board prep Flashcards
1
Q
DSM V criteria for persistent depressive disorder?
A
Persistent depressive disorder is a new term in the DSM-5 that consolidates chronic major depressive disorder and dysthymic disorder. Depressive symptoms must be present for at least 2 years to meet the diagnostic criteria (1 year for children and adolescents), with no longer than 2 months of symptom freedom at a time. Symptoms include poor appetite or overeating, insomnia or hypersomnia, low energy or fatigue, low self-esteem, poor concentration or difficulty making decisions, and feelings of hopelessness.
2
Q
Presentation of SMA type 1? (5) Associated gene to test for?
A
ormal neonatal course followed by the development of fasciculations, hypotonia, respiratory failure, and areflexia. SMN1 gene
3
Q
Difference between brief psychotic disorder, schizophreniform disorder and schizophrenia?
A
The duration of symptoms dictates the diagnosis: symptoms lasting <1 month are consistent with a brief psychotic disorder; symptoms lasting 1 to 6 months are considered a schizophreniform disorder; and symptoms lasting >6 months are considered schizophrenia.
4
Q
What is schizoaffective disorder?
A
combination of schizophrenia and a mood disorder; the psychosis must be present for at least 2 weeks when the mood disorder is NOT present. And mood disorder (bipolar or depression) should be present more than 50% of the time.
5
Q
What is Leber Hereditary Optic Neuropathy?
A
condition due to point mutation on mitochondrial DNA; patient presents with progressive vision loss due to recurrent optic neuropathy; fundoscopic exam shows bilateral optic disc hyperemia and circumpapillary telangiectasia
6
Q
Triad seen in Balint’s syndrome? Which part of the brain is damaged?
A
-ocular apraxia (cannot initiate saccades) , ocular ataxia (trouble with fine movements under visual guidance), and simultagnosia (difficulty interpreting complex visual stimuli); -damage to bilateral parietal-occipital association areas
7
Q
How to distinguish between Alzheimer’s disease and Dementia with Lew Body on FDG-PET scan?
A
decreased metabolic activity in the occipital lobe is seen in DLB, and not in AD
8
Q
Describe Corticobasal ganglionic degeneration?
A
parkinonism-plus syndrome with focal limb rigidity, dystonia, cortical myoclonus, cortical sensory loss, astereognosis, agraphestthesia, 2 point discrimination loss in affected limb, frontal cognitive impairment, apraxia, parkinsonism, and alien limb syndrome. May also have rest tremor, bradykinesia, and rigidity.
9
Q
What is exploding head syndrome?
A
sense of a sudden noise or explosion occurring while falling asleep or when waking after sleep onset. Not associated with pain. +/- myoclonic jerk
10
Q
Name the brainstem syndrome: ipsilateral 3rd nerve palsy and contralateral ataxia and tremor
A
Claude’s syndrome
11
Q
Name the brainstem syndrome: contralateral hemiparesis and ipsilateral 3rd nerve palsy
A
Weber syndrome
12
Q
Name the brainstem syndrome: third nerve palsy with contralateral choreiform movements
A
Benedickt’s syndrome
13
Q
four signs of late onset hypoventilation syndrome with hypothalamic dysfunction?
A
hypothalamic endocrine abnormality, behavioral distrubance, tumor of neural origin, obesity
14
Q
Identify the nerve/root involved? numbness in the quadriceps area
A
femoral nerve
15
Q
Identify the nerve/root involved? weakness of quadriceps?
A
L3 and/or L4 neuropathy
16
Q
Identify the nerve/root involved? weakness of hip flexion?
A
femora; nerve and the L1-L3 nerve roots which innervate the iliopsoas muscle
17
Q
Identify the nerve/root involved?absent achilles reflex
A
S1
18
Q
Identify the nerve/root involved? Foot drop and trendelenburg sign
A
L5 innervates the foot dorsiflexors and hip abductors
19
Q
What is septo-optic dysplasia?
A
absence or dysgenesis of the septum pellucidum with hypoplasia of the optic nerve
20
Q
Most evidenced base therapy for conduct disorder?
