Random Neuro facts Flashcards
Treatment for absence seizures
Ethosuximide and if that fails valproic acid
What type of seizures is phenytoin indicated for?
Partial and generalized tonic clonic seizures
Gerstmann’s syndrome
- usually involves the left parietal lobe damage
- tetrad of acalculia, agraphia wihtout alexia, right left confusion, finger agnosia
- left angular gyrus
What type of lesions is alexia without agraphia seen?
Splenium of the corpus callosum
What is the treatment for trigeminal neuralgia?
carbamazepine and oxcarbazepine
- other treatments: gabapentin, TCAs, tiagabine, opioids, NSAIDS, lidocaine patches, benzos, stereotatic gamma knife
Giant cell arteritis
Medium sized vessels, women affected more than men
- new onset or change in headache with fever, fatigue, myalgia, night sweat, weight loss, jaw claudication. 25% of pts have polymyalgia rheumatica. The temporal artery can demonstrate tenderness with induration and diminished or absent pulse. Can have irreversible and sudden vision loss as a result of central retinal artery occlusion.
Test of choice is ESR, then gold standard temporal artery biopsy.
Treatment is prednisone.
cluster headaches
attaches generally nocturnal
alcohol consumption is a common trigger
Abortive therapies: oxygen, sumatriptan subcutaneous injection, ergotamine
Prophylactic therapies: prednisone, verapamil, depakote, methysergide and lithium
What are causes of acquired peripheral neuropathy?
Vincristine and isoniazid, excess B6 therapy, inhalant abuse (toluene, NFO), heavy metal poisoning, hydrocarbon exposure, vitamin B12 deficiency, niacin deficiency, complications of mono, lupus
dermatomyositis
autoimmune disease that affects skin and muscle, purplish rash on face and eyelids(can also appear on neck, elbows, and knees which are often red and indurated), and muscle weakness
- CPK levels often elevated
- EMG shows myopathy and muscle irritability
- Biopsy shows perifascicular atrophy and “ghost fibers”
- Strong relationship between dermatomyositis and occult neoplasm in up to 50% usually carcinoma
What is the most significant genetic risk factor for Familial Alzheimer’s dementia?
homozygosity for the inheritance of E4 allele of apolipoprotein E (apo E). Apo E4 has been associated with chromosome 19.
What is Saturday night palsy?
Radial nerve entrapment- prolonged armpit compression when the arm is draped over the edge of a chair or on crutches
- weakness in the extensor muscles of the wrist, fingers, tricept weakness, and supinator weakness.
usually resolves after 1-2 months
Ulnar nerve entrapment
- occurs at elbow or wrist (elbow trauma w/ entrapment in cubital tunnel or with compression during surgery)
- weakness in the flexor carpi ulnaris, intrinsic hand muscles, and fourth and fifth deep flexor weakness
Musculocutaneous nerve injury
Injury to brachial plexus such as shoulder dislocation, shoulder compression
- weakness of biceps and brachialis
Median nerve entrapment
Carpal tunnel
tenosynovitis of the transverse carpal ligament places pressure on the median nerve in the tunnel
- causes nocturnal hand paresthesias of thumb and index and middle fingers
- may be sensory loss, thenar atrophy and positive tinels test
- diagnostic test is needle EMG and nerve conduction studies which show delayed sensory latency across the wrist
Suprascapular nerve entrapment
can occur after repetitive forward traction of the shoulder. diffuse aching of posterior shoulder. EMG shows denervation of the infraspinatus and supraspinatus muscles
The most common cause of intracerebral hemorrhage is hypertension, how does this happen?
Chronic hypertension probably causes lipohyalinosis of the small intraparenchymal arteries and charcot bouchard microaneurysms that rupture owing to increased vascular pressure
Where is the most common area of predilection for ICH
putamen followed by the thalamus
Screening test for sarcoidosis
serum ace level
Cryptococcal meningitis
yeast, AIDS w/ CD4 count generally less than 200. opening pressure usually elevated, csf colorless and clear, leukocytosis of 50 to 1000 cells with lymphocytic predominance, elevated protein.
Indian ink - capsule and budding yeast (can also do cryptococcal antigen assay-more sensitive)
Treatment: amphotericin B (but this does have high rate of renal toxicity)
What protein makes up amyloid plaques found in alzheimers disease?
