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155 Cards in this Set
- Front
- Back
sx in serotonin syndrome but not in NMS
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shivering and myoclonus
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deletion PMP22
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hereditary neuropathy with liability to pressure palsies
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duplication PMP22
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CMT 1a
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orofacial dyskinesias lingual and oral dystonia in a 30-year-old patient chorea and peripheral polyneuropathy.
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neuroacanthocytosis, "thorn" shaped blood cells from membrane dysfunction
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dominant (left) posterior cerebral artery occlusion
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alexia w/o agraphia, complete right homonymous hemianopsia, poor color naming
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leg crossing
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peroneal palsy from weight loss
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nystagmus and mild gait btw ataxic attacks. tx acetazolamide. CACNA1A gene on chromosome 19.
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episodic ataxia 2
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HA frequent (7 to 22 per day)
episodes of unilateral, severe, but short-lasting (5 to 45 minutes) Indomethacin is the treatment of choice. |
Paroxysmal hemicrania
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Unilateral anosmia and optic neuropathy in conjunction with signsof raised ICP (papilledema in the opposite eye) are the clinical features of this disorder, which is most
commonly due |
to a planum sphenoidale meningioma that could involve both the olfactory groove and optic
canal. The other disorders listed would not typically have this constellation of signs and symptoms. Foster-Kennedy syndrome |
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Grade II concussion
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(no loss of consciousness but concussion symptoms greater than 15
minutes) and a more recent Grade III concussion(any loss of consciousness). Guidelines state he should be out of play for 1 week in this setting of recurrent concussion. |
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stiff person syndrome tx
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diazepam
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Anton's syndrome (denial of blindness).
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It is a result of severe acute bilateral injury to the
medial occipital lobes and adjacent association cortex, ischemia PCA. The syndrome has also occured after traumatic injury to the optic nerve with associated bifrontal lobe contusions. |
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Mitoxantrone
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secondary progressive ms
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Duchenne's muscular dystrophy gene?
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which is characterized by X-linked recessive
inheritance and a mutation of the dystrophin gene located on Xp21.2 |
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toxoplasmosis tx
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Pyrimethamine
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tuberous sclerosis
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mutations tuberin and merlin
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congenital inability to decarboxylate leucine, isoleucine, and
valine. There is an associated severe metabolic acidosis and hypoglycemia. Death 1st/2nd wk. opisthotonus. |
Maple syrup urine disease (MSUD) is due to a
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Brain death o2
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Presidents Commission recommeded 100% oxygen ventilation for 10 minutes, followed by passive 100%
oxygen for a period long enough to achive a PCO2 of 60. |
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the most common cause of noncommunicating hydrocephalus.
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normal term infant is 35 cm. A head circumference of 45 cm is greater than the 98th percentile. In the absence of other cerebral malformations, congenital aqueductal stenosis most common
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coprolalia
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tourettes swearing, occurs in late adolescence
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descending paralysis involving
cranial nerves, neck, and shoulder girdle.Sluggish or fatiguable pupils |
botulism. tx: Human botulism immunoglobulin
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centrotemporal sharp waves presents from 2 to 13 years of age
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Benign focal epilepsy childhood. 67% infrequent sz. Carbamez prn
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striking peripheral neuropathy.
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Metachromatic leukodystrophy
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Rett's syndrome has micro or macro-ceph?
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micro
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diseases have megaloencephaly and hyperreflexia.
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Alexander, Canavan, and Tay-Sachs
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status epilept
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phenobarbital load 20mg/kg then additional 5 mg/kg x1 prn.
midazolam 0.2 mg/kg slow IV bolus then 0.75 mCg/kg/min to 10 mCg/kg/min IV drip; pentobarbital 5 mg/kg to 15 mg/kg IV bolus over 1 hour followed by 0.5 mg/kg/h to 3.0 mg/kg/h IV drip. |
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progressive extrapyramidal dysfunction, dementia, gait impairment, dystonic posturing, dysarthria, and loss of memory. Onset is most commonly in late childhood. Fe deposition, eye of the tiger sign.