A
multisystemic therapy is an intensive family-focused and community-based txt program
21
Q
Name the dx: 5 yo boy presents with vision and hearing impairment, aphasia, hyperactivity, paralysis, seizures, muscle weakness, adrenal failure, and coma.
A
adrenoleukodystrophy -X-linked disorder of peroxisomal fatty acid beta-oxidation, which ersults in accumulation of very long-chain fatty acids in tissues throughout the body -tissues most severely affected by the VLCFA are myelin in the CNS and adrenal cortex
22
Q
What Piaget’s 4 major stages of cognitive development?
A
I. Sensorimotor stage (age 0 to 24 months): Knowledge is acquired through sensory experience and manipulating objects. II. Preoperational stage (2 to 7 years): Knowledge is acquired through pretend play. Children begin to think symbolically and use words and pictures to represent objects. During this stage, children have difficulty seeing things from others’ perspective. They use concrete thinking. III. Concrete operational stage (7 to 11 years): Thinking becomes more logical but is still very concrete. Thinking is also more organized. Children start to use reasoning from specific situations to a general principle. Children began to understand others’ perspectives. IV. Formal operational stage (11 years to adulthood): The ability to think logically increases, and abstract thinking emerges. Children gain the ability to use deductive reasoning and are able to derive multiple solutions to problems.
23
Q
Human leukocyte antigen (HLA) B51 serotype is associated with
A
Behçet’s disease, which is characterized by herpetiform rash and iritis, and can cause chronic meningitis.
24
Q
What is the intermediate nerve?
A
branch of CN 7. Taste sensations travel from the anterior 2/3 of the tongue via the chordae tympani and then to the intermediate nerve, and from the palate via the greater petrosal nerve.
25
What nerves supply efferent secretomotor signal to the parotid gland?
The otic ganglion contains fibers supplying efferent secretomotor signal to the parotid gland, via the lesser petrosal nerve, and ultimately from the glossopharyngeal nerve.
26
What nerve provides secretomotor signals to the lacrimal gland?
The greater (superficial) petrosal nerve provide secretomotor signals to the lacrimal gland, via the facial nerve.
27
Balint's syndrome localizes to...
bilateral occipital parietal lobes
28
Triad of Balint's syndrome?
optic apraxia (difficulty looking at a target when prompted) and simultagnosia (difficulty identifying objects when shown simultaneously)
29
What is Hyperekplexia
rare genetic disease that has been associated with a variety of gene mutations usually affecting the glycine receptor. The disease is characterized by a triad of generalized stiffness while awake, nocturnal myoclonus, and an exaggerated startle reflex. These features are often apparent at birth. Episodes of hypertonia or tonic spasms occur upon awakening or with auditory or tactile stimuli.
30
X-linked adrenoleukodystrophy
associated with mutations in the ABCD1 gene. The MRI shows hyperintense T2 changes in the white matter involving the parieto-occipital regions and the corpus callosum.
31
Which aspect of memory is typically spared in pseudo-dementia?
Pseudo-dementia, now known as dementia syndrome of depression, is likely to spare implicit memory.
32
Name the gene associated with: Frontotemporal dementia-amyotrophic lateral sclerosis (FTD/ALS) complex
C9orf72 gene
33
Name the gene associated with: prion disease
PRNP gene (PRioN Protein)
34
Name the gene associated with: Pantothenate kinase-associated neurodegeneration (PKAN)
PANK2 -is a neurodegenerative condition caused by excessive accumulation of iron in the basal ganglia, leading to dystonia, parkinsonism, early dementia, and death
35
Hypokalemic periodic paralysis is a disorder caused by mutation of what channel?
Hypokalemic periodic paralysis is a disorder of potassium transport, usually caused by defective calcium channels
36
Difference between chiari I and chiari II malformation?