APP, found on chromosome 21
Creutzfeldt-Jakob disease
Prodromal period of vegetative symptoms such as weakness, sleep and appetite disturbances
Then, rapidly progressive dementia with deficits in memory and concentration, depression, self neglect and personality changes.
Then, global dementia and death typically 2-7 months after onset of symptoms
Diagnostic tests: lumbar puncture CSF assay of 14-3-3 and tau proteins
Internuclear ophthalmoplegia
Seen in MS
lesion localizes to the medial longitudinal fasiculus of the brain stem
- abnormal horizontal ocular movements with absence or delayed adduction of the eye ipsilateral to MLF lesion and course horizontal nystagmus in the abducting eye. Convergence is preserved
Lesion is ipsilateral to the eye that does not adduct
anosognosia
inability to recognize a neurological deficit that is occurring to oneself
MS
the most common inflammatory demyelinating disease
Onset: 15-50 years old
2/3 have relapsing-remitting at onset
- only 20% of patients have primary progressive disease at onset
- heat sensitivity-increases in core body temp can bring on or worsen already existing symptoms (uhthoff’s phenomenon)- conduction block as body temperature rises
optic neuritis
optic neuritis is a common sign of MS- eye pain that increases with movement, central visual loss (scotoma), afferent pupillary defect
Internuclear ophthalmoplegia
common sign of MS
lesion in MLF of brainstem
eye ipsilateral to the lesion cannot adduct past the midline, contralateral eye fully abducts and displays a coarse end gaze nystagmus
What is Lhermitte’s sign
Transient neurological sign-sensation of an electric shock that descends down the spine or the extremities on neck flexion
- suggestive of MS but can be seen in other conditions
Do MS lesions enhance with gadolinium early during an attack and can persist to 8 weeks?
yes
Treatment for MS attack
IV corticosteriods
Aphasias (fluency, comprehension, repetition)
Brocas, Wernickes, conduction, transcortical motor, transcortical sensory, transcortical mixed, global
Brocas (left posterior inferior frontal gyrus): No fluency, Yes comprehension, No repetition
Wernickes(superior temporal gyrus): Yes fluency, No comprehension, No repetition
Conduction(arcuate fasiculus): Yes fluency, Yes comprehension, No repetition
-right homonysmous hemianopsia- with absence of motor and sensory signs
Transcortical motor: No fluency, yes comprehension, yes repetition
Transcortical sensory: Yes fluency, No comprehension, Yes repetition
Transcortical mixed: No fluency, No comprehension, yes repetition (watershet)
Global: No fluency, No comprehension, No repetition
What happens with rapid sodium replacement in hyponatremia?
Central pontine myelinolysis which can result in clinical transection of the pons and locked in syndrome.
- can move eyes up and down and blink
- Most common cause of locked in syndrome is ventral pontine infarct as a consequence of basilar artery thrombosis (other causes: are acute inflammatory demylinating poly neuropathy, myasthenia gravis, and neuromuscular blocking agents)
pseudotumor cerebri (intracranial hypertension)
Classically seen in young obese AA women
Risk factors: Hypervitaminosis A, high does corticosteriods, tetracycline, oral contraceptives, and head trauma
Symptoms: Waxing and waning headache w/ visual obscurations
Exam: papilledema and enlargement of blind spot
Test:: lumbar puncture (opening pressure over 20 cm H20)
Treatment: acetazolamide or prednisone, lumbar puncture, ventriculoperitoneal shunting, lunboperitoneal shunting optic nerve sheath fenestration
Migraine without aura criteria
- at least 5 headache attacks lasting 4-72 hours
which have at least 2 of the four characteristics: - unilateral
- pulsating
- moderate to severe intensity
- aggravated by walking stairs or similar routine physical activity
at least of of the two - photophobia/phonophobia
- nausea/vomiting
inclusion/exclusion criteria for TPA
Inclusion:
- acute ischemic stroke with a clear time of onset less than or equal to 3 hours prior
- neurological deficit measurable on NIH stroke scale
- CT head demonstrating no intracranial hemorrhage
Exclusion:
- rapidly improving symptoms
- seizure at onset of stroke
- prior intracranial hemorrhage
- pretreatment blood pressure greater than 185/110
- major surgery within previous 14 ays
- prior stroke or head injury within the past 3 months
- intestingal or urinary bleeding within 3 weeks prior to stroke
- subarachnoid hemorrhage
- really low or really high sugars
- recent MI
- current use of anticoagulants (PT>15 INR>1.7)
- heparin within past 48 hours
- platelet count of <100,000
- arterial puncture at noncompressible site within past 7 days
Left lateral medullary syndrome (wallenberg’s syndrome)
Caused by: occlusion of one of the vertebral arteries or less commonly posterior inferior cerebellar arteries
- ipsilateral horners
- ipsilateral loss of pain and temperature sensation in the face
- cerebellar ataxia
- weakness of the vocal cord/pharynx, and palate
- contralateral loss of pain and temperature sensation to the hemibody
- no visual system changes
What is the most common offending infectious agent in AIDS-related retinopathy?
