Neurology Flashcards
(256 cards)
1
Q
What is the role of the notochord?
A
Induces overlying ectoderm to differentiate into neuroectoderm and form neural plate.
2
Q
What is the role of the neural plate?
A
Gives rise to neural tube and neural crest cells.
3
Q
What does the notochord become?
A
Nucleus pulposus of the intervertebral discs
4
Q
What does the neural tube become?
A
Spinal cord and brain
5
Q
What are the three primary vesicles and 5 secondary vesicles of the developing brain?
A
Prosencephalon (forebrain; becomes diencephalon and 2 telencephalons), Mesencephalon (midbrain), Rhombencephalon (hindbrain; becomes metencephalon and myelencephalon)
6
Q
What does the neuroectoderm differentiate into?
A
CNS neurons, ependymal cells, oligodendroglia, astrocytes
7
Q
What do the neural crest cells differentiate into?
A
PNS neurons, Schwann cells
8
Q
In what week of gestation do neural tube defects occur?
A
4th week
9
Q
What is an Arnold-Chiari malformation and what is another name for it?
A
Chiari malformation II; Herniation of cerebellar tonsils and vermis through foramen magnum with aqueductal stenosis and hydrocephalus. Often with lumbosacral myelomeningocele and paralysis below defect.
10
Q
What is a Dandy-Walker malformation?
A
Agenesis of cerebellar vermis with cystic enlargement of 4th ventricle. Associated with hydrocephalus and spina bifida.
11
Q
What is the role of microglia?
A
CNS phagocytes. HIV-infected microglia fuse to form multinucleated giant cells in CNS
12
Q
Multiple sclerosis, progressive multifocal leukoencephalopathy (PML), and leukodystrophies are all a result of what?
A
Injured oligodendroglia (myelination in CNS)
13
Q
Guillain-Barré is a result of what?
A
Destruction of Schwann cells; ascending paralysis
14
Q
Where is NE synthesized?
A
Locus ceruleus (pons)
15
Q
Where is DA synthesized?
A
Ventral tegmentum and SNc (midbrain)
16
Q
Where is 5-HT synthesized?
A
Raphe nucleus (pons, medulla, midbrain)
17
Q
Where is ACh synthesized?
A
Basal nucleus of Meynert
18
Q
Where is GABA synthesized?
A
Nucleus accumbens
19
Q
The BBB is formed by which 3 structures?
A
Tight junctions between non-fenestrated capillary endothelial cells, basement membrane, and astrocyte foot processes
20
Q
What specifically makes ADH?
A
Supraoptic nucleus of the hypothalamus
21
Q
What specifically makes oxytocin?
A
Paraventricular nucleus
22
Q
The lateral area of the hypothalamus is responsible for what? What happens in its destruction? What is the lateral area inhibited by?
A
Hunger; Anorexia, failure to thrive; Leptin
23
Q
The ventromedial area of the hypothalamus is responsible for what? What happens in its destruction? What is the ventromedial area stimulated by?
A
Satiety; Hyperphagia; Leptin
24
Q
The anterior hypothalamus is responsible for what?
A
Cooling, parasympathetic. “A/C: Anterior Cooling”
25
The posterior hypothalamus is responsible for what?
Heating, sympathetic.
26
The suprachiasmatic nucleus of the hypothalamus is responsible for what?
Circadian rythm
27
Branchial clefts are derived from which embryonic layer?
Ectoderm
28
Branchial arches are derived from which embryonic layer?
Mesoderm
29
Branchial pouches are derived from which embryonic layer?
Endoderm
30
What does the 1st cleft develop into?
External auditory meatus
31
What do the 2nd-4th clefts form?
Temporary cervical sinuses, which are obliterated by proliferation of 2nd arch mesenchyme.
32
What does a persistent cervical sinus lead to?
Branchial cleft cyst within lateral neck.
33
How is bedwetting (sleep enuresis) treated?
Oral desmopressin acetate (DDAVP) which mimics ADH
34
How are night terrors and sleepwalking treated?
Benzodiazepines
35
What is the input to the VPL nucleus of the thalamus?
Spinothalamic and dorsal columns/medial lemniscus
36
What is the input to the VPM nucleus of the thalamus?
Trigeminal and gustatory pathway
37
What is the input to the LGN nucleus of the thalamus?