Chiari I malformation is characterized by abnormally shaped cerebellar tonsils that are displaced below the level of the foramen magnum. It usually does not become symptomatic until adolescence at the earliest. Symptom onset is usually insidious and can include increased intracranial pressure, occipital headache, and neck pain. Chiari II: Chiari II malformation is characterized by downward displacement of the cerebellar vermis and tonsils, a brainstem malformation with beaked midbrain on neuroimaging, and a spinal myelomeningocele. Diagnosis should be suspected in a baby with clinical evidence of a spinal myelomeningocele.
37
Sleep spindles are a normal sleep transient visualized during stage 2 sleep and are an indication of healthy cerebral architecture in the maturing pediatric brain, becoming increasingly synchronous by what age?
1 to 2 years old
38
Classify Chiari type I based on tonsillar descent (mm)
39
What is Hallervorden Spatz Disease?
NBIA type 1; extrapyramidal dysfunction and dementia in children before 10 years old; has been linked to PANK2 gene on chr 20;
40
Difference between schizencephaly and porencephaly?
* Schizencephaly: cleft between the ventricles and subarachnoid space with associated gray matter heterotopias along the cleft wall (associated with in utero hypoxic insult)
* Porencephaly: cystic communication lined by giotic white matter between the lateral ventricle and the subarachnoid space
41
Name the structure that the arrow is pointing at?
Globus pallidus (in this case hemorrhagic secondary to carbon monoxide poisoning)
42
Describe the baroreceptor reflex?
Baroreceptors in the carotid sinus --\> stimulated by increased blood pressure --\> glossopharyngeal afferents --\> the caudal portion of the nucleus solitarius --\> dorsal motor n. of the vagus nerve --\> parasympathetics to the heart and slow down HR
43
Describe the function and innervation of the genioglossus muscles?
Genioglossus muscles pull the tongue anterior and medial. They are innervated by contralateral hypoglossus nucleus. Thus, if tongue deviation is due to an upper motor neuron lesion affecting the genioglossus projections, tongue deviation would be contralateral. A LMN lesion would cause ipsilateral tongue deivation
44
The gag reflex is mediated by which nucleus?
Nucleus ambiguus
45
What three nerves exits the skull through the jugular foramen?
vagus nerve, glossopharyngeal nerve, spinal accesory nerve
46
Name the antibody.... neuroblastoma + opsoclonus
anti-Hu
47
ataxia syndrome, paraneoplastic cerebellar degeneration. Name the antibody?
anti-Yo (anti-purkinje cell)
48
opsoclonus-myoclonus + breast cancer or lung cancer or gynecological cancer. Name the antibody?
anti-Ri
49
testicular tumors + peripheral neuropathy, name the associated antibody?
anti-Ma2 antibody
50
What MS medication carries an increase risk of seizures?
Dalfampridine
51
How would a lesion at the geniculate ganglion of CN 7 present? How about a pesion just distal to the geniculate ganglion?
**lesion of geniculate ganglion:** affect all modalities of CN 7 since there are no branches prior to it (facial paralysis, hyperacuisis, loss of taste anterior 2/3, loss of lacrimation, loss of salivation)
**lesion just distal to geniculate ganglion:** spares lacrimation since the greater petrosal nerve branches before this point
52
greater than 70% of astrocytomas have mutations in what 2 genes
p53 and ATRX genes
53
molecular marker of oligodendroglioma?
chromosome 1p/19q co-deletion (this is a good prognostic factor)
54
pathophysiology of congenital myasthenia gravis?
mutation in nicotinic acetylcholine receptors
55
Duration of a cluster headache?
15 to 180 minutes
56
57
58
How does intermittent explosive disorder differs from antisocial perdsonality disorder/conduct disorder?
Intermittent explosive disorder = pt has aggressive olutbursts lasting 30 mins that are disproprtionate to stressor affecting day to day life.
There is a lack of personal gain unlike antisocial personality disorder or conduct disorder
59
What is Foster Kennedy syndrome?
p/w ipsilateral optic atrophy and contralateral papilledema. Possible etiology includes olfactory groove meningioma.