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pantothenate kinase
deficiency (PKAN). |
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A child from a hilly region in the Upper Midwest presenting
during the late summer with fever, headache, stiff neck, and focal seizures has.... |
La Crosse encephalitis.
|
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newborn with microcephaly, organ
involvement, and subependymal calcifications |
CMV
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an X-linked dominant syndrome, presumed lethal in males, with agenesis of corpus
callosum, retinal lacunae, development disabilities, and frequently associated with infantile spasms. |
Aicardi syndrome is
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Clusters of head drops with flexor spasms of the trunk occuring around meal time in an otherwise healthy
7-month-old with a normal EEG suggest |
benign myoclonus of infancy.
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weakness, ataxia, absent tendon jerks, Babinski sign, abnormal position sense. Romberg sign Hammer toes, pes cavus deformities, and scoliosis
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Friedreich's ataxia
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chronic transfusion to keep hemoglobin S below 30%
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sickle cell dz
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Inc intracran pressure from a toxin (peds)
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lead
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vitamin cause of pseudotumor
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Vitamins A or D can cause
pseudotumor cerebri, |
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BPPV in peds predicts what dz l8r?
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migraines
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generalized, fast (4 Hz to 6 Hz) spike-and-wave complexes.
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juvenile myoclonic epilepsy generalized, fast (4 Hz to 6 Hz) spike-and-wave complexes. Valproic acid is the treatment of
choice and recurrence of seizures is likely to occur if treatment is stopped. |
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congenital myasthenic syndromes
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are not related to an immune process but are caused by genetic defects
affecting the neuromuscular junction. These include defects in acetylcholine synthesis and packaging (familial infantile myasthenia gravis), end-plate deficiency of acetylcholinesterase, acetylcholine receptor deficiency, and the slow channel syndrome. |
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opsoclonus, myoclonus, ataxia syndrome, alternatively called "OMS", "dancing eyes
syndrome" or Kinsbourne's Syndrome) |
OMS: a paraneoplastic manifestation of occult neuroblastoma CT chest, abdomen, and
pelvis using oral contrast |
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driving w/ dementia, clinical dementia rating...
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0.5 eval, 1 strongly consider not driving
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right parietal lobe mass showed a tumor composed of cells with monotonous round nuclei
surrounded by prominent perinuclear halos ("fried egg" apppearance). These features are characteristic of oligodendroglioma. |
These features are characteristic of
oligodendroglioma. |
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a cephalic disorder that refers to the disproportionate enlargement of the occipital horns of the lateral ventricles and is usually diagnosed early after birth due to seizures. I
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Colpocephaly is
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schizencephaly
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brain loss with fluid-filled component entirely lined by heterotopic grey matter (porencephalic cyst is lined mostly by white matter. ) SG, PW = Sharon Gan POW!
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Leptomeningeal enhancement, parenchymal enhancement, and, especially, pituitary stalk/hypothalamic
involvement in a patient with cranial nerve symptoms is characteristic of |
neurosarcoidosis.
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pachygyria
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neuronal migration
|
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T2-weighted sequence (T2-CISS) shows a cluster of vessels on the dorsal aspect of the cord compatible with
venous dilation due to arteriovenous shunting by a dural fistula. |
Foix-Alajouanine
|
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supplies the anteroinferior portion of the caudate nucleus, the
putamen, and the anterior limb of the internal capsule. off the ACA |
recurrent artery of Heubner arises from the A1
segment of the anterior cerebral artery and |
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It is almost always found just posterior to the foramen of Monro in the anterior aspect of the third ventricle, originating from the roof of the ventricle. Because of its location, it can cause obstructive hydrocephalus
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colloid cyst is a cyst containing gelatinous material in the brain.
