Q?
Neuroanatomy
• cavernous carotid artery? A.
The The inf inferi erior or hypo hypophy physe seal al art arter eryy is is mos mostt com commo monly nly a bra branc nchh of of the the meningohypophyseal trunk.
B.
The The infer inferio iorr hypop hypophy hyse seal al arte artery ry pas passe sess media mediallllyy to the the poste posteri rior or pitu pituititar aryy caps capsul ule. e.
C. Persi Persist stent ent trig trigem emin inal al arte arteri ries es can can also also origi originat natee from from the post posteri erior or vert vertic ical al segment of the cavernous cavern ous ICA and pass posteriorly through the posterior wall of the sinus to join the basilar artery between the origin of the superior and anterior inferior cerebellar arteries. D. The te tentorial aarrter or the aarrter of Be Bernasconi aannd Ca Cassinari is the mo most inconstant branch of the meningohypophyseal trunk. ©™
A. •
Neuroanatomy
. meningohypophyseal trunk the most constant artery (labeled below). Tentorial artery
Cerebral Peduncle Pituitary
Dorsal meningeal artery
•
The meningohypophyseal trunk usually arises from the posterior aspect of the central third of the posterior bend of the artery at the level of the dorsum sellae and frequently gives rise to three branches: the tentorial arter (Bernasconi and Cassinari ), the dorsal meningeal artery, and the inferior hypophyseal artery.
Carotid
CN3 V1
CN4
CN5
Isolan G, de Oliveira Oliveira E, Mattos JP. Microsurgical anatomy of the arterial compartment of the cavernous sinus: analysis analysis of 24
V1
V2
Middle Fossa
©™
Q?
Neuroanatomy
• F
1. Sylvian fissure 2. Rolandic fissure
E G
3. Pars triangularis
H
4. Pars o ercularis 5. Pars orbitalis
B
6. Supramarginal gyrus
A
C D
7. Angular gyrus
©™
A.
Neuroanatomy Motor Motor c cortex ortex
Sensory Sensory cortex cortex
F
1. D, Sylvian fissure 2. E, Ro Rolandic fifissure
E G
3. B, Pars triangularis
H
4. C Pars o ercularis 5. A, Pars orbitalis
B
6. G, Supramarginal gyrus
A
C D
7. H, Angular gy gyrus •
F is the precentral sulcus.
©™
Q?
Neuroradiology
• diagnosis? A. Low grade glioma B. Pleiomorphic xanthoastrocytoma . D. None of the above
©™
A. •
Neuroradiology ,
BOARD FAVORITE!
.
•
N-acetylaspartate N-acetylaspartate (NAA) is predominantly located in neurons and is thus decreased in all neoplasms that cause the neurons to be displaced or replaced with malignant cells. Findings of numerous studies have demonstrated decreased NAA values in glial neoplasms.
•
Choline (Cho) peak contains contributions from glycerophosphocholine, phosphocholine, and , turnover . As in any process that leads to hypercellularity and increased membrane proliferation, the Cho value is consistently elevated in gliomas.
•
Lactate (Lac) indicates that cellular respiration has shifted from the oxidative metabolism of carbohydrates to nonoxidative metabolism. Increased reliance on anaerobic glycolysis is found in hi hl mali nant tumors.
Law M, Hamburger M, Johnson G, Inglese M, Londono A, Golfinos J, Zagzag D, Knopp EA. Differentiating surgical from nonsurgical lesions using perfusion MR imaging and proton MR spectroscopic imaging. Technol Cancer Res Treat. 2004
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Q?
Neuroradiology
• A. Iron deposition B. Macrocrania C. Hypoxemia D. Gene Generraliz alized ed atro trophy phy E. Butterfly fly glioma
©™
A.
Neuroradiology
•
,
•
Hallervorden-Spatz syndrome- Deposition of iron especially globus pallidus & retina
•
Dyst Dystoonia, nia, rigid igiditityy and and neur neurob obeehavi havior oral al chan change gess
•
MRI: Globus pa pallidus on on T2-wei weighted iim mages
•
.
ye-o - e- ger s gn
• •
BOARD FAVORITE!