CNII (vision)
38
What is the input to the MGN nucleus of the thalamus?
Superior olive and inferior colliculus of tectum (hearing)
39
What is the input to the VL nucleus of the thalamus?
Basal ganglia, cerebellum (motor)
40
Which structures make up the limbic system? What is the limbic system responsible for?
Hippocampus, amygdala, fornix, mammillary bodies, and cingulate gyrus. The famous 5 F's: Feeding, Fleeing, Fighting, Feeling, and Fucking
41
What is an intention tremor?
Slow, zig zag motion when pointing/extending toward a target - cerebellar dysfunction
42
What is hemiballismus?
Sudden, wild flailing of 1 arm +/- ipsilateral leg - contralateral subthalamic nucleus (lacunar stroke)
43
What is athetosis?
Slow, writhing movements; especially seen in fingers - basal ganglia (Huntington)
44
What is myoclonus?
Sudden, brief, uncontrolled muscle contraction
45
What is Kluver-Bucy syndrome?
Bilateral lesion of amygdala. Hyperorality, hypersexuality, disinhibited behavior. Associated with HSV-1.
46
What are the consequences of a right parietal-temporal cortex lesion?
Spatial neglect syndrome (agnosia of the contralateral side of world)
47
What are the consequences of a left parietal-temporal cortex lesion?
Agraphia, acalculia, finger agnosia, and L-R disorientation. Aka Gerstmann syndrome.
48
What are the consequences of a lesion to the subthalamic nucleus?
Contralateral hemiballismus
49
What are the consequences of a lesion to the paramedian pontine reticular formation?
Eyes look away from side of lesion
50
What are the consequences of a lesion to the frontal eye fields?
Eyes look toward lesion
51
What disease process results from correcting serum Na too fast?
Central pontine myelinolysis
52
What is central pontine myelinolysis?
A variant of the osmotic demyelination syndrome. Acute paralysis, dysarthria, dysphagia, diplopia, and loss of consciousness. "Locked-in syndrome."
53
What is conduction aphasia?
Poor repetition but fluent speech, intact comprehension
54
What is transcortical motor aphasia?
Nonfluent aphasia with good comprehension and repetition
55
What is transcortical sensory aphasia?
Poor comprehension with fluent speech and repetition
56
What is mixed transcortical aphasia?
Nonfluent speech, poor comprehension, good repetition
57
What is the most common location of a berry aneurysm?
Junction of anterior communicating artery and anterior cerebral artery
58
Which diseases are berry aneurysms associated with?
ADPKD, Ehlers-Danlos, and Marfan syndrome
59
What is a Charcot-Bouchard microaneurysm?
Associated with chronic HTN, affects small vessels (basal ganglia, thalamus)
60
An epidural hematoma is most commonly caused by rupture of which artery?
Middle meningeal artery
61
Does an epidural hematoma cross suture lines? Fax and tentorium?
Does not cross suture lines; can cross falx and tentorium
62
Does a subdural hematoma cross suture lines? Fax and tentorium?
Crosses suture lines; cannot cross falx or tentorium
63
A subdural hematoma is most commonly caused by rupture of what?
Rupture of bridging veins of arachnoid space
64
Which type of intracranial hemorrhage causes bloody or yellow (xanthochromatic) spinal tap?
Subarachnoid hemorrhage
65
Which disease process causes one to be "wet wobbly and wacky?"
Normal pressure hydrocephalus
66
How many spinal nerves do we have?
31. 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, 1 cocygeal
67
What are the common sites of a vertebral disc herniation?
Posterolaterally at L4-L5 or L5-S1
68
Where is a lumbar puncture performed?
Between L3 and L5 (cauda equina)
69
What are characteristics of an UMN lesion?
Spastic paralysis, Babinski +, weakness, hyperreflexia, more tone, clasp knife spasticity
70
What are characteristics of an LMN lesion?
Weakness, atrophy, hyporeflexia, fasciculations, low tone, flaccid paralysis
71
What medication can modestly increase survival of ALS patients?
Riluzole - decreases presynaptic glutamate release
72
A complete occlusion of the anterior spinal artery would spare which portion of the spinal cord?
The dorsal columns and Lissauer tracts
73
What is Tabes dorsalis?
