BOARD PREP Flashcards
Neurosarcoid involves which part of brain often times?
pituitary/hypothalamus
SCA type 1 vs 2 vs 7?
1 - cerebellar and brain stem atrophy ATXN1 Chi 6 - age 30-40
2 - cerebellar worst
7 - also seizures and myoclonus and RETINAL DEGENERATION - often have visual loss at teenage/early adult
Thallium toxicity vs arsenic?
tx for X linked adrenal leukodystrophy?
If no significant neurological exam findings, psychiatric issues and IQ >80 good candidate
ATP7B?
Wilsons disease
T2 changes BG
PKAN vs ATP7B MRI findings?
Wilsons/ATP shows increased T2 in caudate, putamen and GP – in PKAN (pantothenate kinase associated neurodegeneration lower T2 on globes pallid us/substantia nigra)
PKAN? sx?
PKAN (pantothenate kinase associated neurodegeneration
iron deposition in BG - PD, dystonia
Anti GluR5 and Anti Ma1 and 2 associated with what cancers?
The correct response option is anti-Hu and anti-CV2 because these are the most frequently found onco-neuronal antibodies in patients of limbic encephalitis secondary to paraneoplastic syndrome of primary lung malignancy. Paraneoplastic syndrome secondary to lung malignancy has widespread neurological presentations ranging from encephalomyelitis to cerebellar degeneration. The underlying mechanism is an autoimmune response against shared tumor neuronal antigens. Anti-Hu and CV2 (CRMP-5) are the most prevalent antibodies in patients with paraneoplastic limbic encephalitis. Other commonly found antibodies include anti-Yo, anti-ANNA, anti-VGKC, and anti-GABA.
Anti-NDMAR is incorrect because anti-N-methyl-D-aspartate receptor encephalitis is not associated with paraneoplastic syndrome secondary to primary lung malignancy. Anti-NDMAR encephalitis has been infrequently reported with lung malignancies but the association remains very rare. The most commonly associated malignancy with anti-NDMAR encephalitis is an ovarian teratoma. About 58% of the women with anti-NDMAR encephalitis were found to have an ovarian teratoma.
Anti-GAD65 is incorrect because synaptic enzyme glutamic acid decarboxylase (GAD) associated limbic encephalitis occurs in young women and is usually non-paraneoplastic, i.e. autoimmune associated with connective tissue disorders or epileptic syndromes. However, literature does report anti-GAD65 limbic encephalitis secondary to the paraneoplastic syndrome in patients with kidney, breast, colon, and thymus carcinomas. Anti-GAD65 encephalitis is reported to be associated with the paraneoplastic syndrome of primary lung malignancy but again it is very rare and other onco-neuronal antibodies are more commonly found in limbic encephalitis associated with lung cancer.
Anti-Ma1 and anti-Ma2 is incorrect because these are not commonly found in limbic encephalitis associated with the paraneoplastic syndrome of primary lung malignancy. The most common tumor precipitating anti-Ma1 encephalitis is breast, colon, and testicular cancers presenting with limbic encephalitis. Testicular cancer is the most frequent tumor associated with anti-Ma2 encephalitis. Lung malignancy has been reported rarely associated with anti-Ma1 but not with anti-Ma2. Still, these antibodies are not the most likely in the majority of patients with paraneoplastic syndrome causing limbic encephalitis secondary to primary lung malignancy.
Anti-mGLuR5 is incorrect because metabotropic glutamate receptor 5 (mGluR5) antibody-associated encephalitis occurs most commonly with Hodgkin’s lymphoma. The encephalitis of anti-mGLuR5 is not restricted to the limbic region and the majority of patients have gross brain involvement. Very rarely, lung malignancy has been reported with anti-mGLuR5 encephalitis but the incidence remains unknown.
pre optic nucleus function?
The preoptic nucleus in the anterior hypothalamus is the primary thermoregulator of the body. It receives afferent input from thermoreceptors in the skin, which in turn control the activity of organs involved in thermoregulation. The cooling response is controlled by the anterior hypothalamic nucleus, and the warming response is controlled by the posterior hypothalamic nucleus. Damage to the preoptic nucleus can result in either hypothermia or hyperthermia. Additionally, the preoptic nucleus also secretes the gonadotropin-releasing hormone.
Thematic Apperception test?
The TAT is a projective test assessing the person’s interpretation of illustrations of ambiguous interpersonal situations. Thus, it sheds light on a person’s interpersonal function and can also identify psychotic processes in the case of idiosyncratic interpretations.