Central region of hyperintensity –
Primary tissue insult
–
Produces edema
Surroundin h ointensit –
Region high in iron
–
May be 2° process ©™
Yeomans Neurological Surgery 5 th Ed. Editor Richard Winn, 2003. p. 2720
Q?
Neuroradiology
• parents feel that her milestones are delayed. Her head CT is shown on the right. •
Whic Whichh of the the fol follo lowi wing ng is the the MOS MOST T accu accura rate te stat statem emen entt regarding this condition? A. A. Jaun Jaundi dice ce is uite uite comm common on.. B. After After 1 year year of of age, age, these these chil childre drenn rarel rarelyy have have new new complications. C. Incide Incidence nce of of mental mental reta retarda rdatio tionn associ associate atedd with with this this condition is 41-71%. . year old. ©™
A.
Neuroradiology ,
condition is 41-71%. •
This CT head shows Dandy-Walker malformation (DWM)- a malformation associating hypoplasia of the vermis, pseudocystic fourth ventricle, upward displacement of the tentorium, torcular and lateral sinuses and anterio-posterior en argemen o e pos er or ossa.
•
Children more than 1 year of age commonly present with developmental delay and symptoms of elevated intracranial pressure.
1. Klein O, Pierre-Kahn A, Boddaert N, Parisot D, Brunelle F. Dandy-Walker malformation: prenatal diagnosis and prognosis. Childs Nerv Syst. 2003 Aug;19(7-8):484-9 2. Yeomans Neurological Surgery 5 th Ed. Editor Richard Winn, 2003. p. 3285-86
©™
Q?
Neurobiology
• A.
First year of life
B.
Second year of life
C. Third year of life D. Fourth ear of life
©™
A. •
Neurobiology .
•
The The met metop opic ic sutu suture re will will gene genera rallllyy fuse fuse in a chi child ld between 3 and 9 months of age. Ossification occurs after the closure during the second year of life.
•
Clos Closur uree ooff the the meto metopi picc sutu suture re star starts ts unde underr norm normal al until the end of the second year
•
The The pre prematu mature re arre arrest st of grow growth th of th the metopic suture synostosis may present as a spectrum of manifestations including a keel-shaped forehead, retru etrud ded orbit rbital al rims rims and and h oter oterlo lori rism sm..
1. Collman, H., Forensen, N., Kraus, J. Consensus: Trigonocephaly. Child Nerv Syst. 1996; 12:664-668 2. H.L. Vu, J. Panchal, E.E. Parker, N.S. Levine, P. Francel. The timing of physiologic closure of the metopic suture: a review of 159 patients using reconstructed 3D CT scans of the craniofacial region. The Journal of Craniofacial Surgery. 2001; 12(6):527-32. 3. Yeomans Neurological Surgery 5th Ed. Editor Richard Winn, 2003. p. 3300-01
©™
Q?
Neurobiology
• potential: A. A.
.
Volta oltagege-ga gate tedd potass potassiu ium m chann channel elss (also (also call called ed dela delaye yedd recti rectififier er pot potas assi sium um channels) have a delayed response, such that potassium continues to flow out of the cell and initiate depolarization. yperpo ar za on s cause
y
n ux n o a ce .
C. Clos Closin ingg of volt voltage age-g -gat ated ed pota potass ssiu ium m chan channel nelss is both both volt voltag agee- and and tim timeedependent. D. Depol polarization is caused by Na+ efflux from a cell.
©™
A. • •
Neurobiology
The correct answer is C. Closin of volta e- ated otassium channels is both volta e- and timerepolarization initiates the closing of dependent. As potassium exits the cell, the resulting membrane repolarization voltage-gated potassium channels. These channels do not close im immediately in response to a change in membrane potential. Peak 40 Rather, voltage-gated potassium channels (also called delayed rectifier potassium ) V m channels) have a delayed response, such ( cell even after the membrane has fully repolarized. Thus the membrane potential dips below the normal resting membrane potential of the cell for a brief moment; this dip of hyperpolarization is known as the undershoot.
• •
BOARD FAVORITE!
e g a t l 0 o v e n a r b m e M -55
K+ efflux
Threshold Resting potential
-70
from a cell. Depolarization is ca caused by Na+ influx into a cell. Principles of Neural Science 3 rd Ed, Kandel ER, Apple and Lange 1991
Stimulus 0
1
Na+ influx
2 3 Time (ms)
Hyperpolarization 4
5
©™
Q?