Degeneration/demyelination of dorsal columns and roots, caused by tertiary syphilis. Exam will demonstrate Argyll Robertson pupils, Charcot joints, shooting pain, absence of DTRs, and positive Romberg.
74
What are Argyll Robertson pupils?
Small bilateral pupils that further constrict to accommodation and convergence, but not to light. Seen in Tabes dorsalis.
75
What is a positive Romberg sign?
Pt stands up with feet together and can only keep balance with their eyes open.
76
A VitaminB12 or VitE deficiency will affect which portions of the spinal cord?
Dorsal column, lateral corticospinal tracts, and spinocerebellar tracts.
77
What is Werdnig-Hoffman disease?
Congenital degeneration of anterior horns of spinal cord --> LMN lesion. Floppy baby with marked hypotonia and tongue fasciculations. Mutations in the survival motor neuron (SMN1) gene - involved in assembly of snRNPs (spliceosome).
78
What is the inheritance pattern of Freidreich ataxia?
Autosomal recessive
79
What is the inheritance pattern of Werdnig-Hoffman disease?
Autosomal recessive
80
Mutations in the survival motor neuron (SMN1) gene are associated with what disease?
Werdnig-Hoffman disease
81
Freidreich ataxia is do to what molecular process?
Trinucleotide repeat disorder (GAA) on chromosome 9 in gene that encodes frataxin (iron binding protein). Leads to impairment of mitochondrial functioning.
82
What are the clinical findings of Freidreich ataxia?
Muscle weakness, loss of DTRs, vibratory sense, proprioception. Staggering gait, falls, nystagmus, dysarthria, pes cavus, hammer toes, hypertrophic cardiomyopathy (cause of death).
83
How does Freidreich ataxia present in childhood?
Kyphoscoliosis
84
What is Brown-Séquard syndrome and what are its findings?
Hemisection of spinal cord. Ipsilateral UMN signs below level of lesion, ipsilateral LMN at level of lesion, ipsilateral loss of tactile, vibration, proprioreception sense 1-2 levels below level of lesion, contralateral pain and temperature loss below level of lesion.
85
What is the landmark of dermatome C2?
Posterior half of a skull "cap"
86
What is the landmark of dermatome C3?
High turtleneck shirt
87
What is the landmark of dermatome C4?
Low collared shirt
88
What is the landmark of dermatome T4?
Nipple
89
What is the landmark of dermatome T7?
Xiphoid process
90
What is the landmark of dermatome T10?
Belly button
91
What is the landmark of dermatome L1?
Inguinal ligament
92
What is the landmark of dermatome L4?
Kneecaps
93
What is the landmark of dermatome S2, S3, S4?
Erection and sensation of penile and anal zones
94
The biceps reflex is associated with which nerve root?
C5
95
The triceps reflex is associated with which nerve root?
C7
96
The patellar reflex is associated with which nerve root?
L4
97
The achilles reflex is associated with which nerve root?
S1
98
Primitive reflexes are inhibited by what in neurologically intact adults?
Mature/developing frontal lobe
99
What is the moro reflex?
"Hang on for life" reflex - abduct/extend limbs when startled, then draw together
100
What is the rooting reflex?
Movement of head toward one side if cheek or mouth is stroked
101
What is the sucking reflex?
Sucking response when roof of mouth is touched
102
What is the palmar reflex?
Holding on to finger
103
What is the plantar reflex?
Dorsiflexion of large toe and fanning of rest with plantar stimulation. Babinski sign if positive in adults.
104
What is the galant reflex?
Stroking along one side of the spine while newborn is in ventral suspension causes lateral flexion (face down) of lower body toward stimulated side
105
What is the pineal body responsible for?
Melatonin secretion and circadian rhythms
106
What is the superior colliculus responsible for?
Conjugate vertical gaze
107
What is the inferior colliculus responsible for?
Auditory
108
What is Parinaud syndrome?
Paralysis of conjugate vertical gaze due to lesion in superior colliculi
109
Which cranial nerve is responsible for mastication?
Trigeminal
110
Which cranial nerve is responsible for taste from epiglottic region?
Vagus
111
Which cranial nerve is responsible for taste and somatosensation from posterior 1/3 of tongue?
Glossopharyngeal
112
Which cranial nerve is responsible for somatosensation of the anterior 2/3 of tongue?
Trigeminal
113
Which cranial nerve is responsible for swallowing and salivation?
