre syndrome

199
Q

Most common intramedullary spinal cord tumors? How to tell them apart?

A

Ependymomas and astrocytomas (`~90%)
-Ependymoma is more often associated with central axial location, enhancement, syringohydromyelia, hemorrhage, and cap sign (hemosiderin above or below tumor due to prior hemorrhage)

200
Q

Injury to which nerve affects external rotation of the shoulder?

A

Suprascapular nerve

-Infraspinatus muscle

201
Q

Equation for expected PCO2

A

PCO2=(1.5 x HCO3) + 8

202
Q

Activation of MEKK3 signaling is seen in which disease process?

A

Cavernous malformation

203
Q

Eagle Syndrome

A

Syndrome where elongated styloid process compressed the extracranial portion of the glossopharyngeal nerve

204
Q

What stains what?

  • Synaptophysin
  • Vimentin, GFAP
  • Vimentin, EMA
  • S100
A
  • Synaptophysin: Neuronal cells (also stains for neuron-specific enolase)
  • Vimentin, GFAP: Glial tumors
  • Vimentin, EMA: Meningiomas
  • S100: Neural crest cell derived tumors (schwannomas, melanocytes, chondrocytes)
205
Q

Pineal mass, elevated alpha-fetoprotein. ddx?

A

endodermal sinus tumor
embryonal carcinoma
immature teratoma

206
Q

Pineal mass, elevated beta-HCG

A

Choriocarinoma

Mixed germ cell tumors

207
Q

Which form of systemic cancer therapy is associated with an INCREASED risk of radiation necrosis after stereotactic radiosurgery for cerebral metastases?

A 
Systemic immunotherapy (PD-1 inhibitors)
-Immune checkpoint inhibitors (most commonly PD-1 inhibitors) used for systemic immunotherapy amplify the immune system response to tumors by removing checkpoints which would normally temper the immune response.
208
Q

Which form of systemic cancer therapy is associated with an DECREASED risk of radiation necrosis after stereotactic radiosurgery for cerebral metastases?

A 
Cytotoxic chemotherapy (DNA synthesis/mitosis inhibitors)
-Likely due to immunosuppressive effects
209
Q

What radiotherapy modality for intracranial meningioma is associated with the lowest risk of symptomatic radiation injury?

A

Fractionated radiation therapy

210
Q

What clinical deficit can have the most negative impact on overall survival after resection of malignant glioma?

A

Post-op speech deficit
-2yr survival: 0%

Post-op motor deficit
-2yr survival: 8%

211
Q

Gorlin syndrome or nevoid basal cell carcinoma is associated with increased incidence of which tumor?

Which gene is involved?

A

Medulloblastoma

PTCH1 gene (heterozygous germline mutation)

212
Q

Best adjuvant therapy for chordomas after rsxn?

A

Proton beam radiotherapy

213
Q

Treatment for germinomas

A

Radiation (GKRS)

214
Q

Dorsal midbrain syndrome. Triad of symptoms:

A
  • Vertical gaze palsy
  • Pupillary light-near dissociation
  • Convergence retraction nystagmus
215
Q

Parkinson’s triangle on the lateral wall of the cavernous sinus is bounded by which two cranial nerves or cranial nerve divisions?

A

Parkinson’s triangle (infratrochlear triangle)

  • Trochlear nerve
  • Ophthalmic division of trigeminal nerve (V1)
216
Q

The addition of which chemotherapeutic agent to radiation therapy has been shown to increase overall survival in patients with newly diagnosed anaplastic oligodendroglioma?

Radiation dose?

A

PCV: Procarbazine/Lomustine (CCNU)/Vincristine

60Gy (in fractions of 1.8Gy)

217
Q

Best therapy for patients with symptomatic spine metastases without instability or neurologic deficit, especially in the setting of widely metastatic bone disease (primary is known)?

A

Fractionated external beam radiotherapy

218
Q

Most appropriate treatment for diffuse pontine glioma in a child

A

Conformal radiotherapy

219
Q

Stupp protocol

A

Concurrent TMZ and radiation (60Gy given over 30 fractions)

220
Q

Best treatment for JPA

A

Gross total resection (especially of mural nodule/enhancing mass)
-Cyst walls typically do not enhance and are not neoplastic

221
Q

Which gene is involved in NF1?