Associated w/ PLP1 gene? Symptoms?
Pelizaeus-Merzbacher disease is caused by mutations of the PLP1 gene located on the long arm of the X chromosome. The most common mutation, duplication of the PLP1 gene, has been proposed to be caused by defective DNA replication. Clinical signs and symptoms are varied. Usually, nystagmus is of a pendular nature; it can often have horizontal and rotatory components. Over 95% of patients have nystagmus. This sign may disappear later during childhood. The patient’s age at onset of nystagmus alone does not predict clinical severity.
What to give pregnant women if on phenytoin?
folate and vit K prior to delivery
gene associated with hereditary neuralgic amyotrophy?
hereditary neuralgic amyotrophy (HNA). HNA is a rare autosomal dominant condition that presents with recurrent attacks of severe pain, weakness, and sensory symptoms in the distribution of the brachial plexus. It is due to a mutation in the SEPT9 gene on chromosome 17. Patients with this disorder typically first experience attacks when they are in their twenties, but some may begin having problems in their childhood. The pattern of nerve damage is patchy. The pain may be described as burning, stabbing, or aching. Winging of the scapula is common from long thoracic nerve involvement. Individuals may have dysmorphic features, including hypotelorism, epicanthal folds, cleft palate, bifid uvula, facial asymmetry, excess skin folds in the neck, short stature, and partial syndactyly.
Niemann pick disease accumulates what? symptoms? cherry macula?
Niemann-Pick disease is the correct option because this patient’s clinical features (hepatosplenomegaly, cherry-red macular spots, and psychomotor retardation) are consistent with Niemann-Pick disease. This condition is caused by the accumulation of sphingomyelin primarily in lysosomes of the reticuloendothelial system and CNS.
Hunt and Hess scale?
Grade 1 is described as asymptomatic or presence of mild headache with the patient being alert and oriented and minimal if any nuchal rigidity.
Grade 2 is described as the presence of nuchal rigidity, moderate to severe headache in an alert and oriented patient with cranial nerve palsy.
3: Presence of lethargy and confusion with mild focal neurological deficits is the correct answer as it is described as grade 3.
Grade 4 is described as the presence of stupor and severe focal deficits.
Grade 5 is described as the presence of coma with signs of severe neurological impairment, such as decerebrate posturing.
side effects of risperidone? trazodone?
1) The most frequent adverse effect with eszopiclone is unpleasant taste; (2) Constant use of benzodiazepine hypnotic agents should be discouraged in the long-term care setting because of the risk of side effects such as such as memory impairment, falls, excessive daytime sleepiness, and accidents; (3) Side effects of tricyclic antidepressants include anticholinergic effects and varying levels of REM sleep suppression; (4) Potential side effects of trazodone include induction of cardiac arrhythmias in patients with heart disease and orthostatic hypotension; (5) Patients with renal and hepatic insufficiency may be at higher risk for side effects from sedatives; (6) In patients with pre-existing obstructive sleep apnea, hypnotics can produce further nocturnal hypoxemia; (7) The anticholinergic and sedative effects of tricyclic antidepressants and antihistamines may increase cognitive deficits; and (8) Antipsychotic medications can cause orthostatic hypotension, which may increase the risk of falls.
How do the following interact with oral contraceptives?
lamictal
phenytoin
CBZ
topamax
phenobarbital
Lamotrigine is an antiepileptic drug that works by selectively binding to sodium channels, although its exact mechanism of action is not understood. Oral contraceptives reduce the serum concentration of lamotrigine by inducing its metabolism and, therefore, may result in decreased drug efficacy. Phenytoin is a cytochrome P450 inducer that can decrease the serum concentration of oral contraceptives, resulting in reduced efficacy and potential contraception failure. Phenobarbital is a cytochrome P450 inducer and can decrease the serum concentration of oral contraceptives resulting in reduced efficacy and potential failure of contraception. Topiramate is a cytochrome P450 inducer that can decrease the serum concentration of oral contraceptives, resulting in reduced efficacy and potential contraception failure. Carbamazepine is a cytochrome P450 inducer that can decrease the serum concentration of oral contraceptives, resulting in decreased efficacy and potential contraception failure.
landau-kleffner syndrome? Age?
Panayiotopoulos syndrome? age?