Glossopharyngeal
114
The nuclei of which cranial nerves reside in the medulla?
IX, X, XII
115
The nuclei of which cranial nerves reside in the pons?
V, VI, VII, VIII
116
The nuclei of which cranial nerves reside in the midbrain?
III, IV
117
The nuclei of which cranial nerves reside in the spinal cord?
XI
118
What are some etiologies for Horner syndrome?
Pancoast tumor, Brown-Sequard syndrome, late-stage syringomyelia
119
The arcuate nucleus of the hypothalamus is responsible for what?
Secretion of DA, GHRH, and gonadotropin-releasing hormone
120
The paraventricular nucleus of the hypothalamus is responsible for what?
Secretion of ADH, CTRH, oxytocin, and thyrotropin-releasing hormone.
121
Where is the chemoreceptor trigger zone located?
The dorsal surface of the medulla at the caudal end of the 4th ventricle
122
What is the mnemonic to remember clinical reflexes?
S1,2 "buckle my shoe" (Achilles)
L3,4 "kick the door" (Patellar)
C5,6 "pick up sticks" (Biceps)
C7,8 "lay them straight" (Triceps)
L,2 Cremaster
S3,4 Anal wink ;)
123
What is the most common pineal gland mass?
Germinona
124
What is Parinaud syndrome?
Paralysis of upward gaze, ptosis, and pupillary abnormalities. Due to direct compression of the pretectal region of midbrain.
125
What is Wallenberg syndrome and what does it lead to?
Lateral medullary syndrome. Contralateral loss of pain and temperature sensations along with ipsilateral paralysis of CNs V, IX, X, XI
126
What does Medial medullary syndrome cause?
Contralateral spastic paralysis, contralateral sensory loss of vibration and proprioception, and ipsilateral flaccid paralysis of the tongue (CN XII)
127
What is the nucleus solitarius (vagal nucleus) responsible for?
Visceral sensory info (taste, baroreceptors, gut distention) - VII, IX, X
128
What is the nucleus ambiguus (vagal nucleus) responsible for?
Motor innervation of pharynx, larynx, and upper esophagus - IX X, XI
129
What is the dorsal motor nucleus (vagal nucleus) responsible for?
Sends autonomic (parasympathetic) fibers to heart, lungs, and upper GI - CN X
130
Where is the cavernous sinus located?
On either side of the pituitary
131
Which nerves pass through the cavernous sinus?
CN III, IV, V1, V2, and VI and postganglionic sympathetic fibers en route to the orbit (and internal carotid artery)
132
What are the clinical manifestations of cavernous sinus syndrome?
Ophthalmoplegia and decreased corneal and maxillary sensation with normal visual acuity. CN VI commonly affected as well.
133
In a CN V motor lesion, does the jaw deviate toward or away lesion?
Toward; due to unopposed force from the opposite pterygoid muscle
134
In CN X lesion, what sign can be seen in the mouth?
Uvula deviates away from side of lesion. Weak side collapses and uvula points away.
135
What is the clinical manifestation of a CN XI lesion?
Weakness turning head to contralateral side of lesion (SCM muscle), and shoulder droop on ipsilateral side of lesion (trapezius)
136
What is the clinical manifestation of a CN XII lesion?
Tongue deviates toward side of lesion
137
What does the Rinne test test?
Tests air to bone conduction (place tuning fork behind ear then in front of ear and ask pt when they stop hearing)
138
What does the Weber test test?
Differentiates between conductive and sensorineural hearing loss (place tuning fork on top of head)
139
If a patient has conductive hearing loss, what happens in the Rinne and Weber tests?
Rinne: Abnormal (bone>air)
Weber: Localizes to affected ear
140
If a patient has sensorineural hearing loss, what happens in the Rinne and Weber tests?
Rinne: Normal (air>bone)
Weber: Localizes to unaffected ear
141
Is the forehead paralyzed in a facial UMN lesion?
No - bilateral UMN innervation of forehead
142
Is the forehead paralyzed in a facial UMN lesion?
Yes
143
What is the treatment of a facial nerve palsy?
Corticosteroids
144
What are the 3 muscles of mastication?
Masseter, temporalis, and medial pterygoid
145
What is hyperopia?
Eye too short for refractive power
146
What is myopia?