Cytomegalovirus
What does JV virus cause?
progressive multifocal leukoencephalopathy in AIDS patients
Is intramuscular phenytoin a good choice for status epilepticus? What are useful treatments
No because its erratic rate of absorption
- useful: rectal or IV diazepam, IV lorazepam, IV phenytoin or fosphenytoin, IV valproic acid, oxygen by nasal cannula and airway protection, IV phenobarbitol (but not great narrow therapeutic window)
Which anticonvulsant has the broadest spectrum of coverage?
Depakote
- blockade voltage gated sodium channels and GABA enhancement
Foot flexion involves which nerve root and which muscles?
- tibialis anterior muscle
- L5 (also controls extension of extensor hallucis longus)
- Lesion at L5 often causes foot drop
S1 nerve root
- foot extension (pushing down on the gas petal)
Leg extension involves which muscles and which nerve roots?
quads
L3/L4 nerve roots
Hip flexion uses which muscles and which nerve roots?
iliopsoas muscle
L1, L2, L3
Achilles motor reflex is a function of which motor nerve root?
S1
Brown Sequard syndrome
hemisection of the spinal cord
- ipsilateral and below lesion: loss of motor control and posterior column function (proprioception and vibration)
- contralateral one or two dermatomal levels below the level of the lesion: loss of pain and temperature sensation
Pure motor hemiparesis is expected from an infarct in what area?
internal capsule, the basis pontis, or the corona radiata
What is a lacunar stroke?
ischemic strokes resulting from small vellel lipohyalinosis that is caused by htn and diabetes
microembolism may also be a mechanism for lacunar stroke
Pure sensory lacunar syndrome result from a lesion where?
ventroposterolateral nucleus of the THALAMUS
Lesion to what causes left-arm hemiballismus?
right subthalamic nucleus
Reserpine interferes with the magnesium and ATP-dependent uptake of amines which results in the depletion of dopamine, NE, and serotonin and can relieve symptoms of what but also cause what?
- can relieve symptoms of dystonia but can also cause depression, sedation, parkinson’s like symptoms
arachnoid granulations are the major site for what?
drainage of CSF into the blood
what is responsible for the majority of CSF production
chorioid plexus
What drugs can temporarily reduce production of CSF and ease increased intracranial pressure?
Mannitor and acetazolamide
States of hypothermia, hypocarbia, hypoxia and hyperosmolality
What is virchow-robin spaces
act as a duct for substances in the subarachnoid space to enter the brain
What is the most common place of intracranial hemorrhage?
putamen then lobar
How do you reverse anticoagnulation?
protamine sulfate for heparine
parental vitamin K or fresh frozen plasma for warfarin
What releases melatonin?
pineal gland (secreted at night)
- feedback look between pineal gland and suprachiasmatic nucleus in the hypothalamus
What is botulinum toxin used for?
cervical dystonia, blepharospasm, hemifacial spasm, stabismus, axillary hyperhydrosis, cosmetically for hyperactive glabellar lines of the procerus muscles of the forehead
not fda approved but used for MS spasticity , mingraines
- blockade of presynaptic acetylcholine release
Bailar migraine
Occiptial headache and neurologic symptoms attributable to the brainstem. Aura can involve visual and sensory phenomena, accompanied by vertigo, dysarthria, tinnitus, speech deficits. May have LOC. Resemble stroke-like symptoms in the vetebrosbasilar territory
Paroxysmal hemicrania
indomethacin-responsive headache syndromes
- women more than men
- unitlateral brief in the periorbital or temporal areas
5 or more daily lasting 20 minutes
- accompanied by autonomic features such as conjunctival injection, lacrimation, ptosis, rhinorrhea
- treatment indomethacin
What is the generator of migraines thought to be?