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_________(tumor) typically produce a large cyst with superficially positioned mural nodule. Most
lesions arise within the temporal or parietal lobes. |
Pleomorphic xanthoastrocytoma
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most common site for hypertensive hemorrhage
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Putamen, also thalamic haemorrhage
pontine haemorrhage cerebellar haemorrhage |
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open ring on MRI
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(Schilder's) variety of multiple sclerosis (large tumor-like in appearance, bilateral.
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pseudorosettes
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ependymoma
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pseudopalisading
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GBM
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fried egg
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oligodendroglioma
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MRI spine: hyperintense signal is seen on both T1-weighted and T2-weighted images,
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Benign lipoma
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The microscopic appearance is that of a capillary-rich neoplasm with abundant foamy cells containing lipid.
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hemangioblastoma The tumor arises independently or in conjunction with von Hippel-Lindau disease.
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tumor: are composed of astrocytes with elongate, bipolar, eosinophilic cell
processes; these tumors often show Rosenthal fibers. |
Pilocytic astrocytomas, also
commonly found in the cerebellum, |
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tumor: commonly found in the cerebellum, composed of astrocytes with elongate, bipolar, eosinophilic cell
processes; these tumors often show Rosenthal fibers. |
Pilocytic astrocytomas,
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Toxoplasmosis resides also in basal ganglia, but then there is usually also mass effect.
________enters the intracranial space through the perivascular spaces, and the organisms are surrounded by gelatinous material that is seen as high signal foci. This correlates with "soap-bubble" abscesses seen grossly. |
The correct answer is cryptococcus infection.
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____is caused by migrational arrest of the
affected neuroblasts. The cause of the incomplete migration is unknown. Three kinds of heterotopic gray matter have been described: (1) nodular (periventricular), (2) laminar (in deep white matter), like in this patient, and (3) band heterotopia (double cortex). |
Heterotopia
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__________________ occurs in Hispanic
individuals and is characterized by intraparenchymal cavernous malformations that can produce seizures, impairment of function, or hemorrhage. The photograph illustrates a multi-channeled vascular malformation composed of closely juxtaposed hyalinized, non-arterial vessels. |
Autosomal dominant cerebral cavernous angioma (malformation) (CCM1) syndrome
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________________ the most common of the neural tube closure defects. Folate supplementation has been
known to lower the recurrence risk of these defects however a recent report also documents the presence of anti-folate receptor antibodies in some pregnancies complicated by neural tube closure defects. |
anencephaly,
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______________ typically produce a large cyst with superficially positioned mural nodule. Most
lesions arise within the temporal or parietal lobes. This lesion is surrounded with minimal hemosiderin rim but no edema, and this excludes abscess and hematomas. |
Pleomorphic xanthoastrocytoma
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The signal characteristics heterogeneous (popcorn).
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cavernous hemangioma
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Megalocephaly (abnormally enlarged head). defective ASPA gene which is responsible for the production of the enzyme aspartoacylase.
early infancy and progress rapidly, may include mental retardation, loss of previously acquired motor skills, feeding difficulties, abnormal muscle tone (i.e., floppiness or stiffness), poor head control, Paralysis, blindness, or seizures may also occur. |
Canavan disease, also called Canavan-Van Bogaert-Bertrand disease, aspartoacylase deficiency or aminoacylase 2 deficiency,[1] is an autosomal recessive[2] degenerative disorder that causes progressive damage to nerve cells in the brain.
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dorsal column PLUS dorsal spinocerebellar columns
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Friedrich's ataxia. autosomal recessive inherited disease that causes progressive damage to the nervous system, resulting in symptoms ranging from gait disturbance to speech problems; it can also lead to heart disease and diabetes.
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intradural, extramedullary mass in the thoracic spine is causing significant cord compression.
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This
represents either neurofibroma or meningioma |
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temp lesion: T1-weighted images and high signal on T2-weighted images. Enhances.