Neurobiology
• invaginating through: A. The notochord B. Hensen’s no node C. The ca caudal em eminence D. The uncinat nate proce ocess
©™
A.
Neurobiology BOARD FAVORITE!
• •
,
’
.
Hensen’s node (primitive node) is the regional thickening of cells anterior of the primitive streak through which gastrulating gastrulating cells migrate anteriorly to form tissues in the future head and neck. Hensen’s node is also responsible for the secretion secretion of cellular signals essential to gastrulationgastrulation- including fibroblast growth factors, sonic hedgehog, and retinoic acid . Differential secretion of these factors by the node also causes development of the right-left axis in the embryo. Gastrulation
Notochord Ectoderm Ectoderm
Amniotic cavity
Primitive node
Yolk sac Endoderm
Primitive Primitive pit p it
Notochordal process Primitive streak
Yeomans Neurological Surgery 5 th Ed. Editor Richard Winn, 2003. p. 4241
Oral placode
©™
Q?
Neuropathology
• atrophy that is consistent with: A. Multiple sclerosis B. Pick’s disease C. Alzheimer’s r’s di disease D. Acut Acutee cere cerebr bral al inf infarct arctio ionn
©™
A. •
Neuropathology ’
Diffuse Atrophy
disease. •
Alzheimer’s disease is the most common dementing illness in adults, characterized by progressive dementia over several years. There is increased frequency with , usually earlier onset.
•
Note the diffuse diffuse brain atrophy atrophy of Alzhiemer’s disease , unlike Pick’s disease which has mostly frontal lobe atrophy.
•
Risk is increased in Down’s syndrome (BOARD FAVORITE).
Diffuse Atrophy ©™
Kuljis RO: Modular corticocerebral pathology in Alzheimer's disease. In: Mangone CA, Allegri RF, Ariza, eds. Dementia: A Multidisciplinary Approach. 1997: 143-55.
Q?
Neuropathology
• A. Intracytoplasmic B. α-synuclein immunoreactive C. Tau-p au-pro rote tein in immu immuno nore reac actitive ve D. Ubi uitin immunoreactive
©™
A.
Neuropathology
BOARD FAVORITE!
, immunoreactive. Nucleus
•
Lewy bodies are intracytoplasmic inclusions, stain for ubiquitin and αprotein. n. AlphaAlphasynuclein, but NOT tau protei synuc e n s norma y so u e an predominantly a presynaptic neuronal protein of unknown function (possible function as a molecular chaperone in the formation of SNARE complexes), but can also be foundLewy in body g a ce ce s.
•
Neurofibrillary tangles are immunoreactive for tau-protein. Dalfo E, Ferrer I. Early alpha-synuclein lipoxidation in neocortex in lewy body diseases. Neurobiol Neurobiol Aging. 2006 Dec 11
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Q?
Neuropathology
• consistent with: A.. Gemis A emisttocyt ocytic ic astr astroc ocyt ytom omaa B. Pituitary adenoma C. Pilocytic as astrocytoma D. Giant ce cell gl glioblas blasttoma E. Oligodendroglima
©™
A.
Neuropathology
•
,
BOARD FAVORITE!
.
•
Giant cell GBM has numerous multinucleated giant cells plus the 4 - typica typicall featu features res of of classic GBM: nuclear pleomorphism, endothelial hyperplasia, mitotic activity (MIB-1, Ki67), and central necrosis surrounded by viable tumor nuclei giving appearance of palisading which is called pseudopalis pseudopalisading ading.. Necrosis Necrosis is found in glioblastoma multiforme but NOT ana lastic astroc toma.
•
Molecular Pathway to Giant cell GBM
Arise de novo like primary GBM
PTEN loss ant ce
Progenitor cell p53 loss
©™ Aldape KD, Okcu MF, Bondy ML, Wrensch M. Molecular epidemiology of glioblastoma. Cancer J. 2003 Mar-Apr;9(2):99-106. Review.
Q?
Neuropathology
• consistent with: A.. Gemis A emisttocyt ocytic ic astr astroc ocyt ytom omaa B. Paraganglioma C. Craniopharyngioma D. Epidermoid E. Giant cell glioblas blasttoma
©™
A.