Trigeminal nucleus cuadalis (TNC) in the pons, ergot and triptan medications effectively block neuronal firing int he TNC and can abort an attack
How does carbidopa work?
it inhibits DOPA decarboxylase and prevents the peripheral metabolism of levodopa to dopamine before it can cross the blood brain barrier
How do the dopamine agnoists ropinerole, pramipexole, pergolide and bromocriptine work?
direct postsynaptic dopamine receptor agonist
What do COMT (catecholo-o-methyltransferase) inhibitors do?
tolcapone and entacapone prevent peripheral and central degradation of levodopa and dopamine thereby increasing central levodopa and dopamine levels
What anticholinergic agents such as trihexyphenidyl and benztropine do in parkinson’s disease?
reduce tremor by blockade of postsynaptic muscarinic receptor
apoptosis
programmed neuronal cell death
Pain syndrome or reflex sympathetic dystrophy
regional pain and sensory changes following trauma or noxious event
Stage 1(acute): pain is out of proportion to the initial injury
Stage 2 (dystrophic): tissue edema and skin that is cool cyanotic, hyperhidrotic, with livedo reticularis
Stage 3 (atrophic): paroxysmal pain and irreversible tissue damage
Treatment: Physical therapy, steriods, phenoxybenzamine , regional block
Sheehan’s syndrome
postpartum pituitary infarction or apoplexy
- treatment generally supportive but may need corticosteriod replacement or surgical decompression
cushing’s disease
Endogenous overproduction of andrenocorticotropic hormone from the anterior pituitary gland
- result of exposure to excessive endogenous or exogenous corticosteriods
Hypertension, truncal obesity, impaired glucose tolerance/DM, menstrual irregularities, hirsutism, acne, purple striae, osteoporosis, thin skin, excessive bruising, proximal myopathy
Test: dexamethasone supression test
What is the treatment for NPH?
ventriculoperitoneal shunting
Diffuse Lewy-body dementia
second most prevalent dementia is after alzheimers
- triad of dementia, parkinsonism, and VH
- extreme sensitivity to dopamine blockade- severe parkinsonism
What does Wernicke’s show on MRI and autopsy?
MRI: abnormalities in the periqueductal regions, bilateral mamillary bodies, and medial thalami
Autopsy: Multiple small hemorrhages in teh periventricular gray matter mainly around the aqueduct and third and fourth ventricles
Sydenham’s chorea
result of rheumatic fever
-more frequent in girls 5-15
- chorea, irritability, obsessive compulsive traits, restlessness
MRI shows enlargement of the basil ganglia
- self limited symptoms 6 months
- anti-basal ganglia antibodies can be detected by western blot testing
- valproic acid is useful for the chorea
What is cerebellum perfused by?
vertebrobasilar arterial system
What are symptoms that suggest carotid territory ischemic attack?
- ipsilateral monocular blindness (amaurosis fugax)
- contralateral body weakness or sensory loss
- aphasia with dominant hemisphere
- contralateral homonymous visual field deficits
What are symptoms of vertebrobasilar territory stroke?
bilateral, shifting, or crosssed weakness or sensory loss (ispilateral face with contralateral body)
- bilateral or contralateral homonymous visual field defects or binocular vision loss and two or more of vertigo, diplopia, dysphagia, dysarthria, ataxia
Essential tremor
postural and kinetic tremor of the forearms and hands that gradually increases in amplitude over time
- 2/3 pt w/ fam hx
Treatment: beta blocker (main*), primidone, benzos, botulinum toxin, stereotactic thalamotomy, thalamic brain stimulation
Desipramine and TCA-not helpful! can worsen
Migraine is most likely a hereditary disorder that maps to which chromosome?
19
Familial hemiplegic migraine associated with 19
Miller fisher syndrom
varient of guillain-barre
- ataxia, areflexia, opthalmoplegia
GBS CSF
cytoalbuminergic dissociation: elevated protein with an acellular fluid
Is depression less common in cortical dementia (such as Alzheimers) than subcortical disease (prkinsons)
Yes
Which surgical intervensions can be used for the treatment of idiopathic parkinsons disease?