60
What is the cell of origin of a meningioma?
arachnoid cap cells
61
localization of fencer's posture
frontal lobe contralateral to the arm that is abducted, externally rotated and flexed
62
What is fukuyama-type congenital muscular dystrophy?
congenital muscular dystrophy in Japanese population; mutation of futukin gene causing disruption of connections between cytoskeleton and basal lamina
63
Name the disease: progressive degeneration of motor neurons in spinal cord; presents as infant with progressive hypotonia who is unable to sit, dificulty swallowing, dyspnea, and absent or diminished reflexes
SMA 1
64
name the disease: progressive neuromuscular disease caused by degeneration of motor neurons of the spinal cord; presents as a child who is able to sit but cannot stand or walk
SMA2
65
66
Name 3 genes and their chromosomes identified in early onset AD?
PSEN2 on chromosome 1
PSEN1 on chromosome 14
APP gene on chromosome 21
67
fluent aphasia with inability to name or write word representing an item; circumlocution, repetition and comprehension are preserved.
Name the type of aphasia?
anomic aphasia
68
localization of apraxia
non-dominant parietal lobe
69
mechanism of action of Memantine?
noncompetitive NMDA receptor antagonist
70
HSAN type 2?
AR disorder that results in loss of pain, temperature, touch and pressure sensation in infancy. DTRs are depressed. +/- hypotonia
71
HSAN3?
aka familial dysautonomia or Riley-Day syndrome
AR, progressive sensorimotor neuropathy with autonomic features; onset in infancy; ulcerds, loss of reflexes, hypotonia, dysphagia
72
HSAN type 4?
AR; congenital insensitivity to pain with anhidrosis,onset infancy
p/w hyperthermia, microcephaly, self mutilation and injury
73
What is mononeuritis multiplex?
subacute onset of asymmetric weakness, typically beginning in the distal hands, WITHOUT sensory involvement
74
ICP waveforms: explain A waves, B waves and C waves
A waves (aka plateau waves) are pathological; loss of internal intracranial compliance and faulty autoregulation; 50-100 mmHg and can last 20 mins
B waves are related to respiratory cycle; 20-30 mmHg
C waves are related to the cardiac cycle (related to arterial pressure)
75
What is Brun's nystagmus?
gazed evoked and vestibular nystagmus associated with large cerebropontine angle tumors
76
Ocular bobbing nystagmus localizes to...
the pons
77
Which TCAs have less anticholinergic side effects?
Nortriptylline and Desipramine
78
This nerve provides sensory innervation to the dorsal first web space of the foot?
Deep peroneal nerve
79
Role of the superior colliculus vs inferior colliculus?
The choclear nerve auditory pathway involves the inferior colliculus.
The superior colliculus is involved in the visual reflex center.
80
Mutation of the DCX gene can lead to?
abnormal migration of a group of neurons which can lead to:
1. subcortical band heterotopia (double cortex)
2. Lissencephaly
81
Prosapagnosia localizes to:
the fusiform gyrus
82
What are wickets
typically bilateral, or unilateral sometimes, are in the temporal region only and are 6-11 Hz. These occur while awake or in light sleep.
83
genetic mutation commonly seen in patients with hereditary neuropathy with liability to pressure palsy (HNPP)?
Deletion of PMP 22
84
Treatment of hypnic headache
Lithium and caffeine
85
86
Presentation of L1CAM gene mutation? (4)
congenital aqueductal stenosis --\> leads to hydrocephalus, adducted thumbs, corpus callosal abnormalities and spastic paraplegia
87
How to make the clinical diagnosis of myoclonic epilepsy with ragged red fibers? (What are the four main features of this disease?)
myoclonus, generalized epilepsy, ataxia, and ragged red fibers (RRF) in the muscle biopsy
https://www.ncbi.nlm.nih.gov/pubmed/20301693
88
Symptoms of TCA overdose?
anticholinergic effects: flushing, dilated pupils, hypotension, hyperthermia, tachycardia, ileus, urinary retention
antihistamine effects: decreased LOC and delirium
GABA receptor inhibition: seizures
89
X linked bulbospinal muscular atrophy (aka Kennedy's disease):
genetic abnormality? presenting symptoms?
CAG expansion on androgen receptor chrXq12
presents with LMN findings including facial/tongue fasciculations, myopathy, elevated CK. Can have transaminitis from myogenic source.
