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161 Cards in this Set

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Brain tumor associated with NF2:
* Meningioma
Brain tumor associated with NF1, looks like hair:
* Pilocytic astrocytoma (occurs in the cerebellum)
Low grade glial tumor that has a "fried egg" appearance histologically:
* Oligodendroglioma
Cauliflower looking tumor that occurs in the 4th ventricle and spinal cord:
* Ependymoma (causes hydrocephalus)
Common category 1 CNS tumor:
* Medulloblastoma (embryonal origin)
Category 2 CNS tumors arise from what components:
* Glial and choroid plexus (astrocytomas, gangliomas)
Category 3 CNS tumors have features of:
* Leptomeninges and mesenchymal tissue (meningiomas)
A secondary brain tumor due to metastasis from another site would be what category?
* Category 5 (secondary tumors)
Category 4 CNS neoplasia are what type of tumors:
* Germ cell tumors
Blue cell tumor seen in kids that is seen often in the cerebellum:
* Medulloblastoma
Highly malignant tumor seen in kids that infiltrates the leptomeninges and has features of epithelium:
* Medulloepithelioma
Complication seen with medulloblastomas:
* Hydrocephalus
Retinoblastoma is due to deletion of:
* The RB1 gene
Most common type of glial (astrocytoma) tumor:
* Glioblastoma
A pt with a constant gait ataxia probably has a lesion where?
* Cerebellum
Differential for someone with "vertigo":
* BPPV, vestibular neuritis, CN 8 lesion, drug toxicity (aminoglycosides, chemo), meniere syndrome
Most common cause of vertigo:
* BPPV (benign paroxysmal positional vertigo)

Sx: No N&V, symptoms occur with head movement, vertigo less than 1 minute
What causes BPPV:
* Endolymph debris-- loose otoconia (treat using the dix-hallpike maneuver)
Management of BPPV:
* Epley maneuver and Brandt exercises
What causes meniere's diseae?
* Idiopathic endolymphatic hydrops (Tx with Acetazolamide, HCTZ, or surgery)
A pt with episodic vertigo, tinnutis, and hearing loss along with N&V probably has:
* Meniere's disease
A very miserable pt with vertigo, prominent N&V and might have tinnitus or hearing loss:
* Vestibular neuritis or Labrynthitis (if they have tinnitus/hearing loss)

TX: Benzo's, steroids, antihistamines
Common cause of recurrent vertigo with PHOTOPHOBIA, headache, and N&V:
* Central vertigo = basilar migraine
When you lose the 4 features of consciousness (arousal, wakefulness, responsiveness, and awareness to self/environment) you are in:
* A Coma
What brain system controls arousal and when damaged you are in a true coma?
* Ascending reticular activating system
A pt with episodic vertigo, tinnutis, and hearing loss along with N&V probably has:
* Meniere's disease
A very miserable pt with vertigo, prominent N&V and might have tinnitus or hearing loss:
* Vestibular neuritis or Labrynthitis (if they have tinnitus/hearing loss)

TX: Benzo's, steroids, antihistamines
Common cause of recurrent vertigo with PHOTOPHOBIA, headache, and N&V:
* Central vertigo = basilar migraine
When you lose the 4 features of consciousness (arousal, wakefulness, responsiveness, and awareness to self/environment) you are in:
* A Coma
What brain system controls arousal and when damaged you are in a true coma?
* Ascending reticular activating system (ascends through the periaqueductal gray)
If you have a lesion in the pons, what body part will be affected?
* The eyes
A glasgow coma scale of 8 or less means:
* Coma
What is decorticate positioning?
* Arms flexed, legs extended (this would be supratentorial damage)
A pt with both arms and legs extended has a lesion where?
* Decerebrate positioning-- lesions is at the midbrain or lower (infratentorial)
How do you tell if a lesion is UMN or LMN?
* UMN has reflexes, LMN doesn't
Pt with normal (reactive) pupils, cheyne-stokes resp, decorticate, eyes look toward lesion and away from paresis has a lesion where?
* Cortex
Pt with small (but reactive) pupils, cheyne-stokes, decorticate, and eyes look toward lesion and away from paresis has a lesion where?
* Thalamus
Pt with dilated/unreactive pupils, dysconjugate gaze, abduction paralysis, decerebrate, apneustic resp?
* Midbrain
A pt with pinpoint pupils, intact light reflex, looks away from lesion or toward paresis, paralysis of lateral eye movement, decerebrate, and apneustic resp; where is the lesion?
* Pons
What is the big give-away with a midbrain lesion localization?
* Dysconjugate gaze (one eye straight while one eye looks at the wall)
A person in a coma but has a normal neuro exam probably is in:
* Metabolic state induced coma
A comatose patient with bilateral dilated pupils that get bigger and bigger, bilateral medial rectus paralysis, then decorticate to decerebate and then death, has what type of herniation?
* Central herniation
Classic sign of transtentorial (uncal) herniation:
* Ipsilateral CN III (oculomoter) paralysis