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HSV
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spongiform change of the cerebral cotex . Neuronal loss and
gliosis |
consistent with a prion disease
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coronal images note that the sulci at the high parietal convexity are compressed, as
compared to the markedly dilated posterior extent of the Sylvian fissure. |
communicating hydrocephalus
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is associated with periodic long-interval diffuse discharges in the EEG that
recur every 4 to 15 seconds. |
Subacute sclerosing panencephalitis from measles/rubeola)
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CT and MRI scan findings are highly consistent with Cerebral lesions in _____________ are most frequently found in the parieto-occipital area. On CT scans, parenchymal calcification is found
adjacent to the leptomeningeal lesions in a pericapillary distribution in the fourth cortical layer, possibly secondary to chronic tissue hypoxia. Noncontrast MR images in SWS typically show unilateral cerebral atrophy with enlargement of the ipsilateral subarachnoid spaces and ventricular cavities. Enlarged tubular regions of flow void and gyriform hypointensities (decreased signal on T1-weighted and T2-weighted images) reflect venous collateralization and cortical "serpentine" calcification, respectively |
Sturge-Weber syndrome (SWS).
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Familial frontotemporal dementia chromosome?
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that arises from a mutation on chromosome 17 results in a mutation of the
gene encoding for tau protein. |
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inorganic arsenic, lead, mercury, and thallium intox can cause......
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Peripheral neuropathies can be caused by
|
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Aluminum intoxication
can produce |
encephalopathy but does not produce a neuropathy.
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open
ring, |
a finding characteristic of the large lesion (Schilder's) variety of multiple sclerosis.
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subacute process, with quick worsening
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listeriosis preferencefor the brainstem.
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popcorn
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cavernous anioma
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meningioma, neurofibroma, or schwannoma...do they enhance?
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yes!
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refers to increased perfusion in the periphery of an ischemic area. It is best documented with
PET or SPECT. Some authors have used the term to refer to gray matter enhancement, which is best seen on MRI 3 days to several weeks after a cerebral infarct and results from contrast leaking into the perivascular space in an area of ischemic insult. |
Luxury perfusion
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Toluene abuse or "glue sniffing" Huffing glue causes.....
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as become widespread, especially among children or ... imaging (MRI) findings have shown diffuse white matter demyelination and gliosis (diffuse leukoencephalopathy_
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Dolichoectasia (fusiform aneurysm) most commonly affects the supraclinoid segment of the internal carotid
artery and the basilar artery, and is seen in patients with>>>> |
advanced cerebral atherosclerosis.
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is chronic inflammation of the brain, with infiltration of T lymphocytes into the brain tissue. This affects only one cerebral hemisphere,
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Rasmussen's encephalitis
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Critical care myopathy is an underrecognized disorder characterized by selective loss of ________ from myofibers.
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myosin.
Also known as myosin-losing myopathy, this disorder is most commonly seen in critically ill patients treated with corticosteroids and neuromuscular blockers. |
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methanol poisoning causes damages to _____ part of the brain
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Hemorrhagic necrosis of the putamen is characteristic of
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_________ classically presents with progressive asymmetric apraxias, rigidity, and/or aphasia due
to neuronal loss and atrophy of the peri-Sylvian region. Ballooned neurons and astrocytic plaques with tau-immunoreactivity are a diagnostic feature of _________ |
Corticobasal degeneration
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anti jo
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dermatomyositis
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perifasicular atrophy
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dermatomyositis
|
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Neurogenic atrophy (group atrophy) of skeletal muscle is characteristic of
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spinal
muscular atrophy type I (Werdnig-Hoffman disease). Crappy, most kids die >2 years |
|
The lesions (cysts) most pronounced in the distribution of the anterior and middle cerebral arteries. They are the
result of circulatory disturbances during the latter half of pregnancy or the neonatal period. |
multicystic encephalopathy or multicystic encephalomalacia.