- thalamotomy
- subthalamic nucleus deep brain stimulation
- pallidotomy
- pallidal deep brain stimulaion
What does right-left disorientation result of?
lesion to the dominant angular gyrus
- one of the symptoms of grestmanns syndrome
Diabetic third nerve palsy
spares pupillary function
third nerve palsy from aneurysm
dilated pupil, eyelid ptosis
transient global amnesia
reversible anterograde and retrograde memory loss
- inability to learn newly acquired information and total amnesia of events during the attacks
- retain personal information and identity
- the person may ask the same question repeatedly
-men more than women usually 50s or older
- onset often occurs after physical exertion or exposure to extreme temps
-MOA unknown
B. burgdorferi, lyme disease
early infection:
- meningitis, a unilateral or bilateral bell’s palsy
- painful radiculoneuritis
- optic nueritis
- mononeuritis multiplex
- guillain barre
then:
- lyme rash
Diagnosis: ELISA confirmed by western blot or CSF lyme antibody pcr
Treatment: ceftriaxone or penicillin IV 2-4 weeks
CSF aseptic or viral meningitis
mild to moderate lymphocytic pleocytosis, normal glucose, normal to mildly elevated protein
gram stain and cultures negative
CSF bacterial meningitis
- marked lymphocytosis, polymophonuclear neutriphils
- markedly increased protein
- decreased glucose
Fungal CSF
- moderate lymphocytic pleocytosis
- mildly decreased glucose
- mildly increased protein
Herpetic meningitis CSF
similar to aseptic meningitis but also maybe red blood cells
Guillain barre CSF
- high protein and absence of pleocytosis
Lower motor neuron signs
hypotonia, muscle atrophy, fasiculations, hyporeflexia, flaccidity, muscle cramps, and marked motor weakness
distal to anterior horn cells
upper motor neuron
hyperreflexia, spactificy, bab, clonus, pseudobulbar palsy, loss of dexterity, mild motor weakness
brandt-daroff exercises
exercises in benign paroxysmal positional vertigo
nystagmus and bppv
fast phase of nystagmus is in direction of the lower ear (the ear with the vestibular problem) when the patient looks toward the affected side
persistent vegetative state
period of 1 month must elapse
-perserved sleep wake cycles
-spontaneous movements may be noted and eye opening, eye tracking and swallowing can be preserved.
- no response to noxious stimulation
West’s syndrome
triad of:
infantile spasms
hypsarrhythmia (posterior diffuse slow and sharp wave spikes)
psychomotor development arrest
Treatment: adrencorticoptropic hormone, presnisone, prednisolone
- poor prognosis
Papez circuit
hippocampus with thalamus, hypothalamus, and cortex
What causes benign familial neonatal convulsion?
AD
defect in voltage gated potassium channels
- generalized tonic clonic seizures disappear spontaneously in a few weeks to months
what do malignant hyerthermia, hypokalemic periodic paralysis and familial hemiplegic migraine all have in common?
all genetic disorders involving gene mutations that result in abnormal voltage-gated calcium channels
arnold chiari type 1 malformation
- descent of the cerebellar tonsils below the level of the foramen magnum with or without displacement of the medulla.
- low intracranial pressure
- often accompanied by syringomyelia, syringobulbia, hydrocephalus
Headache, cranial neuropathies, visual distrubances
-shawl distribution of sensory deficit over the shoulders
Chiari type 2 malformation
caudal displacement of medula and 4th ventricle ( hydrocephalus )and addition of lumbar myelomeningocele (paralysis/sensory loss at and below the level of the lesion).
lissencephaly
Lissencepahly
agyria-disorder of early neuroblast migration
- cortex remains smooth
Schizencephaly
defective genetic expression of the EMX2 gene. It results in clefts in the cerebral hemispheres.
Dandy–Walker syndrome
- cystic enlargement or pouching of the fourth ventricle
- posterior portion of the cerebellar vermis is hypoplastic or aplastic
- noncommunicating hydrocephalus
- MR and spastic diplegia
ICA stensosis treatment by endarterectomy when how much stenosed and symptomatic
70-99%
what is the most common bacterial infection that precedes onset of guillain-barre syndrome
campylobacter jejuni
What type of solid tumor metastasizes most frequently to the brain?
lung, non small cell lung carcinoma
Compression of the lateral femoral cutaneous nerve leads to what?
meralgia paresthetica
as the nerve passes beneath the inguinal ligament cause painful sensory syndrome (pain and sensory numbness to the lateral thigh)
risk factors: obesity, pregnancy, tight or heavy belt
can use conservative management or TCA/anticonvulsant
What is the most common congenital viral infection in newborns?