The ipsilateral pupil is dilated and unreactive
A pt with tonsillar herniation will have what signs:
* Paralysis of lateral eye movement, the pressure on the medulla will eventually cause death (they will be decerebrate and then become flaccid)
What is a duret hemorrhage?
* Complete uncal herniation-- too late at this point
Causes the brain damage seen in car accidents:
* Torque on the brainstem
2 classic signs of skull fracture:
* Coons eyes and battle signs
Being unable to recall the period of time after a brain injury is called:
* Anterograde amnesia
Where is CSF produced and absorbed?
* Produced by choroid and absorbed by arachnoid villi
Triad of symptoms seen with hydrocephalus (arachnoid villi aren't plugged/scarred):
* Wet, wacky, wobbly
If someone reaches out to touch a doorknob and they begin to have a tremor this is called ___ and the dysfunction is where?
* Called an intention tremor and is IPSILATERAL cerebellar dysfunction
Where is the dysfunction in a resting tremor?
* CONTRALATERAL basal ganglia
What is dementia and is it a clinical dx?
* Progressive decline in intellectual function and it is NOT a clinical dx
A pt with dementia and gait disturbance has problems where in the brain?
* Frontal lobe
Classic brain finding seen with Alzheimers:
* Neurofibrillary tangles (plaques aren't as important)
Where is atrophy most prominent in Alzheimers?
* Medial temporal limbic areas
How do you tell if a pt's dementia is d/t to Pick's disease?
* Only one lobe atrophies (focal)
A pt with coarsening of primary-emotional behaviors, social withdrawl/negativity, aphasias, anomias, and enhanced artistic realization and muscial ability has a problem where in the brain?
* Left frontotemporal
Main treatment for Alzheimers:
* Acetylcholinesterase inhibitors (you want to increase ACh in the brain)
A pt who has loss of language and social skills, coldness, and enhanced artistic realization has problems where in the brain?
* Bitemporal
Characteristic tremor seen in Parkinsons:
* Resting tremor
A tremor that begins anytime someone starts to move is:
* Essential tremor (give them some EtOH and the tremor will slow down)
What causes Huntington's Chorea?
* Increased CAG repeats on chromosome 4 (AD disease) need at least 36 repeats for the disease
Where does degeneration occur in the brain of someone with Huntingtons?
* The caudate head
A person on metaclopromide/haldol who always looks like their chewing gum when they aren't (abnormal mouth movements) has:
* Tardive dyskinesia
Toxic-metabolic disorder than can cause myoclonus:
Hepatic encephalopathy can cause this "flapping tremor":
* Asterixis
A pt with hemiballism on the right has damage where in the brain?
* Left (contralateral) subthalamic nucleus
Dopa cells are lost where in pt's with Parkinsons?
* Substantia nigra pars compacta
Most effective management of neuropathic pain:
* Antidepressants and antiepileptics
Most common trigger for migraine:
* Adrenaline (due to stress let downs)
Only NSAID that can prevent migraine:
* Naproxen (Triptans are the best abortive agents)
Best way to differentiate a migraine from a cluster headache (aka trigeminal autonomic cephalgia):
* Migraines make you want to sleep
Cluster = CAN'T sleep
2 neurotransmitters involved in seizure:
* Too much glutamate (excitatory) or not enough GABA (inhibitory)
What is seen on an EEG with seizure?
* Spikes and waves
Describe a typical grand mal seizure (generalized tonic-clonic seizure):
Start out with a cry, get stiff all over (tonic), then become clonic (jerking), can be incontinent, unconscious, last about a minute, after jerking they are post-ictal, maybe sleepy for up to an hour
5 or more minutes of continued seizure activity is termed:
* Status epilepticus
CSF with WBC's, low glucose, and elevated protein has what type of meningitis?
* Bacterial (WBC to 5k) or Fungal (WBC to 10k)
How would you differentiate bacterial from viral meningitis?
* With Viral the glucose is NORMAL (50 - 80mg/dl or 65% or serum glucose)

Bacterial/Fungal the glucose is low
Organism that causes most general bacterial meningitis:
* Strep pneumonia
Most common cause of sporadic viral encphalitis:
* Herpes simplex (goes to medial temporal lobes)