Dysfunctional androgen receptor: gynecomastia and infertility
90
**What is Coat’s disease?**
It's when you have FSHD symptoms that are also associated with vascular retinal degeneration and deafness .
91
**What is diabetic amyotrophy?**
it is also known as diabetic lumbosacral radiculoplexus neuropathy. it is a vasculitic axonal neuropathy that presents with severe unilateral thigh pain . It can extend distally and spread contralaterally. it is followed by progressive atrophy and weakness . It is unrelated to glycemic control or duration of diabetes .
92
**Which charcot Marie tooth disease are associated with acute episodes of ataxia, dysarthria, and plus or minus a symmetric weakness ?**
CMT 1X; white matter changes are evident within the posterior fossa during these episodes . They are triggered by metabolic stressors .
93
**Genetic defect in CMT1x?**
Mutation of the GJB1 gene that encodes protein connexin-32.
94
## Footnote
**Describe the typical presentation of CIDP. How long do symptoms have to be present?**
classical CIDP typically presents with symmetric proximal and distal weakness and numbness . Symptoms must be present for greater than 8 weeks to make the diagnosis.
95
**What are the results of slow repetitive nerve stimulation in a patient with myasthenia gravis ?**
Repetitive stimulation causing decrement of CMAP amplitudes with slow repetitive nerve stimulation (2-3Hz) with a decrement are greater than 10% is consistent with myasthenia gravis .
96
Presentation of DM1 vs DM2?
DM1 can present at any age including congenital myotonic dystrophy . The main symptoms are distal muscle weakness and myotonia especially in the hands , and also ptosis, facial muscle weakness, and temporal muscle wasting . DM2 is adult onset with proximal muscle weakness, myalgia, and less myotonia . It does not have a congenital form. both can cause frontal baldness , cataracts , cardiac conduction defects and central sleep apnea.
97
**How does multi focal acquired demyelinating sensory motor neuropathy present?**
MADSAM presents with asymmetric motor and sensory loss with conduction block and features of demyelination that start with the distal upper limbs. It is a type of CIDP .
98
What are the similarities and differences between Walker warberg syndrome and muscle-eye-brain disease?
Walker Warberg syndrome and muscle eye brain disease both have muscular dystrophy with cerebral and eye malformation. In Walker Warberg syndrome, retinal and eye malformations are severe and can include cataracts, retinal dysplasia, optic atrophy.
muscle eye brain disease: less severe findings (ie myopia)
Both have cobblestone lissencephaly
99
What is a primary prevention strategy of stroke in children with sickle cell disease?
Chronic exchange transfusions therapy
100
Which charcot Marie tooth disease has early involvement of hands?
CMT2D
101
Which extraocular muscle is most commonly and severely affected in myastenia gravis?
Medial rectus muscle is most commonly and severely affected in myasthenia gravis. Upgaze can cause can cause ptosis and weakness of the medial rectus muscle which can then lead to a pseudo INO on subsequent lateral gaze.
102
How to differentiate early myoclonic encephalopathy and ohtahara syndrome?
ohtahara syndrome: shows burst suppression pattern on EEG in both wakefulness and sleep.
early myoclonic encephalopathy: has burst suppresion pattern on EEG in sleep only
103
What is erythromelegia syndrome?
It is caused by a mutation in SCN9A which leads to hyper-excitability of the peripheral sensory neurons. Anything that causes increased temperature can trigger episodes where the area becomes hot, red, and painful
104
Which fibers are involved in small fiber neuropathy?
Myelinated A delta fibers and unmyelinated C fibers
105
What are the EMG findings in LEMS with both low frequency repetitive stimulation and high frequency repetitive stimulation?
low CMAPs +
low freq rep stim: decrement
high freq rep stim: increment
106
EMG findings of McArdle's?
electrical silence
107
What is aphemia?
Aphemia is apraxia of speech, commonly known as a pure word mutism, or anarthria. Patients will present with a complete inability to speak, but written language and verbal comprehension are intact. Lesions to the dominant premotor cortex, insula and Broca’s area have been observed to cause aphemia.