Landau-Kleffner syndrome is incorrect. This rare childhood epileptic disorder has a peak occurrence between 5 to 8 years of age. It is characterized by acquired aphasia which can be sudden or progressive. EEG shows spike and sharp waves predominantly over temporoparietal and occipital regions during sleep. Panayiotopoulos syndrome is incorrect. This disorder has a peak incidence between 3–6 years of age. Most of the seizures occur in sleep and are accompanied by autonomic manifestations, such as flushing, thermoregulatory irregularities, hypersalivation, vomiting, and retching.
Fregoli vs Capgras syndrome?
Fregoli - strangers assume role of lost loved one (HYPERfamiliarity)
Capgras - familiar people replaced with imposters (hypo familiarity) - “strangers put a CAP in your loved one”
CD type cells with dermatomyositis vs poly?
dermatologist - CD 4 - fascicular atrophy, F for Four
Poly - CD8
segmental zoster paresis pathophysiology?
Soon after she experienced this rash, motor weakness occurred in the same dermatomal distribution. Such weakness is due to the spread of the virus from the dorsal root ganglion to the anterior horn of the spinal cord.
Mechanism of action:
dipyridamole
aspirin
clopidogrel
cilostazol
ticlopidine
Dipyridamole is the correct answer because its mechanism of action involves inhibiting cyclic nucleotide phosphodiesterase, the enzyme responsible for the uptake of adenosine. When cyclic nucleotide phosphodiesterase is inhibited, the uptake of adenosine is affected, leading to increased levels of cyclic adenosine monophosphate and decreased levels of calcium, which result in inhibition of platelet aggregation.
Aspirin is incorrect as it is an antiplatelet drug that irreversibly inhibits cyclooxygenase which then decreases the synthesis of thromboxane resulting in the prevention of platelet aggregation.
Clopidogrel is incorrect as it prevents the binding of adenosine diphosphate (ADP) to the platelet ADP receptor; it inhibits the activation of glycoprotein IIb/IIIa receptor thus preventing platelet aggregation.
Cilostazol is incorrect as it works by inhibiting phosphodiesterase 3. It is mainly used in claudication and peripheral vascular disease.
Ticlopidine is incorrect because it has a similar mechanism of action as clopidogrel. However, it is reserved for patients who cannot be given other antiplatelet agents because it requires monitoring of white blood cell and platelet count due to the adverse effects that include neutropenia, aplastic anemia, thrombotic thrombocytopenic purpura, and agranulocytosis.
westphal variant of Huntingtons disease?
The Westphal variant of Huntington’s disease develops before the age of 21 years with bradykinesia, dystonia, and parkinsonian features presenting more dominantly than chorea. It is associated with neuropsychiatric manifestations, myoclonus, and epilepsy.
optic gliomas in NF type 1 or 2?
Vestibular schwannomas?
gene product affected?
MRI hemorrhage: T1 and T2
hyper acute <24 hours
Subacute
Chronic
mnemonic for blood on MRI based on time?
Psychological factors affecting a medical condition?
compared to somatic sx?
The core feature of this diagnosis is the presence of psychological or behavioral factors that adversely affect a medical condition by increasing the risk of suffering, disability, or death. These factors must have influenced the course of the medical condition as shown by a close temporal association between the psychological factors and development or exacerbation of, or delayed recovery from, the medical condition.
somatic sx usually don’t have actual medical condition
VPA interact with lamotrigine?
Lamotrigine, a second-generation antiepileptic drug, is used to treat both focal and generalized seizures. It exhibits first order linear pharmacokinetics and is metabolized by the liver. It is metabolically inactivated by UDP-glucuronosyltransferases (UGT), mainly by UGT1A4 and UGT2B7.
When concomitant administration of valproic acid occurs, it leads to a reduction in lamotrigine clearance by 58%. This is due to the interaction of valproic acid with UGT, leading to competitive inhibition of glucuronidation. This, in turn, causes lamotrigine toxicity. Therefore, lamotrigine dosing has to be considerably reduced when valproic acid is added to the regimen. Initiation of valproic acid requires a reduction of lamotrigine dose by 50%.
which SSRI approved for kids for depression? OCD?
Fluoxetine, ages 8 years-old and above, and escitalopram for ages 12 years old and above. Sertraline has FDA approval for obsessive-compulsive disorder but not depression.
Morvan syndrome antibodies?
Morvan syndrome or Morvan’s fibrillary chorea (MFC) is a rare constellation of neurological symptoms, consisting of peripheral nerve hyperexcitability, autonomic instability, and encephalopathy often associated with autoantibodies to voltage-gated potassium channel complexes (VGKCs) and CASPR (CASPR2 (contactin-associated protein 2))
middle meningeal passes through which foramen?
spinous
Diseases w/ alpha syncuclein?