Eye too long for refractive power
147
What is astigmatism?
Abnormal curvature of cornea resulting in different refractive power at different axes
148
What is presbyopia?
Decrease in focusing ability during accommodation due to sclerosis and decreased elasticity
149
What are the usual causes of retinitis?
CMV, HSV, HZV
150
What are the two types of diabetic retinopathy?
Proliferative and non-proliferative
151
Describe non-proliferative diabetic retinopathy
Damaged capillaries leak blood --> lipids and fluid seep into retina --> hemorrhages and macular edema.
152
Describe proliferative diabetic retinopathy
Chronic hypoxia results in new blood vessel formation with resultant traction on retina.
153
What is the treatment for non-proliferative diabetic retinopathy?
Glucose control and macular laser
154
What is the treatment for proliferative diabetic retinopathy?
Peripheral retinal photocoagulatoin, anti-VEGF injections
155
Define glaucoma
Optic disc atrophy with characteristic cupping, usu with increased IOP and progressive peripheral visual field loss
156
What is open angle glaucoma associated with?
Age, African-American race, family hx. Painless.
157
What is secondary open angle glaucoma caused by?
Blocked trabecular meshwork from WBCs (uveitis), RBCs, retinal elements
158
What is primary closed/narrow angel glaucoma?
Forward pushing of lens against iris blocks trabecular network, fluid buildup and increased IOP
159
What is secondary closed/narrow angel glaucoma?
Hypoxia from retinal disease induces vasoproliferation in iris which contracts angle
160
What does papilledema indicate?
Increased intracranial pressure
161
How does the eye look with CN III damage/compression?
Eyes look down and out, ptosis, pupillary dilation, loss of accommodation
162
How does the eye look with CN IV damage?
Eyes move upward and head tilt toward side of lesion (because the superior oblique muscle rotates eye inward)
163
How does the eye look with CN VI damage?
Medially directed eye that cannot abduct (lateral rectus lesion)
164
Describe the pupillary light reflex
Light in either retina sends a signal via CN II to pretectal nuclei in midbrain that activates bilateral Edinger-Westphal nuclei and both pupils constrict
165
What is an afferent pupillary defect?
When there is less bilateral pupillary constriction when light is shone in affected eye relative to unaffected eye. Due to optic nerve damage or severe retinal injury.
166
What is CN III composed of?
Motor and parasympathetic fibers
167
If the upper left visual field is blind, where is lesion? (left upper quadrantic anopia)
Right Meyer loop (temporal lobe)
168
If the lower left visual field is blind, where is lesion? (left lower quadranti anopia)
Right dorsal optic radiation (parietal lobe)
169
If only the left center visual field is blind, where is the lesion?
Left macula
170
If the left visual field is blind sparing the macula, where is the lesion?
PCA infarct of contralateral side
171
Where is the brain does the Meyer loop travel?
Loops around inferior horn of lateral ventricle
172
Where in the brain does the dorsal optic radiation travel?
Internal capsule
173
Internuclear ophthalmoplegia (INO) occurs when what tract is damaged (usu with MS)?
MLF
174
What is the role of the MLF in terms of vision?
Allows for crosstalk between CN VI and CN III nuclei - coordinates both eyes to move in same horizontal direction.
175
In INO, what is seen?
With a left MLF lesion, the right eye looks laterally but the left medial rectus cannot contract and left eye stays looking straight. Right eye will have nystagmus because CN VI overfires to try to stimulate CN III. Convergence is normal.
176
An orbital floor fracture will damage what nerve?
Infraorbital nerve (continuation of maxillary nerve).
177
Atrophy of cortex leads to what disease process?
Dementia
178
Atrophy of basal ganglia leads to what kind of diseases?
Movement disorders
179
What is the neurotransmitter abnormality in Alzheimer's?
Low ACh; due to decreased choline acetyltransferase activity in the nucleus basalis
180
What are the hallmarks of Alzheimers?
Senile plaques composed on extracellular beta amyloid, and neurofibrillary tangles composed of intracellular hyperphosphorylated tau protein
181
Early onset Alzheimers is due to which altered proteins?
APP (Chr 21), presenilin-1 (Chr 14), and presenilin-2 (Chr 1)
182
Late onset Alzheimers is due to which altered protein?
ApoE4 (Chr 9)
183
Which protein expression is protective against Alzheimers?