Reference: Ottomeyer, Caroline, et al. “Aphemia: an Isolated Disorder of Speech Associated with an Ischemic Lesion of the Left Precentral Gyrus.” Journal of Neurology, vol. 256, no. 7, Jan. 2009, pp. 1166–1168., doi:10.1007/s00415-009-5055-0.
108
What type of seizures are activated in REM sleep?
NONE; REM sleep is protective! However seizures can most commonly occur during stage 2 sleep
109
Triad of congenital toxoplasmosis?
Intracranial calcifications
Hydrocephalus
Choreoretinitis
110
What is the Landau reflex and by what age does it disappear?
superman (extends arms and legs) seen in infants when held horizontally in the air in the prone position. Disappears by age 2
111
What type of neuropathy is critical illness neuropathy?
b/l symmetrical motor and senosry axonal neuropathy
112
What is another name for parasonage turner syndrome?
neuralgic amyotrophy
113
What antibody in polymyositis is associated with a negative risk for malignancy?
antibodies to extractable nuclear antigens (anti-ENA) are associated with a negative risk for malignancy
114
genetic defect in hereditary neuralgic amyotrophy?
SEPT9 (chr 17); can have recurrent episodes and otherwise presents similarly to Parsonage turner syndrome
115
What is NARP and Leigh's syndrome?
NARP = neuropathy, ataxia, and retinitis pigmentosa and is secondary to mitochondrial DNA mutation in the MT-ATP 6 gene.
116
localization of acute onset hemiballismus?
contralateral STN
117
infarction of the anterior nucleus of the thalmus presents with?
memory disturbance
118
infarction of the centro-edian nucleus of the thalamus presents with?
coma
119
Hutchinson teeth is seen in?
congenital syphyllis
120
Treatment for lihtium induced tremor?
Propranolol
121
Dandy Walker syndrome
congenital absence of the cerebellar vermis which leads to enlarged 4th ventricle
122
presentation of infantile hydrocephalus?
macrocephaly, frontal bossing, prominent scalp veins, motor delay, Lower extremity spasticity
123
steroid induced myopathy causes atrophy of __ muscle fibers?
type 2 muscle fibers
124
autoantibodies of Myasthenia gravis effect the AChR by?
preventing binding of ACh to AChR, crosslinking and internalizing AChR and complement mediated destruction of AChR
125
Describe the breathing patterns in the following:
1) medullary infarct
2) pontine infarct
3) unilateral hemispheric stroke?
4) high spinal cord lesion?
1) medullary infarct - ataxic breathing (irregular pattern)
2) pontine infarct --\> apneustic breath (sustained deep inspiration followed by rapid excellation)
3) unilateral hemispheric stroke - Cheynne Stoke (crescendo-descrescendo pattern)
4) high spinal cord lesion - rarely can cause complete apnea (poor prognosis)
126
Hemimegalencephaly is an extreme form of?
unilateral cortical dysgenesis with hamartoma
127
Where does the recurrent artery of Huebner originate from and what does it supply?
branch of the ACA; supplies the anterior limb of the internal capsule, inferior part of the caudate, anterior portion of the globus pallidus
128
The thalamus receives its blood supply from?
branches of the PCA and PCOMM
129
Where does the anterior choroidal artery originate from and what does it supply?
originates from the ICA; supplies the posterior limb of the internal capsule; GPi, geniculocalarine tract, and choroid plexus
130
131
What isthe nucleus soltarius? Nucleus ambiguus?
Nucleus soltarius combine afferents from CN 7, 9, and 10 for visceral sensation. Nucleus ambiguus contain the motor neurons of CN 9 and 10 for larynx and pharynx and other thoracic organs.
132
What is the difference between type 1 and type 2 muscle fibers?
Type 1 muscle fibers are slow twitch fibers that depend on aerobic/oxidative metabolism. They are recruited during endurance tasks. Type 2 are fast twich muscle fibers that depend primarily on anaerobic/glycolytic metabolism.