Tx: Acyclovir
Familial prion disease:
* Gestermann-Strausller (on chrom 20)
Most common cause of meningitis in neonates:
* H. influenzae
Why does hydrocephalus occur as a complication of meningitis?
* Scarring of the arachnoid villi
Who usually gets a subdural empyema?
* Adults after having some surgery involving the head
This complication of neurosyphilis occurs decades after infection and atrophies the dorsal root ganglia:
* Tabes dorsalis
The types of fungi that cause brain abscesses are ____ while the ones that cause meningitis are ____.
* Abscess = filaments (mucor, aspergillus)

Meningitis = yeasts (cryptococcus, histoplasmosis)
How does mucormycosis typically infect the brain?
* It ascends through the cribiform plate
The structure of a prion:
* Beta pleated sheets
Where does herpes enchephalitis infect the brain?
* Temporal lobes
Agent causing subacute sclerosing panencephalitis:
* Measles
Agent causing progressive multifocal leukoencephalopathy (PML):
* the JC virus -- demyelinates the oligodendroglia
Is muscle fasciculations UMN or LMN?
Where is the damage range in the spinal cord if there is ventilator dependence?
* C1 to C3
Where is the damage if thereare no upper limb movement, but they can breathe on their own?
* C4
What causes subacute combined degeneration?
* B12 deficiency
What disease is characterized by gliosis of the corticospinal tracts and muscle atrophy:
* AML -- motor neuron only!

Oculomotor muscles are the only muscles spared
Only drug for ALS:
* Riluzole
What are the signs and symptoms of transverse myelitis?
* Rapid onset, monophasic, paraparesis, bowel/bladder dysfunction, brisk DTR’s, bilateral BABINKSI, sensory level
Relapsing and remitting (recurrent) demyelinating disease:
* Multiple sclerosis
What syndrome has upper limbs weaker than lower limbs, with UMN signs in the upper limbs and LMN signs in the lower limbs?
* Syringomyelic syndrome
3 genetic trisomies that can be carried to term:
* Trisomy 13, 18, and 21 (21-- Downs is the only one that lives longer than 1 year though)
What is a neural tube malformation that is d/t failure of closure of the posterior neuropore?
* Chiari malformations
What is a neural tube malformation that is d/t failure of closure of the anterior neuropore?
* Anencephaly (absent calavarium, forebrain among other things)

Early indicator = Increased AFP
What is common to Chiari I, Chiari II, and Chiari III malformations?
* All have cerebellar ectopia and small posterior fossa
In addition to cerebellar ectopia and small posterior fossa, what else characterizes Chiari II (Arnold-Chiari malformation)?
* Brainstem malformation with BEAKED midbrain and spinal meningomyelocele
This chiari malformation is rare and is characterized by cerebellar ectopia, small posterior fossa, inferior displacement of the brainstem, and occipito-cervical encephalocele:
* Chiari III
What are the characteristics of chiari IV malformation?
* Severe cerebellar hypoplasia and meningomyelocele
Chiari malformation with a beaked midbrain but NO meningomyelocele:
* Chiari 1.5 (if it was beaked and had a meningomyelocele it would be chiari II)
Chiari malformation characterized by cerebellar hypoplasia:
* Chiari IV
What is the deficiency in PKU?
* Deficiency of phenylalanine hydroxylase (converts pheynlalanine to tyrosine)

AR disorder on chromosome 12
Discuss Tay-Sachs:
* AR on chrom 15, Ashkenazi Jews, beta-HEX-A deficiency, accumulation of glycosphingolipid accumulation in neurons