Tau?
ALPHA - Parkinson’s disease (PD), multiple system atrophy, and dementia with Lewy bodies. This patient is most likely suffering from Lewy body dementia.
TAU - Alzheimer’s disease, frontotemporal dementia, progressive supranuclear palsy, argyrophilic grain disease, corticobasal degeneration, and chronic traumatic encephalopathy
tarsal tunnel syndrome nerve? sensory and motor weakness?
This patient most likely has tarsal tunnel syndrome, which can occur from compression of the posterior tibial nerve in the tarsal tunnel, located posterior to the medial malleolus. The most common causes include trauma (e.g., ankle fracture or sprain), external compression from a tight plaster cast or footwear, an enlarged or abnormal structure (e.g., ganglion cyst, arthritic bone spur, or varicose vein), having flat feet, or systemic diseases (e.g., diabetes or hypothyroidism). Compression of the nerve results in weakness and atrophy of intrinsic foot muscles. There is sharp shooting pain over the tarsal tunnel that radiates to the heel or the calf and loss of sensation, tingling, or burning on the plantar surface of the foot. Sciatic nerve is incorrect. A complete sciatic neuropathy results in weakness of knee flexion and all movements of the ankle and toes; it can result in foot drop. Sensations below the knee are also impaired (except the medial part of the leg and foot). The ankle jerk is diminished or absent. Common peroneal nerve is incorrect. Compression of this nerve is associated with foot drop (i.e., weakness of toe and ankle dorsiflexion) and weakness of foot eversion. There is loss of sensation on the lateral shin and dorsum of the foot. Femoral nerve is incorrect. Damage to this nerve results in weakness of hip flexion and leg extension. Sensory loss can be elicited on the anterior aspect of the thigh and medial aspect of the calf. The quadriceps reflex is depressed or absent. Obturator nerve is incorrect. Damage to this nerve leads to weakness in adduction of the thigh and impaired sensation in a small area located on the medial aspect of the thigh.
B6 deficiency neuropathy p[athophysiology?
accumulation of S-adenosylmethionine is part of the underlying pathology of peripheral neuropathy caused by vitamin B6 deficiency
Hypomelanosis of Ito?
linear nevus sebaceous? seizures?
neurocutaneous melanosis?
Incontintia pigmenti?
Sjogren Larrsson?
The correct response is linear nevus sebaceous because the scenario describes a patient with an intellectual disability who has a typical birthmark. Linear nevus sebaceous is a neurocutaneous disorder characterized by intellectual disability, seizures, and a birthmark nevus.
Hypomelanosis of Ito is incorrect because linear hypopigmentation arranged in whorls is found in this condition, in contrast to the findings here. It is also associated with iris hypopigmentation.
Neurocutaneous melanosis is incorrect because hairy skin nevi and leptomeningeal melanosis characterize it. Neurocutaneous melanosis carries a risk of malignancy.
Incontinentia pigmenti is incorrect because, in addition to seizures and intellectual disability, it is also associated with macular dystrophy, microcephaly, and brain dysgenesis.
Sjögren-Larsson syndrome is incorrect because it is an autosomal recessive disorder characterized by skin thickening and fissuring, intellectual disability, juvenile macular dystrophy, seizures, and spasticity.
Wilsons disease inheritance?
AR
with neurogenic bladder, if PVR is <100 what tx with? >100 ml?
<100 - oxybutynin (anticholinergic)
>100 self Cath
Urine complete examination and measuring post-micturition residual volume is the correct option. This patient has presented with symptoms suggestive of neurogenic detrusor overactivity because of chronic spastic paraparesis. It occurs due to damage to the nerves supplying the detrusor muscles. Estimation of the post-micturition residual volume can guide the clinician about further management. The patient should also have a urine complete examination to rule out infection as patients with neurogenic detrusor overactivity (NDO) have frequent urinary tract infections.
shuddering attacks age of onset? resolution? how long last?
Shuddering attacks are recurrent, rapid tremors of the head, shoulder, and trunk lasting 10 seconds. They present at the age of 4-6 months and resolve spontaneously by the age of 8 years.
AED safer w/ lactation? why?
VPA since highly protein bound
Anterior interosseous function? sensory?