Neuropathology
•
,
BOARD FAVORITE!
.
•
Paragangliomas (aka chemodectomas) arise from the glomus cells (special chemoreceptors located along blood vessels that have a role in regulating blood pressure and blood flow) and are derived from the embryonic neural crest.
•
Secretory granules may contain many neuropeptide hormones including adrenocorticotropic , , , .
•
Microscopy demonstrates clusters “zellballen” of epitheliod (chief) cells and are invested in a highly vascular capillary stroma. Histologic criteria does not predict their malignant potential. Malignancy is determined by the presence of metastasis.
•
There are 4 tumor types:
•
-
•
Glomus jugulare- arise from the superior vagal ganglion
•
Glomus tympanicum- arise from the auricular branch of the vagus nerve
•
Glomus intravagale- arise from the inferior vagal ganglion.
Al-Mefty O, Teixeira A: Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg 2002; 97: 1356-66
©™
Q?
Neurosurgery
• A. Stable B. Unstable C. Affecting 1 column D. Affectin 2 or more columns E. B + D
©™
A. • • •
Neurosurgery
The correct answer is E. The image to the right presents a burst fracture and the three column theory classification. In the burst fracture, both the anterior and middle column are affected. This
unstable.
Three column theory
1. Browner B, Jupiter JB, Levine A, Trafton P, editors. editors. Skeletal trauma. 2nd ed. Philadelphia: W.B. Saunders Company; 1998. p 967-981. 2. Yeomans Neurological Surgery 5 th Ed. Editor Richard Winn, 2003. p. 4183
Burst Fracture
©™
Q?
Neurosurgery
• A. Extens Extension ion of tthe he dist distal al interp interphala halangea ngeall (DIP) (DIP) jjoint oint of index index finger finger.. B. Increas Increased ed flex flexion ion of the metacar metacarpal pal phalang phalangeal eal (MCP (MCP)) joint joint of thumb. thumb. C. Weakn Weakness ess of forced forced supina supinatio tionn of forearm forearm with with elbow elbow flexed. flexed. D. Sensor chan es in the index fin er.
©™
A. •
Neurosurgery ,
BOARD FAVORITE!
.
•
There are NO sensory changes with anterior interosseous syndrome.
•
Anter nterio iorr Int Intero erosseo sseouus Synd Syndrrome ome (AIS) AIS) is a type type of median nerve entrapment syndrome (anterior interosseous branch of the median nerve). Nerve fibers carried in this branch are purely motor and innervate the flexor digitorum profundus 1 and 2, flexor pollicis longus, and pronator quadratus . Motor skills are affected by this syndrome, such as the “pinch sign” (inability to form a circle with the n ex ng nger an um . ensory percep on s no a ec e .
NORMAL NORMAL
PINCH PINCH SIGN SIGN
1. Van Beek AL: Management of nerve compression syndromes and painful neuromas. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders; 1990. 2. Yeomans Neurological Surgery 5 th Ed. Editor Richard Winn, 2003. p. 3925
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Q?
Neurology
•
A. Retinal detachment B. Su Supe peri rior or colli ollicculus ulus lesi lesion on C. Optic nerve atrophy D. Lat Lateral eral enic enicul ulat atee nuc nucleus leus
©™
A. •
Neurology ,
.
•
The Marcus-Gunn pupil is also known as the pupillary escape reflex, and is a relative afferent pupillary defect. The mechanism by which this phenomenon occurs is thought to be by a reduction in the number of fibers subserving subser ving the light reflex on the affected side.
•
Test by the swinging-flashlight test -the patient's pupil dilates instead of constricting when transmission. Both eyes constrict when the light shines in the unaffected (good) eye.
•
A lesi lesion on of of an opt optic ic tra tract ct may may res resul ultt in a sli sligh ghtt supp suppre ress ssio ionn of the the pup pupililla lary ry ligh lightt re reflflex ex;; lesi lesion onss at the level of the lateral geniculate nucleus, or in the visual radiations result in a contralateral homonymous hemianopsia with no change in the pupillary light reflex.
O'Connor PS, Kasdon D, Tredici TJ, Ivan DJ. The Marcus Gunn pupil in experimental tract lesions. Ophthalmology. 1982 Feb;89(2):160-4.