A 
Neurofibromin gene (on chromosome 17)
-Tumor suppressor gene: negatively regulates the ras oncogene
222
Q

Diagnostic criteria for NF1

A

Two or more of the following:
1) Six or more cafe-au-lait macules

2) Two or more neurofibromas of any type, or one plexiform neurofibroma
3) Freckling in the axillary or inguinal region
4) Optic glioma
5) Two or more lisch nodules (iris hamartomas)
6) A characteristic osseous lesion, such as sphenoid wing dysplasia or thinning of long bone cortex
7) A first-degree relative with NF1 by the above criteria

223
Q
  • Early cerebellar dysfunction
  • Immunocompromise
  • Development of capillary telangiectasias (especially sclera of the eye)

Dx?

A

Ataxia-Telangiectasia

224
Q

Which genetic or chromosomal alteration is associated with primary adult glioblastoma?

  • TP53 and ATRX mutation
  • Wnt signaling pathway
  • SMARCB1/INI1 mutation or deletion
  • 1p19q chromosomal deletion
  • EGFR and CDNK2A/CDNK2B mutations
A

-EGFR and CDNK2A/CDNK2B mutations
(IDH wild type)

  • TP53 and ATRX mutations: low grade astrocytomas and secondary GBM
  • SMARCB1/INI1: ATRT (atypical teratoid/rhabdoid tumors)
225
Q

What do the following on MR spect demonstrate?

  • NAA
  • Creatine
  • Choline
A
  • NAA: Marker for neuronal viability
  • Cr: Internal marker or reference
  • Cho: marker of cellular turnover
226
Q

Compensatory direction for rotatory diplopia in setting of trochlear nerve pathology

A

Head tilt away from affected side

227
Q

Facial colliculus syndrome

A
  • Ipsilateral facial nerve weakness

- Ipsilateral abducens nerve palsy (horizontal diplopia, conjugate gaze palsy)

228
Q

4th ventricular tumor without contrast enhancement. Histopathology shows nests of small blue cells amid a pink fibrillary matrix. Dx?

A

Subependymoma

229
Q

Borders of the Kawase’s triangle/rhomboid

A
  • GSPN
  • Arcuate eminence
  • Superior petrosal sinus
  • V3
230
Q

Branches of the meningohypophyseal trunk

A
  • Inferior hypophyseal
  • Dorsal meningeal
  • Tentorial arteries of Bernasconi and Cassinari
231
Q

What is the recommended radiotherapy regimen for GBM patients older than 70 years of age with poor performance status?

A

Hypofractionated radiation therapy (40Gy in 15 fractions over 3 weeks)
-Has been associated with superior survival and less corticosteroid requirement

Conventional fractionated radiation therapy = 60Gy in 30 fractions over 6 weeks

232
Q

RTOG criteria for the maximum radiation dose that should be given in a single fraction

A

For patients with previously irradiated brain tumors < 4cm

  • 24Gy for < 20mm
  • 18Gy for 21-30mm
  • 15Gy for 31-40mm

Tumors >20mm were 7-16x more likely to experience radiation necrosis than tumors < 20mm

233
Q

Mutation in the gene encoding for tumor suppressor protein Merlin results in which disease?

A

NF2

  • Chromosome 22
  • NF2 gene found on chromosome 22 encodes the tumor suppressor protein Merlin
234
Q

What primary intracranial tumor type has the highest frequency of BRAF-V600E mutations?

A

Gangliogliomas

235
Q

histopathological analysis demonstrates a well-circumscribed, uniformly cellular tumor with no atypia and numerous ectatic, thin-walled branching vessels in staghorn configurations. Dx?

What is a possible associated clinical development?

A

Hemangiopericytoma

  • Met outside the CNS. 20-50% met to lungs, liver, bone
  • High rate of recurrence

-Staghorn configuration = gaping sinusoidal spaces

236
Q

Most likely complication after vestibular schwannoma radiosurgery?

A

Hearing loss
-Useful hearing = (SDS > 50%, pure tone avg < 50dB)

Preservation of useful hearing after radiosurgery = approx 60%

237
Q

Following resection of a frontal supplementary motor area (SMA) cortex tumor, the patient develops impaired speech fluency. What pathway is responsible?