Patients present with pain in the proximal forearm, mild paresis of the pronator quadratus, paresis of the flexor digitorum profundus I and II and flexor pollicis longus. Patients are unable to effectively pinch the thumb and index finger together. Common etiologies for the anterior interosseus nerve syndrome include strenuous exercise or trauma (e.g., cutdowns, venipuncture), fibrous band constricting nerve, compression by accessory head of flexor pollicis longus muscle (Gantzer’s muscle), and the nerve is frequently affected in Parsonage-Turner syndrome (neuralgic amyotrophy).
nerve roots that innervate supraspinatus? what is this muscles function?
Suprascapular nerve, C5, C6 is correct. The supraspinatus muscle initiates abduction of the shoulder for the first 15 degrees. The deltoid then abducts the arm up to 90 degrees. The trapezius and serratus anterior muscle then elevate the arm through scapular rotation and abduction beyond 90 degrees. The nerve that supplies the supraspinatus muscle is the suprascapular nerve, whose root is C5, C6.
Germinal matrix scoring?
tx for SMA?
nusinersin - alternative splicing of SNM2 gene
perilymphatic fistula sx?
causes by barotrauma/valsalva
leads to hearing loss, vertigo
Tullio phenomenon - can worsen with valsalva or loud noises
toxicity of methanol affects areas of MRI brain?
CO?
methanol -putamen
CO - think lenticulostriate/deep areas first due to hypoxia
Landua Kleffner?
Landau-Kleffner syndrome is correct. This is a rare childhood epileptic disorder with a peak occurrence between 5 to 8 years of age. It is characterized by acquired aphasia, which can be sudden or progressive. Affected children initially demonstrate age-appropriate development but then fail to respond to commands due to auditory verbal agnosia followed by progressive language deficits. These children also demonstrate behavioral problems, including hyperactivity, outbursts of anger, or anxiety. Electroencephalogram (EEG) shows spike and sharp waves predominantly over the temporoparieto-occipital regions during sleep. Valproic acid and clobazam may be used to treat seizures associated with Landau-Kleffner Syndrome.
Autosomal dominant epilepsy with auditory features
can cause receptive aphasia, focal seizures, typically not altered awareness - the receptive aphasia makes appearance of agitation/confusion
meds that cause ototoxicity?
Loop diuretics, cisplatin, and high-dose aspirin are also associated with ototoxicity. Gentamicin
SCD - tx to reduce risk of stroke? percentage want below for Hgb S?
Transfusion therapy with a goal hemoglobin S less than 30% significantly lowers the recurrent stroke risk compared to no transfusions
Benedikt syndrome
Red nucleus is the correct answer. Benedict syndrome is characterized by oculomotor nerve palsy with contralateral cerebellar ataxia, tremor, corticospinal signs, and sometimes choreoathetosis. It involves the tegmentum of the midbrain, affecting the 3rd cranial nerve, red nucleus, corticospinal tract, and superior cerebellar peduncles below the decussation.
Wallenberg?
Lateral spinothalamic tract, olivocerebellar tract, and spinocerebellar tract are all incorrect as they are involved in Wallenberg syndrome, which is characterized by ipsilateral palsy of cranial nerves V, IX, X, and XI, Horner syndrome, cerebellar ataxia with contralateral loss of pain and temperature sense. It involves the lateral tegmentum of the medulla, the lateral spinothalamic tract, descending pupillodilator fibers, spinocerebellar, and olivocerebellar tracts.
electrodecremental response in infant seen in what disorder? what eeg show?
infantile spasms
hypsarrythmia followed by flattening of eeg
lethargy and hypotonia, with NORMAL anion gap and glucose should concern you for what type of disorder?
urea cycle
what is included in the cavernous sinus? lesion here do you get mitosis or mydriasis?
mitosis - symphonic chain affected
antipsychotics most associated with weight gain?
afferent CN and efferent CN for gag reflex?
nucleus ambiguous motor or sensory?
CN cough reflex?
motor
cough both tenth CN
nerves of mastication are considered somatic efferent or special visceral?
Pantothenate kinase-associated neurodegeneration (PKAN) involves iron deposition in the globus pallidus with a central area of necrosis and gliosis. This has been described on an MRI as the “eye of the tiger” sign.
Alexander disease sx? MRI findings?
Macrocephaly
spasticity, seizures
frontal predominant changes, can have enhancement in gray/white matter
CHARGE syndrome? Gene?
CHD7
hypo or hyperkalemic periodic paralysis have SHORTER/MORE are quest episodes?
HYPER
neurotransmitter made in ventral tegmentum? nucleus accumbent?
nerves in jugular foramen?
IX, X, XI