Less than 10% have complication such as jaundice, hepatosplenomegaly, microcephaly, chorioretinitis, ataxia and seizures
Inclusion body myositis
- common myopathy in pts over 50
- men more than women
- affects strength of distal muscles
- finger and wrist flexors
- no muscle pain
- generally chronic and progressive
- biopsy shows endomysial inflammation, macrophage invasion, rimmed vacuoles, and inclusion bodies in the nuclei
EMG: fibrillation potentials and positive sharp waves
facioscapulohumeral dystrophay
autosomal dominant w/ anticipation
- weakness of orofacial muscles, weak shoulder muscles (winging of scapula), arms and legs weak
oculopharyngeal musclar dystrophy
AD
40-60s
- eye muscle weakness and ptosis then difficulty swallowing
Duchenne’s muscular dystrophy and becker muscular dystrophy are x linked recessive disorders of muscle, what does this result in a deficiency of?
dystrophin
How do entacapone and tolcapone work?
catechol-o-methyltransferase (COMT) inhibitor - block periopheral degradation of levodopa and central degradation of l-DOPA and dopamine
Argyll robertson pupils
late syphilis
- small irregular pupils that constrict to accommodation
tabes dorsalis
spinal form of syphilis
- clinical triad of sensory ataxia, lightning pains, and urinary incontinence
- DTR absent
- postive rombergs
- charcots (neuropathic joints)
- argyll robertson pupils/pupil abnormalities
marcus gunn pupil (relative afferent pupillary defect)
swinging flashlight -light applied to affected eye will have lesser constriction with an initial relative dilation
todds paralysis
transient hemiparesis and hemiplegia
tropical spastic paraparesis
chronic progressive myelopathy associated with HTLV-1
- men more than women after 30
- CSF pcr detection of HTLV-1 antiboides
- after 10 years most cannot walk
battle sign
hematoma overlying the mastoid that results from basilar skull fracture extending into the mastoid portion of the temporal bone
What are treatments for spasticity ?
Lioresal (baclofen)
gabapentin
diazepam
clonidine
tizanidine
dantrolene
asterixis
sudden loss of postural tone which manifests as flapping tremor of the hands
Kluver Bucy syndrome
- bilateral destruction of amygdaloid bodies and the inferior temporal cortex
- hypersexuality, placidity, hyperorality
- can be caused by picks syndrome, stroke, alzheimers.
- visual agnosia, hyperphagia, prosopagnosia (inability to recognize faces)
lenticulostriate territory ischemia
- small penetrating branching arteries off the middle cerebral artery that feed the striatum
- can produce lacunar infarcts of the internal capsule that result in pure contralateral motor hemiparesis
anterior cerebral artery ischemia
produce a contralateral hemiparesis of the leg preferentially
middle cerebral artery ischemia
dominant: aphasia
non dominan: hemineglect, anosognia, visual tactile extenction, aprosody of speech, contralateral limb apraxia f
transverse myelitis
segmental inflammatory syndrome of the bilateral spinal cord
- immunologic response in origin and often follows and infection or vaccination or result of demyelination due to MS
-rapid onset of bilateral leg weakness that presents with a clear cut sensory level below the affected lesion - pain and temp affected
-proprioception and vibration spared
imaging: MRI
treatment: IV steriods
vacuolar myelopathy
AIDS pts
-similar to vitamin b12 deficiency
-slower and more progressive
acute disseminating encephalomyelitis
follows systemic infection or vaccination
involves whole CNS
horner’s syndrome
-interruption to the sympathetic fibers supplying the pupil, upper eyelid, facial sweat glands and facial blood vessels
- can be seen as part of medullary stroke syndrome (wallenber’s syndrome), carotid occusion/dissection, high spinal cord lesions, neoplasms like pancoasts tumor that affect the cervical ganglia or intracranial hemorrhage
- if doesnt affect sweat glands then usually internal carotid artery
Prosopagnosia
inability to recognize familiar faces
- bilateral occipital-temporal lesions
- often associated with agraphia and achromatopsia
- almost always associated with visual field deficits
simultanagnosia
inability to perceive a scene with multiple parts to it