|
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capillary-rich neoplasm with abundant foamy cells containing lipid. This
is a hemangioblastoma. The cell origin of the hemangioblastoma is still unknown, despite many investigative efforts. The tumor arises independently or in conjunction with __________ disease. The most common location is in the cerebellum, but the spinal cord can also be a site of origin. |
von Hippel-Lindau disease.
|
|
__________ syndrome occurs in Hispanic
individuals and is characterized by intraparenchymal cavernous malformations that can produce seizures, impairment of function, or hemorrhage. The photograph illustrates a multi-channeled vascular malformation composed of closely juxtaposed hyalinized, non-arterial vessels. |
Autosomal dominant cerebral cavernous angioma (malformation) (CCM1)
|
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________ infarcts often result in profound apathy, lack of motivational intent, akinetic mutism,
weakness of the lower extremities and urinary incontinence. |
Bilateral anterior
cerebral artery territory |
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periventricular leukomalacia (PVL) occurs at what age
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prematurity
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nucleus of________ of the sacral cord is spared in motor neuron disease
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Onufrowicz
|
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Paraneoplastic cerebellar degneration is most commonly associated with what cancers.....
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ovarian carcinoma and some breast
tumors. It can also be seen with Hodgkin's lymphoma and small cell carcinoma of the lung. |
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_______ nucleus is site of the major cholinergic projections to the cerebral
cortex, and with depopulation of this projection nucleus there is a corresponding reduction in cortical choline acetyltransferase. |
basal nucleus
|
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_________ toxicity has birefringent calcium oxalate deposits in and around blood vessels. Oxalic acid
crystals may be detected in the urine. |
Ethylene glycol
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__________ results from missense mutations of the Notch 3 gene on chromosome 19. The small deep white matter vessels
show replacement of the media by eosinophilic, periodic acid-Schiff positive, Congo red negative, granular material. Ultrastructurally, there is compact electron-dense material known as granular osmophilic material surrounding myocytes in the arterial media. Although the predominant effects of ________ are on the central nervous system, it is a systemic vasculopathy that can be appreciated on skin or nerve biopsy. Vasculitis is not seen in this dz. |
dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
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______ has the following: astrocytic hamartomas
called tubers and candle gutterings are present. Subependymal giant cell astrocytomas are true neoplasms with malignant degeneration. in the vicinity of the foramen of Monro. These tumors may produce CSF obstruction, and they may rarely |
TSclerosis
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_____ is a variant of meningioma that often has aggressive clinical behavior with numerous
recurrences and the potential for metastasis. Grade III. |
Rhabdoid meningioma
|
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The type of edema seen with acute, obstructive, high-pressure hydrocephalus
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is interstitial edema.
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________ edema is seen when high arterial pressures overcome cerebrovascular resistance and drive water across the
capillary walls into the extracellular space. |
Hydrostatic
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_________ is the most frequent type of edema and is seen
around tumors or traumatic lesions. |
Vasogenic edema
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cytotoxic edema is most commonly caused by
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hypoxia.
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Neurosarcoidosis anatomic site most common......
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hypothalamus
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cadasil which chromosome
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19, notch 3
|
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triptans act where?
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5HT1 receptors,
specifically 1b and 1d, resulting in decreased activity in the trigeminovascular system. Drugs acting at adrenergic, specifically beta and alpha, receptors provide prophylactic effects. |
|
____________________ are molecular gateways in sensory systems, an interface between
the environment and the nervous system. Several (channels) transduce thermal, chemical, and mechanical stimuli into inward currents, an essential first step for eliciting thermal and pain sensations. |
Transient receptor potential (TRP) ion channels
|
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Cluster headache It may be triggered by ______ . TX w/ ______ or (other options) acutely. Tx ppx w/ ______.
|
It may be triggered or exacerbated by vasodilating substances such as nitroglycerin, histamine, and
ethanol. Drugs such as triptans, steroids, and dihydroergotamine may also be helpful in acute headache management. Verapamil, lithium, and methysergide can be effective prophylactic agents for cluster headaches. |
|
Cessation of electrical activity due to hyperpolarization that occurs acutely after an ischemic insult is the result
of activation of _____ channels. |
potassium (K+) membrane
|
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Direct dopa agonists: name and SE
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Pramipexole, bromocriptine, , ropinirole, rotigotine, and injectable apomorphine
SE neuropsychiatric hallucinations |
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dopa SE
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dyskinesias
|
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__________________ is a prodrug that is transformed in the liver to a potent agonist of alpha receptors in arteries and veins.