ApoE2 (Chr 19)
184
Are there focal neurological deficits in Alzheimers?
No! Diffuse process
185
What is another name for Pick disease?
Frontotemporal dementia
186
What is the hallmark of Pick disease?
Pick bodies: spherical tau protein aggregates
187
What is Lewy body dementia and what is its hallmark?
Initially dementia and visual hallucinations followed by Parkinsononian features; alpha-synuclein defect
188
What is seen on EEG with Creutzfeldt-Jakob disease (CJD)?
Spike wave complexes
189
What builds up in the brain in CJD?
Prions (beta pleated sheets resistant to proteases)
190
What is the most common cause of dementia in the elderly?
Azheimer
191
What is the second most common cause of dementia in the elderly?
Multi-infarct
192
What is a very vulnerable area of the brain to infarct?
Hippocampus
193
Where is the defect in restless leg syndrome (RLS)?
Dopaminergic pathway
194
What diseases is RLS associated with?
CKD and DM
195
What is the treatment of RLS?
DA agonists (ropinirole, pramipexole)
196
Which HLA type is MS associated with?
HLA DR2
197
What are the CSF findings in a patient with MS?
Increased lymphs, increased IgG with oligoclonal IgG bands, and myelin basic protein
198
What is the gold standard for diagnosis of MS?
MRI - periventricular plaques
199
What are three treatment options for MS?
Beta-interferon, immunosuppression, and natalizumab.
200
What is acute inflammatory demyelinating polyradiculopathy?
Autoimmune condition that destroys Schwann cells. (Guillain-Barre) Results in ascending weakness/paralysis.
201
Which bug causes subacute sclerosing panencephalitis?
Measles
202
Lesions of the subthalamic nucleus usually cause what?
Contralateral hemiballismus
203
Homovanillic acid is a metabolite of what?
DA
204
Low CSF hydroxyindole-acetic acid has been associated with what?
Impulsive, destructive behaviors
205
Elevated CSF levels of 14-3-3 protein are helpful for diagnosing what?
CJD
206
Progressive multifocal leukoencephalopathy (PML) is associated with which virus?
JC virus
207
Increased risk of PML is seen with which medication?
Natalizumab
208
What is acute disseminated (postinfectious) encephalomyelitis?
Multifocal perivenular inflammation and demyelination after infection (commonly measles or VZV) or certain vaccinations
209
What is Metachromatic Leukodystrophy and what is its inheritance pattern?
An autosomal recessive lysosomal storage disease, most commonly due to arylsulfatase A deficiency. Buildup of sulfatides leads to impaired production of myelin sheath.
210
What are the findings of Metachromatic Leukodystrophy?
Central and peripheral demyelination with ataxia, dementia.
211
What is Charcot-Marie-Tooth?
A group of inherited (usu autosomal dominant) progressive nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves or myelin sheath
212
What is Krabbe disease?
Autosomal recessive lysosomal storage disease due to deficiency of galactocerebrosidase. Buildup of galactocerebroside and psychosine in macrophages destroys myelin sheath.
213
What are the findings of Krabbe disease?
Peripheral neuropathy, developmental delay, optic atrophy, globoid cells
214
What is adrenoleukodystrophy (ALD)? What's its inheritance pattern?
X-linked genetic disorder usu in males. Disrupts metabolism of very-long-chain fatty acids --> excessive buildup in nervous system, adrenal gland, and testes. Can lead to long-term coma/death and adrenal crisis.
215
What is the difference between simple and complex seizure?
Consciousness (lose it in complex)
216
What is status epilepticus?
Continuous seizure for >30min or recurrent seizures without regaining consciousness between seizures for >30min. Medical emergency.
217
What is a proper treatment for cluster headaches?
Sumatriptan
218
What is a proper treatment for migraine headaches?
Abortive therapies (triptans, NSAIDs), Prophylactic therapies (propranolol, topiramate, CCBs, amitriptyline)
219
What is the difference between peripheral and central vertigo?
Peripheral is more common and involves the inner ear. Central is a brainstem or cerebellar lesion.
220
What is Sturge-Weber syndrome? What are all of its manifestations? *this is a long one...