astereognosia
inability to recognize and identify items by weight, texture and form alone
- form of tactile agnosia
opsoclonus-myoclonus
paraneoplastic disorder- dancing eyes dancing feet
- most often seen in 6-18 months and in 50% or more of cases associated with infantile neoblastoma
in adults most common cause lung cancer
- can also be noted as a result of postviral encephalitis, MS, thalmic hemorrhage, and hyperosmolar coma
caused by lesions to the pause cells in the pons
treamtent: steriods adrenocroticotropic hormones
ramsay hunt syndrome
herpetic cranial neuritis
facial and acoustic nerves
-painful fcail palsy, vertigo, ipsilateral hearing loss, and vesciles in the external auditory canal and somtimes ont he pinna
- infection presumed to localize to the geniculate ganglion
glioblastoma
most common primary brain tumor in about 50% or more of patients over 60
anaplastic astrocytoma
bimodal peak incidence first and third decade
ependymomas
third most common cns tumor in children
meningiomas
20-25% of brain tumors
more likely to occur after age 50
occure twice as frequently in females
80% are benign
peak incidence of acoustic neuromas
4-5 decade
anton’s syndrome
agnosia characterized by cortical blindness
- lesions to bilateral occipital lobes, particuallry in calcarine cortex and visual association cortex
- deny that they are blind and confabulate , may also suffer visual hallucinations
What is the most common central nervous system (CNS) cancer in AIDS?
primary cns lymphoma
-headache, aphasia, hemiparesis, altered mental status, behavioral changes, ataxia
constitutional symptoms generally absent
-polymerase chain reaction (PCR) testing on CSF revealing Epstein–Barr virus DNA helps to corroborate the diagnosis
aids dementia
poor attention and concentration, bradyphrenia, forgetfulness, poor balance, uncoordination, personality changes, apathy, and depression
What is the most frequent CNS opportunistic infection in AIDS
toxoplasma gondii
cd4<200
-headache, focal neurological deficits, with or without fever
- aphasia, seizures, and hemiparesis
severe: confusion, lethargy, coma
CT or MRI w/ ring enhancing lesions
treatment: primethamine and sufadiazine
HIV vaculolar myelopathy
most common cause of spinal cord pathology in aids pts.
- spasticity, gait instability, lower extremity weakness, loss of proprioception and vibration sensation and sphincter dysfunction
-hyperreflexia, babinski
Distal sensory neuropathy
most common peripheral nerve syndrome that complicates AIDS
- diminished ankle jerk reflexes
PML
JC virus
PML
JV virus- form of human papilloma virus
-demyelination preferentially in the subcortical white matter of parietal and occipital lobe
-hemiparesis, aphasia, sonsory deficits, ataxia and visual field deficits
MRI: multiple or coalesced non enhancing white mater lession in the parietal and occiptal lobe
kuru
-prion disease seen in the cannibalistic fore people looks like progressive cerebellar ataxia
gerstmann-straussler-scheinker syndrome
inherited form of prion disease
- symptoms in 30s-40s
- slowest of the spongiform encephalopathies
- ataxia, parkinson’s plus like symptoms or progressive dementia
fatal familial insomnia
- occurs between ages 35-60
- progressive insomnia and sympathetic autonomic hyperactivity, mild cognitive impairment
- ataxia, tremor, myoclonus, confusion, halluciantions
creutzfeldt jakob disease
sporatic, iatrogenic, or familial
- 14-3-3 diagnostic test
- 50-75
stage 1: neuropsychitric symptoms- fatigue, sleep disturbances memory and concentration deficits and personality changes
stage 2: generalized cognitive deficits, psychosis, hallucinosis
stage 3: profound dementia, myoclonus, choreoathetosis
devic’s disease (neuromyelitis optica)
variant of MS
- cervical myelopathy and bilateral optic neuropathy
the most common causse of bells palsy
herpes simplex infection
what does botulinum toxin release
neurotoxin that blocks acetylcholine
what are classic symptoms of botulism
dysphagia, dysarthria, ptosis, diploplia, limb paralysis, resp paralysis
GI symtpoms
picks disease behavioral type has both behavioral disinhibition and a lack of motivation, how is depression/behavioral problems treated?