Given its predictable absorption and peak effect, it is the sympathomimetic of choice for treatment of neurogenic orthostatic hypotension. One common side effect is scalp pruritus, a manifestation of alpha adrenergic mediated piloerection. Its most potentially serious side effect is supine hypertension. |
Midodrine
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RLS worse with >>>>, tx w/ >>>>>
|
iron deficiency, uremia, pregnancy,
neuropathy, and possibly other conditions. Among the current treatment options offered for the treatment of RLS, dopaminergic agents cabergoline, lisuride, pergolide, (pramipexole, ropinirole are non-ergot fewer SE), rotigotine, have provided the best evidence for efficacy in symptom relief. |
|
is characterized by progressive loss of pain and temperature sensation,
lancinating pain, and severe generalized autonomic failure. Mutations transthyretin, the protein that transports thyroxine and retinol binding protein. |
Type 1 (Andrade) familial amyloidosis, inherited as an autosomal dominant, is associated with amyloid
deposition in peripheral nerves. It |
|
_________________in classic cases, manifests in early childhood with walking problems due to dystonia of the lower limbs. The dystonia is frequently accompanied by parkinsonian features such as reduced
facial expression or slowing of fine finger movements. Biochemically, the disorder is typically characterized by low levels of the neurotransmitter metabolite homovanillic acid and reduced levels of neopterin and tetrahydrobiopterin (BH4) in the cerebrospinal fluid. This is due to heterozygote mutations of the GTP cyclohydrolase I gene, which is the rate-limiting enzyme in the synthesis of BH4. BH4 is an essential cofactor for tyrosine hydroxylase (TH), the rate-limiting enzyme in the synthesis of dopamine. Reduced levels of BH4 lead to __________________ because of reduced TH activity. Other genes implicated in the pathogenesis of this disorder are the TH gene itself and the parkin gene. |
Dopa-responsive dystonia (DRD),
|
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tx postherpetic neuralgia
|
tx antiviral w/in 72 hrs of onset of rash....post herp neur occurs 1-6 months later, However, tricyclic antidepressants (amitriptyline, nortriptyline, desipramine, and maprotiline), gabapentin,
pregabalin, opioids, and topical lidocaine patches are effective in reducing pain and improving quality of life and should be used in the treatment of postherpetic neuralgia. |
|
___________________ impairs mitochondrial fatty acid
oxidation and oxidative phosphorylation. ____________ raises levels of malonylcarnitine that inhibits CPT1, blocking long chain fatty acid transport into mitochondria. Complex II function is secondarily inhibited. Children have limited carbohydrate stores, and require larger doses of ________________for sedation, thus placing them at higher risk of this syndrome than adults. Inadequate caloric intake before or during the infusion further increases the risk, by leading to inability to meet metabolic demands and suppress fat metabolism. Hemofiltration has been shown to correct the metabolic abnormalities in propofol infusion syndrome. Carbohydrate intakes of 6 mg/kg/min to 8 mg/kg/min during propofol infusion might prevent it. |
The findings are most consistent with propofol infusion syndrome. Propofol. Follow CK, triglyc, lactate.