A

Frontal aslant tract (FAT)

  • Connects superior frontal gyrus with SMA
  • Deficits resolve within 3months
238
Q

What combination of IDH and 1p-19q status in diffuse glioma is most predictive of detecting a P53 gene mutation?

A
  • IDH mutant
  • 1p-19q intact

Diffuse astrocytomas, TP53 mutation in 90%

239
Q

On Histo:

  • presence of clusters of large neurons
  • lack of neoplastic glial cells clustering around neurons
  • presence of fibrosis
  • calcification
  • Binucleate neurons
  • Positive for BRAF V600E mutation
A

Ganglioglioma

240
Q

Pt p/w sudden onset right sdied ear pain, facial paralysis, hearing loss, and vertigo.

MRI demonstrates no mass lesion. Dx?

A

Zoster oticus/Ramsay-Hunt syndrome

-MRI may demonstrate linear enhancement of IAC with extension into tympanic segment of facial nerve

241
Q

How much of the occipital condyle can be removed?

What anatomy structure can be used as a landmark?

A

1/3 to 1/2 of condyle

Anterior condylar vein

242
Q

According to randomized control trials of hemicraniectomy for malignant MCA infarction, what is the time frame for the performance of hemicraniectomy associated with reduced mortality?

A

within 48hrs

-DECIMAL, DESTINY, HAMLET

243
Q

Three parts of normal ICP waveform?
P1
P2
P3

What change in waveform is an early sign of elevated ICP?

A
  • P1: Percussion wave - arterial pulsation
  • P2: Tidal wave - Intracranial compliance
  • P3: Dicrotic wave: Aortic valve closure
  • Normal: P1 has highest upstroke
  • Elevated ICP: P2 higher than P1
244
Q

Cushing’s response

A
  • HTN
  • Bradycardia
  • Respiratory depression
  • Peripheral vasoconstriction and catecholamine release –> HTN
  • Medullary ischemia –> Bradycardia
245
Q

In a patient with Guillain-Barre syndrome, what does the presence of significant cerebrospinal fluid pleocytosis (elevated WBC count) suggest?

A

Coexistent HIV type 1 infection

246
Q

Symptoms of propofol infusion syndrome

A
  • altered mental status
  • hyperkalemia
  • hepatomegaly
  • lipemia
  • rhabdomyolysis
  • hypertriglyceridemia
  • metabolic acidosis
  • kidney failure
247
Q

MOA of baclofen

A

GABA-B agonist

248
Q

Following aneurysmal subarachnoid hemorrhage, which cardiac complication is the most significant independent predictor of mortality?

A

Cardiac failure

-Increased in-hospital mortality (1.6x), LOS, and morbidity

249
Q

formula for expected respiratory compensation in metabolic acidosis

A

pCO2=1.5xHCO3+8 +/- 2

250
Q

Elderly pt in nursing home with h/o dementia and relatively rapid decline.

Bran bx/histology demonstrates: regional distribution of tau aggregates, abnormal tau accumulation in neurons and glia, in an irregular, focal, perivascular distribution and at the depths of cortical sulci

Dx?

A

Chronic traumatic encephalopathy.

Histology: abnormal tau accumulation in neurons and glia, in an irregular, focal, perivascular distribution and at the depths of cortical sulci - characteristic

251
Q

Symptoms of malignant hyperthermia and tx?

A
  • Hypercapnia
  • Hyperthermia
  • Muscular rigidity
  • Hypoxia

Tx: Dantrolene

252
Q

New onset muscle weakness and vision difficulties that improve after administration of edrophonium

Dx?

A

Myasthenia gravis

  • ptosis and oculobulbar muscle weakness , generalized weakness that worsens as the day progresses
  • auto-antibodies against ACh receptors

Edrophonium: Cholinesterase inhibitor –> Increases ACh

253
Q

Mcgregor’s and Chamberlain’s line

A

-Mcgregor’s: post edge of hard plate to most caudal point of occipital curve. If tip of the odontoid >4.5mm above then basilar invagination likely

Chamberlain’s line: post end of hard palate to posterior lip of foramen magnum. If tip of odontoid >3mm above, then basilar invagination likely

254
Q

Most common adverse event following laminoplasty for cervical spondylotic myelopathy?

A

Loss of cervical ROM

255
Q

Which spinal disorder are children of mothers with DM most at risk?

A

Sacral agenesis

Neurosurgery Board Prep (4 decks)

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