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Q? •
Neurology
, ventricles, now complains of worsening headache. head ache. Fundoscopic exam is shown below. below. Which of the following statements is TRUE? A. A. It is is best best trea treate tedd wit withh Dia Diamo mox. x. B. Like Likely ly has shunt shunt fail failure ure and and sti stifff ven ventr tric icle les. s. C. Like Likely ly has has ven venou ouss thro thromb mbos osis is.. D. Ther Theree is no caus causee for for conc concern ern about about thi thiss hea headac dache. he.
©™
A.
Neurology
•
, . should be present when evaluating a patient with shunted pseudotumor cerebri (PTC). Approximately 40% of shunts placed in children will fail in the 1st year and almost all children will require shunt revision at some point. In PTC shunt malfunction may result in blindness.
•
PTC is PTC is enco encoun unte tere redd most most freq freque uent ntly ly in in youn young, g, over overwe weig ight ht wom women en betw betwee eenn the the age agess of 20 and and 45. 45. Head Headac ache he is the the most most comm common on rese resent ntin in com com lain laintt occu occurr rrin in in more more than than 90 erce ercent nt of cases. Dizziness, nausea, and vomiting may also be encountered, but typically there are no alterations of consciousness or higher cognitive function.
•
It is def defin ined ed cli clini nica callllyy by four four cri crite teri ria: a: (1) (1) ele eleva vate tedd intr intrac acra rani nial al pres pressu sure re as as demo demons nstr trat ated ed by by lumbar puncture; (2) normal cerebral anatomy, as demonstrated by neuroradiographic evaluation; (3) normal cerebrospinal fluid composition; and (4) signs and symptoms of increased intracranial pressure, including papilledema.
Martin TJ, Corbett JJ: Pseudotumor cerebri, in Youmans JR(ed): Neurological Surgery, ed 4. Philadelphia: WB Saunders,1996, Vol 4, pp 2980–2997
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Q?
Neurology
• A. Immediately B. 2 to 8 days C. 10 to 25 days D. 20 to 35 da s E. 30 to 40 days
©™
A.
Neurology ,
BOARD FAVORITE!
.
•
Fibrillation potentials are caused by injury to a single nerve fiber, are biphasic or triphasic, and last 1-5 milliseconds. Fibrillations are seen on electromyography (EMG) 10 to 25 days after axon death. They are associated with anterolateral sclerosis, poliomyelitis, some myopathies (polymyositis) or peripheral nerve injury inju ry e.g. ransec on
•
Fibrillation potentials are not seen thru the skin , whereas fasciculation potentials are seen throught the skin (quivering muscle).
•
Not seen in neuromuscular junction defects (myasthenia gravis, Lambert-Eaton)
• unit), are polyphasic, and last 5-15 milliseconds. They are associated with anterolateral sclerosis, poliomyelitis or radiculopathy. radiculopathy. Nandedkar SD, Barkhaus PE, Sanders DB, et al: Some observations on fibrillations and positive sharp waves. Muscle Nerve 2000 Jun; 23(6): 888-94.
©™
Q.
Neurocritical Care
disorder? A.. Decr A Decrea ease sedd fibr fibrin in-s -spl plitit prod produc ucts ts B. Increased factor X C. Low platelets D. Decreased d-dimer
©™
A.
Neuro-Critical Care
, . fibrin-split products and d-dimers increase. Inciting factor: Ex. crush injury, endotoxin
Widespread intravascular fibrin deposition
Consumption of platelets and clotting factors
Thrombosis & organ failure
Thrombosis & organ failure ©™ DIC
Q?
Neurocritical Care
• drug? A. Olanzapine (Zy (Zyprexa) B. Risper peridone (Risper perdal) C. Haloperidol (Haldol) D. Ziprasidon done (Geodo odon)
©™
A.
Neurocritical Care BOARD FAVORITE!
,
.
•
Tardive dy dyskinesia is is mo mostly se seen in in older neuroleptic drugs, such as Haldol and the typical antipsychotics.
•
Newe Newerr aty atypi pica call antip antipsy sych chot otic icss such such as olanz olanzap apin inee and and rris ispe peri ridon donee aappe ppear ar to caus causee tardive dyskinesia somewhat less frequently. frequently.