|
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best way to tx opiod w/drawal:
|
combination of naltrexone, clonidine, and buprenorphine. mydriasis, lacrimation, rhinorrhea,
piloerection, yawning, sneezing, anorexia nausea, vomiting, and diarrhea. |
|
___________________ typically presents in the first 3 decades with episodes of
weakness occurring following rest after exercise, particularly if the subject is exposed to high-carbohydrate meals, emotional stress, or cold. The respiratory and cardiac muscles are almost always spared, and attacks last for hours. Symptoms often resolve in later life, although patients may be left with residual weakness. Most patients have mutations in the skeletal muscle calcium channel gene, although mutations may be found in sodium and potassium channels in the remainder. Secondary hypokalemic paralysis occurs in older patients with chronic medical problems predisposing them to hypokalemia. Preventive treatment with acetazolamide is usually effective in abolishing or attenuating episodes, and oral potassium supplements may be helpful during acute attacks. Some patients require the use of potassium-sparing diuretics such as spironolactone or triamterene. |
Autosomal dominant hypokalemic periodic paralysis
|
|
Autosomal dominant hypokalemic periodic paralysis where is the mutation?
|
mutations in the skeletal muscle calcium channel gene, although mutations may be found in
sodium and potassium channels in the remainder. Nephrolithiasis is a complication of long-term therapy with acetazolamide and should be screened for with annual ultrasound examinations. |
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HA throughout the day.....and tx.....
|
Chronic paroxysmal hemicrania is considered a syndrome with two pivotal characteristics: (1) unilateral
headache of moderate intensity, with episodes lasting under 45 minutes on average, occurring multiple times throughout the day, and at a 3:1 ratio female:male; and (2) absolute response to indomethacin. Patients may also complain of a continuous low-grade headache in between the more severe attacks (hemicrania continua). |
|
tx resting tremor in PD.
|
trihexyphenidyl (Anticholinergic drugs) for younger PD patients (<60 years) with severe resting
tremor but cognitive function is preserved. Levodopa is probably more effective for the control of tremor and other parkinsonian features than any other drug. COMT inhibitors andMAO-B inhibitors such as tolcapone and selegiline, and amantadine are indicated for motor fluctuations associated with PD and to provide neuroprotection. No propanolol. |
|
prodrug that is converted in the liver to an alpha agonist. Its predictable absorption and
pharmacokinetics makes it the drug of choice for treatment of orthostatic hypotension unresponsive to fludrocortisone. |
Midodrine esp with MSA
|
|
hypocretin-1 and hypocretin-2. The cause of. Over 90%
of patients with ________________ carry HLA-DQB1*0602. |
narcolepsy with cataplexy is still under investigation
|
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A-delta nociceptive fibers use _______________ as their fast transmitter.
|
glutamate
|
|
C-fiber slower transmitted nociception involves
a number of neurotransmitters and neuromodulators, the most important of which is: |
substance P.
|
|
TX Juvenile myoclonic epilepsy
|
valproate.
is a common type of epilepsy with onset occurring during adolescence. This is a fairly common type of epilepsy, which can develop between 8 and 26 years of age but usually starts between ages 12 and 16. Historically, the large majority of patients become seizure-free when treated with valproate. |
|
_____________ is a rare condition of spontaneous and continuous muscle fiber activity of peripheral nerve
origin. It represents the more severe phenotype of peripheral nerve hyperexcitability and, when acquired, is often associated with antibodies to voltage-gated potassium channels. There are no specific published electromyographic or clinical diagnostic criteria for this disorder. Carbamazapine, topiramate, and gabapentin have all shown efficicacy. |
Neuromyotonia
|
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Neuromyotonia has AB to ______________ channels.
|
voltage-gated potassium
|
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HyperKalemic periodic paralysis. tx
|
Glucose or other
carbohydrates (sugars) given during an attack may reduce the severity. Intravenous calcium or diuretics such as furosemide may need to be given to stop sudden attacks. Intravenous glucose and insulin cause potassium to move into the cell and may reduce weakness without a loss of total body potassium. A high-carbohydrate diet may be recommended. Acetazolamide, a medication that prevents attacks of familial periodic paralysis, is also effective in preventing attacks of hyperkalemic periodic paralysis. Thiazide diuretics such as chlorothiazide are also effective and have fewer side effects than acetazolamide. |
|
tx tics in tourettes
|
Alfa-adrenergic agents such as guanifencine and clonidine are first-choice treatments for TS.