133
Ataxia telangiectasia (genetics, presentation, diagnosed by, treatment)
Caused by mutation of the ATM1 gene on chr 11. It presents with cerebellar ataxia, oculocutaneous telangiectasias. eye movement abnormalities, and immune deficiency with increased risk of infections. It also has interstitial fibrosis, neuromuscular weakness and dysphagia (increased risk of asp PNA).
Diagnosis is made with low serum IgA, increased alpha fetal protein, and genetic testing.
Txt: supportive
134
Identify Papez circuit? (6)
hippocampus, fornix, mamillary bodies, anterior thalamus, cingulate, entorhinal cortex
135
How to test the following types of memory:
immediate memory?
working memory?
recent memory?
remote memory?
immediate memory - forward digit span
working memory - manipulation of information retained in immediate memory (ie reciting a series of numbers backwards)
Recent memory - register and recall specific items after a delay ( needs hippocampus and parahippocampal areas of the medial temporal lobe for storage and retrieval; usually impaired first in AD)
Remote memory - historical life events
136
How to interpret findings on brain PETscan?
green/blue = areas of decreased metabolism
137
Describe the function of the following thalamic nuclei?
## Footnote
anterior nucleus
VPM
pulvinar
dorsomedial
VPL
anterior nucleus - part of Papez circuit and needed for memory formation and limbic relay.
VPM - sensory relay from face
pulvinar - processing visual information and sensory integration
dorsomedial - lesion here causes abulia, anterograde amnesia, social disinhibition
VPL - sensory relay from the body
138
What aspect of memory is affected in TGA?
Recent memory is affected
139
MOA of the following:
1) Donepezil
2) Rivastigmine
3) Galantamine
4) Memantine
1) Donepezil - pure acetylcholinesterase inhibitor
2) Rivastigmine - acetylcholinesterase and butyrylcholinesterase inhibitor
3) Galantamine - acetylcholinesterase inhibitor and allosteric nicotinic modulator
4) Memantine - NMDA antagonist
140
The PrP gene (defective in CJD) is located on chromosome?
20
141
How does bilateral anterior operculum syndrome?
severe dysarthria, bilateral voluntary paralysis of the lower cranial nerves, preserved involuntary emotional innervation
142
Where does prosopagnosia localize to?
bilateral lesions of temporo-occipital regions (bilateral fusiform gri)
143
Topgraphagnosia (def and localization)
defect in spatial orientation; localizes to nondominant posterior parahippocampal region, infracalrine cortex, nondominant parietal lobe
144
Asomatognosia (definition and localization)
indifferent inability to recognize one's own body part
145
Misoplegia
severe hatred of the limb
146
What is aphemia? Where does it localize to and how is it different from Broca's aphasia
Aphemia = pure word mutism (verbal apraxia) and is marked by an inability to speak fluently, impaired repetition, and intact auditory comprehension. In aphemia, there is retained ability to write and comprehend written language unlike Broca's aphasia where the patient cannot speak, write, name or repeat.
localizes to dominant frontal operculum (anterior and superior to Broca's area)
147
pharmcologic treatment of pseudobulbar affect?
dextromethorphan-quinidine
148
What medications can provoke IIH?
isotretinoin and tetracyclines (doxycycline and minocycline).
149
What is hypnic headache? How long does it last for and what is the treatment?
Hypnic headache is a rare primary headache disorder that only occurs during sleep and causes awakening due to pain. For this reason, is it sometimes called “alarm clock headache.” It typically lasts for 15 minutes to 4 hours after awakening. The etiology remains unclear. One unique feature of this headache is that it classically responds best to caffeine and lithium. Evening intake of 100 mg to 200 mg caffeine and 200-600 mg of lithium carbonate is reported to be very effective.
150
What is benign paroxysmal vertigo and benign paroxysmal torticollis?
Not to be confused with benign paroxysmal positional vertigo (BPPV), BPV is felt to be a migraine aura seen in children younger than 6 years of age. Benign paroxysmal torticollis is also felt to be a migraine aura but typically presents with recurrent episodes of head-turning, vomiting, and pallor.
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