•
receptor. It occurs in Tardive dyskinesia is due to hypersensitivity of the dopamine receptor. 15-30% of patients receiving treatment with antipsychotic neuroleptic medications for 3 months or longer. longer. It is characterized by the urge urg e to perform p erform repetitive, involuntary involu ntary,, purposeless movements. Features of the disorder may include grimacing, tongue protrusion, lip smacking, and rapid eye blinking. Rapid movements of the arms, legs, and trunk may also occur. Impaired movements of the fingers may appear as though the patient is playing an invisible guitar or piano. de Leon J. The effect of atypical versus typical antipsychotics on tardive dyskinesia : A naturalistic study. Eur Arch Psychiatry Clin Neurosci. 2006 Dec 5
©™
Q?
Neurocritical Care
• A. Alcohol, primidone, β-blockers B. Ad Adre rene nerg rgic ics, s, met metha hado done ne,, antic anticho holiline nerg rgic icss C. Alc Alcohol ohol,, nal nalttrex rexone, one, ant antab abus usee D. Alcohol, ro anolol, naltrexone
©™
A.
Neurocritical Care BOARD FAVORITE!
•
,
,
, -
.
•
Medical: Treatment of essential tremor (ET) is not always undertaken but depends on the severity of presenting presenting symptoms. Drug treatment includes tranquilizers, tranquilizers, β-blockers, and antiepileptic drugs. The two most common medications prescribed are the anticonvulsant primidone (Mysoline®) and the β-blocker propranolol (Inderal®).
•
, considered. Stereotactic thalamotomy and thalamic ventralis intermedius nucleus deep brain stimulation (DBS) are the procedures of choice.
•
Both proc Both proced edur ures es offe offerr high high rate ratess of trem tremor or redu reduct ctio ionn in the the contralateral arm. Recent information suggests that they are also useful in reducing head and voice tremor. Bilateral thalamotom is associated wi with a relativel hi h ririsk of d sarthria, occurrin in as man as 29% of patients, and a risk of cerebral hemorrhage.
Whitney CM. CM. Essential tremor. Neurologist. 2006 Nov;12(6):331-2.
©™
Q? •
Neurogenetics
. begin with auditory hallucinations:humming, hallucinations:humming, buzzing, or ringing. During her seizure she she is unable to understand language. Her father has the same condition, and as part of a study she she was told she had a mutation in LGI1. She is concerned that she will will pass her condition on to her children. Her husband’s husband’s family has no history of seizures. A. Yes B. No
©™
A.
Neurogenetics BOARD FAVORITE!
•
.
•
The patient has autosomal dominant partial epilepsy with auditory features (ADPEAF)
•
ADP ADPEA EAF F is inhe inheri rite tedd in in an an aut autos osom omal al dom dominan inantt man manne nerr.
•
Most patients with ADPEAF have an affected parent.
•
Alth Althou ough gh the the con condi ditition on is is tra trans nsmi mitt tted ed in an an auto autoso soma mall domi domina nant nt fas fashi hion on,, pen penet etra ranc ncee is es ma e a . us some pa en s o no ex e syn rome.
Kalachikov, Sergey et Al: Mutations in LGI1 cause autosomal-dominant partial epilepsy with auditory features. Nature Genetics Genetics 30, 335 - 341 (2002)
©™
Q?
Neurogenetics
• subtype? A. HLA-B27 B. HLA-DR2 C. HLA-DQB1 D. HLA-DQ2
©™
A.
Neurogenetics BOARD FAVORITE!
. •
The The mol molec ecul ular ar basi basiss of of the the asso associ ciat atio ionn of of HLA HLA-D -DR2 R2 in in tthe he pat patho hoge gene nesi siss of multiple sclerosis has been well established but how it occurs is still under investigation.
•
associated ed with multipl multiplee arthropathie arthropathiess –e.g. ankylo HLA-B27 is associat ankylosin sing g spondy spondylit litis is
•
HLA-DQB1 is associated with narcolepsy
•
HLA-DQ2 is associated with celiac disease
Dyment, D, et al: Genetic susceptibility to MS: a second stage analysis analysis in Canadian MS families. Neurogenetics 3:145,2001
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