Neuroleptics are the most consistently useful medications for tic suppression; a number are available but some are more effective than others (for example, haloperidol and pimozide). |
|
Cabergoline, pergolide, bromocriptine, and dihydroergocriptine are all ergot-derived dopamine agonists that have been associated with what side effect.
|
serosal fibrosis syndromes. Ropinirole is a nonergoline and does not have this adverse effect.
|
|
Vigabatrin enhances GABAergic transmission by inhibiting _________________ thus increasing GABA
concentration at the synapse. |
GABA transaminase,
|
|
In compression neuropathies, sensory fibers are usually affected first with conduction changes. The median
palmar sensory latency prolongation is the earliest change seen on EMG/nerve conduction studies in patients |
with mild carpal tunnel syndrome.
|
|
In a _______________ rapid repetitive stimulation causes no change in the
compound muscle action potential. |
postsynaptic disorder (ie, myasthenia gravis), There would typically be a >10% decrement seen on slow repetitive
stimulation. |
|
A significant increment on rapid repetitive stimulation is typically seen in
|
presynaptic disorders
(Lambert-Eaton myasthenic syndrome, botulism). |
|
periodic lateralized sharp wave discharges in a patient with a recent febrile illness and onset of
seizures would strongly suggest |
herpes simplex encephalitis.
|
|
presence of generalized periodic 1 Hz sharp
waves with progressive dementia is strongly suggestive |
of Creutzfeldt-Jakob disease.
|
|
Injury to _______________ weakens the toe
and foot dorsiflexors, with sensory changes over the web of the skin between the first and second toe. |
the deep peroneal
|
|
Lesion of
the ____________ affects the everters, with sensory deficit over most of the dorsum of the foot. |
superficial peroneal
|
|
Injury to the _________
weakens the quadriceps muscle. |
femoral nerve
|
|
Injury to the __________ weakens the adductors, internal and
external rotators of the thigh. |
obturator nerve
|
|
Injury to the ___________ weakens the foot inverters, dorsiflexors and intrinsic foot
muscles. |
tibial nerve
|
|
Mutations of the FIL-1 gene in chromosome Xq28, encoding for filamin-1, produce _____________
This reflects a complete failure of neuronal migration that may cause seizures. It is dominant in females and lethal in males. Filamin-1 interacts with actin and is critically involved in the early phases of migration. |
periventricular heterotopy.
|
|
Familial periodic paralyses
|
most common channel episodic-weakness phenotypes is a long-lasting depolarization that inactivates sodium ion (Na+)
channels, initiating the action potential. |
|
Drugs that inh metabolism of carbamez:
|
These include phenytoin,
cimetidine, diltiazem, erythromycin, verapamil, fluoxetine, and isoniazid. |
|
carbamazapine can accelerate hepatic breakdown of a number of drugs, including its own metabolism.
|
The most common interaction is with oral contraceptives, sodium valproate, ethosuximide, corticosteroids, anticoagulants,
antipsychotics, cyclosporine, and methylphenidate. |
|
Meperidine and methylphenyldate can _________ the seizure
threshold in epilepsy patients resulting in worsening seizures independent of antiepileptic therapy. |
lower
|
|
worst loss of acetylchol in what type dementia
|
DLB
|
|
AED that has also been associated with changes in serum lithium
levels and lithium toxicity and therefore would not be indicated for primary treatment of epilepsy in bipolar patients well controlled on lithium. |
Topiramate
|
|
____________ interacts with actin and may contribute to
structural and functional stability of the plasma membrane. |
dystrophin
|
|
AED......can produce hair loss that rarely requires discontinuation of the drug.
|
Valproate
|
|
In compression neuropathies, _______________ fibers are usually affected first with conduction changes.
|
sensory
|