Congenital non-inherited developmental anomaly of neural crest derivatives due to activating mutation of GNAQ gene. Affects small blood vessels --> port wine stain of face in CN V1/V2 distribution; ipsilateral leptomeningeal angioma --> seizures/epilepsy; intellectual disability; episcleral hemangioma --> increased IOP --> early onset glaucoma.
221
What are all the manifestations of tuberous sclerosis? (Hint: use mnemonic HAMARTOMAS)
Hamartomas in CNS and skin, Angiofibromas, Mitral regurgitation, Ash-leaf spots, cardiac Rhabdomyoma, (Tuberous sclerosis), autosomal dOminant, Mental retardation, renal Angiomyolipoma, Seizures, Shagreen patches.
222
Patients with tuberous sclerosis have increased incidence of what?
Subependymal astrocytomas and ungual fibromas
223
What are the clinical findings of NF1?
Cafe-au-lait spots, Lisch nodules, neurofibromas in skin, optic gliomas, pheochromocytomas.
224
What is the cause and inheritance pattern of NF1?
Mutation NF1 tumor suppressor gene - neurofibromin, a negative regulator of Ras) on chr 17; autosomal dominant
225
What are the clinical findings of von Hippel-Lindau disease?
Cavernous hemangiomas in skin, mucosa, organs; bilateral renal cell carcinomas; hemangioblastoma in retina, brain stem, cerebellum; and pheochromocytomas
226
What is the cause and inheritance pattern of von Hippel-Lindau?
Autosomal dominant; mutated VHL tumor suppressor gene on chr 3, which results in a constitutive expression of HIF (transcription factor) and activation of angiogenic growth factors
227
About what percent of brain tumors are primary?
50%
228
"Pseudopalisading" is a buzzword for which brain tumor?
Glioblastoma multiforme
229
A meningioma arises from which cells?
Arachnoid cells
230
Which type of brain tumor occurs more commonly in females?
Meningioma (expresses estrogen R)
231
Which brain tumor can produce EPO and lead to polycythemia?
Hemangioblastoma
232
Schwannomas are usually found where?
Cerebellopontine angle
233
Which brain tumor is S-100 positive?
Schwannoma
234
Bilateral acoustic schwannomas are found in what disease?
NF-2
235
"Chicken-wire capillary pattern" is a buzzword for what brain tumor?
Oligodendroglioma
236
"Rosenthal fibers" are a buzzword for what brain tumor?
Pilocytic astrocytoma
237
Which two brain tumors are GFAP positive?
Glioblastoma and Pilocytic astrocytoma
238
"Homer-Wright rosettes" are a buzzword for what brain tumor?
Medulloblastoma
239
What passes through the foramen magnum?
Spinal roots of CN XI, brain stem, vertebral arteries
240
What passes through the foramen rotundum?
CN V2 (maxillary)
241
What passes through the foramen ovale?
CN V3 (mandibular)
242
What passes through the jugular foramen?
CNs IX, X, XI, jugular vein
243
What passes through the cribriform plate?
CN I olfactory bundles
244
What passes through the optic canal?
CN II, ophthalmic artery, central retinal vein
245
What passes through the superior orbital fissure?
CNs III, IV, V1, VI, ophthalmic vein, sympathetic fibers
246
What passes through the foramen spinosum?
Middle meningeal artery and vein
247
What passes through the internal acoustic meatus?
CNs VII and VIII
248
What passes through the hypoglossal canal?
CN XII
249
What is pseudobulbar palsy?
Caused by neuro disorders such as MS; Dysarthria, dysphagia, dysphonia, and impaired movement of tongue and facial muscles
250
What is Eaten-Lambert syndrome and what disease process is it associated with?
An autoimmune disorder of the NMJ seen primarily in patients with small cell lung cancer
251
Conduction aphasia is a result of a lesion where?
Arcuate fasciculus (insula)
252
Transcortical motor aphasia is due to a lesion where?
Near Broca area
253
Transcortical sensory aphasia is due to a lesion where?
Near Wernicke area
254
Which nerves are unmyelinated?
Group C nerve fibers: Autonomic postganglionic nerves, afferent neurons that conduct heat sensation, afferent neurons that transmit slow-onset dull, burning or visceral pain, and first order bipolar sensory neurons of olfaction
255
What are the consequences of a left frontal lobe lesion?
Apathy and depression
256
What are the consequences of a right frontal lobe lesion?
Disinhibited behavior