Imaging show macrocephaly without hydrocephaly
Group of disorders of the development of movement and posture causing activity limitation is:
* Cerebral Palsy
This disorder is a cleft of spinal cord result from failure of the neural folds to form the neural tube leading to an “OPEN BOOK” defect:
* Myeloschisis
What happens in a meningomyelocele?
* Herniation of meninges & spinal cord through the vertebra-- neural tube defect
A protrusion of the brain through a congenital opening in the skull (80-90% are occipital) that is also associated with hypoplastic lungs is:
* Encephalocele
This is a terminal fixation of the conus medullaris leading to restricted upward movement of the spinal cord:
* Tethered cord syndrome
What maternal disease is associated with holoprosencephaly (single ventricular cavity)?
* Maternal diabetes or EtOH consumption/abuse
3 most constant changes in this disease are: abnormal/agenesis of cerebellar vermis, cystic dilation of 4th ventricle, and enlargement of the posterior fossa:
* Dandy-Walker syndrome
Widespread loss of gyri associated with a genetic defect:
* Miller-Dieker syndrome (no gyri in the brain)
Most common interventricular tumor in adults occuring in the midline usually (peak occurance in 3rd decade):
* Neurocytoma
Meningiomas (most are dural based) are associated with what disorder:
* NF2
Hemangioblastomas are associated with what disease?
* VHL (von-hippel lindau disease)
Tumor found in the paramedian location of the cerebellum and associated with VHL:
* Hemangioblastoma
Tumor arising from Rathkes pouch:
* Craniopharyngioma
Primary CNS lymphoma is often associated with:
* the EB virus and large diffuse B-cell tumors
Common histo component to neuroblastomas:
* Homer-wright rosettes
Schwannomas most commonly occur where?
* On the 8th crainal nerve
Nerve that is pinched in carpal tunnel syndrome:
* the MEDIAN nerve (become trapped underneath the flexor retinaculum)
This muscle has weakness/wasting in carpal tunnel:
* Abductor Pollicis Brevis (APB)
A positive Tinnel or Phalens sign is indicative of:
* Carpel tunnel
What causes meralgia paresthetica?
* Entrapment of the lateral cutaneous nerve of the thigh
This is an immune acute aquired inflammatory demyelinating polyneuropathy:
* Guillain-Barre Syndrome (GBS)
What are the clinical features of GBS?
* Progressive ascending symmetric flaccid weakness of the limbs, can eventually ascend to cranial nerves and be fatal

Tx: IV immunoglobins
How does ALS present?
* Progressive weakness over months/years that generally starts in one extremity
Hallmark of the post-synaptic NMJ disorder myasthenia gravis:
* Fatiguable weakness -- they will have arm drift and can't sustain an upward gaze
Drug to help dx myasthenia gravis:
* Endrophonium
What is the general pattern of weakness in myopathy?
* Proximal weakness is greater than distal (getting out of a seat, climbing stairs, lifting arms above head)

Will have elevated CPK, nerve conduction is normal though
Duchenne and Becker muscular dystrophy are both disorders of:
* Dystrophin both are x-linked as well (kids with Becker live longer though)
What area of the brain does Alzheimers affect the most?
* Temporal lobe-- especially the hippocampus
Prototypical frontotemporal dementia:
* Pick's disease (have Aphasia, Apraxia, and Agnosia)
What causes amyotrophic lateral sclerosis (ALS)?
* Loss of motor neurons in the anterior horn of the spinal cord (LMN) as well as in the motor cortex (UMN)
What visual field disturbance is most common with a temporal lobe tumor?
* Superior quadrantanopsia
Where is the tumor: cyclical vomiting, occipital headache, vertigo, nystagmus, ataxia, ipsilateral hypotonia and hyporeflexia, brainstem signs and hydrocephalus occur late:
* Cerebellum
Increased ICP affects this cranial nerve:
* Abducens (CN VI)
Cerebral edema that responds to osmotic agents, and is a failure of the ATP-dependent Na/K pump:
* Cytotoxic cerebral edema (seen with strokes)
Cerebral edema that is d/t to seeping of CSF into the parenchyma d/t CSF flow obstruction:
* Interstitial cerebral edema
Which type of cerebral edema is most common and where does it occur?
* Vasogenic, occurs mainly in white matter, around brain tumors, abscesses or lesions, and is caused by BBB disruption with increased permeability-- responds to steroids
A patient with early nunchal rigidity and head tilt followed by coma and respiratory arrest might have a this herniation:
* Tonsillar herniation
A patient with a dilated ipsilater pupil, followed by ipsilateral hemiparesis might have this herniation:
* Uncal herniation
Where do menengiomas arise from?
* Arachnoid cells, develop at a dural site
A subdural hematoma is damage to:
* The bridging veins (pt's will show a crescent shaped blood on CT/MRI and it can be acute or a delayed presentation)
A epidural hematoma is damage to:
* Middle meningeal artery-- presentation is acute (hours or less)
How do the best treatments for myasthenia gravis work?
* The carbamylate the acetylcholinesterase enzyme (Neostigmine and Pyridostigmine)
Pt's who OD on tricyclic antidepressants often die from:
* Lethal arrythmias
What are Beta waves and Delta waves associated with in relation to sleep?
* Beta = REM or alert wakefulness
Delta = Stage 4 (deep, non-rem sleep)
Tabes dorsalis affects what in the spinal cord?
* The dorsal COLUMN
Most common complication of meningitis:
* Hydrocephalus
Where does the greatest amount of salt and water retention occur in cerebral edema?
* The white matter
Typical location for hypertensive hemorrhages:
* Basal ganglia
Rupture of a Berry aneurysm can lead to:
* Subarachnoid hemorrhage