SSRI
Atypical antipsychotics
What are hirano bodies seen in?
Creutzfeldt-jakob disease and alzhieimers
hallmark of idiopathic Parkinson’s disease
lewy bodies in surviving neurons in the pars reticulata of the substania nigra in the midbrain
-substantia nigra is depigmented and pail and demontrates gliosis and neuronal loss
where is atrophy in huntingtons seen
head of the caudate nucleus but it does not correlate with the severity of the disease
acute epidural spinal cord compression
-back pain most frequent presenting feature
- motor weakness, sensory deficits, spastic paraparesis or paraplegia with a sensory level that is usually several levels below the actual lesion
- bowel and bladd incontinence, urinary retion or constipation are seen in a majority of pts
screening: sagittal screening MRI of entire spine
treatment: corticosteriods and readition therapy,
subacute combined degeneration
b12 deficiency
- macrocytic anemia, atrophic glossitis, neurological deficits
- lesions to the lateral and posterior columns of the spinal cord
- peripheral neuropathy, optic atrophy, and brain lesions
-postereior columb deficits: upper motor neuron limb weakness, spacticity and babinski sign
- loss of proprioception and vibration sense
- behavioral changes, forgetfulness, dementia
-hyperreflexia with absent ankle jerk
Balint syndrome
rare stroke syndrome- ischemic lesions to the bilateral pareital-occipital lobes or the occipital lobes alone
- can sometimes be result of “top of the basilar syndrome”
inability to perceive the visual field as a whole (simultanagnosia), difficulty in fixating the eyes (oculomotor apraxia), and inability to move the hand to a specific object by using vision (optic ataxia).
antons syndrome
cortical blindness
- lesions to bilateral occipital lobs
- deny that they are blind, may confabulate
- prosopagnosia
meiges syndrome
Dystonia syndrome
blepharospasm and oromandibular dystonia
treatment: botulinum toxin
meiges syndrome
blepharospasm and oromandibular dystonia
treatment: botulinum toxin
scanning speech can be caused by what
lesions of the cerebellum
aphemia
- motor speech disorder characterized by near muteness with normal reading, writing and comprehension
- likely reult from lesions to the primary motor cortex or broca’s area
agnosia
inability to recognize and identify objects
abulia
akinetic mutism
severe apathy
Lasegue’s sign
straight leg raiding produces pain in siactic nerve distribution-siactic nerve compression due to mechanical interruptionof the nerve trajectory
Gagenhalten
clasp knife rigidity in extremities
examples: can be seen in stroke, ms and catatonia
what is vagal nerve stimulation a treatment for?
- adjunctive treatment of refractory partial complex epilepsy (left vagal nerve)
- intractable MDD
what is carbamazepine approved for?
temporal lobe and generalized epilepsy and trigeminal neuralgia
auscultation of the head that reveals a bruit is a classic halmark of what?
arteriovenous malformation (seizures are commonn presenting symptom of AVM, can rupture and have intracerebral bleeding)
colloid cyst of the third ventricle
- presents in middle aged adults
- round, well circumscribed lesion situated in anterior aspect of the third ventricle, “button nose” in middle of ventricular system
- intermittent headaches, drop attacks
hallervorden-spatz
rare autosomal recessive disease of childhood onset that presents with combination of dementia and parkinsonism
- enzymatic deficiency in cysteinee dioxygenase, leads to increased cysteine in the brain which chelates iron which deposits in the basal ganglia
what type of amnesia is most commonly associated with alcohol abuse
anterograde amnesia
korsakoff’s syndrome
impaired recent memory and anterograde memory
Vitamin B deficiency
distal sensorimotor peripheral neuropathy , parasthesias, sensory loss, motor weakness
bitamin A deficiency
night blindness
hypervitaminosis A
pseduotumor cerebri
west nile
-arbovirus
most are asymptomatic but 20% develop fever and 1 in 150 pts go on to have meningitis or encephalitis picture. may progress to demylinating or axonal neuropathy
serious infection may respond to IV anti west nile virus immunoglobulin
arenavrius
rodant borne and infect humans when a person comes in contact with infected rodant fecal matter
- Ex: lymphocytic choriomeningitis and Lassa fever
filovirus
Ex: ebola and marburg
reservoir not known
sever hemorrhagic encphalitis and myositis and muscle pain
retroviruses
HIV, HTLV-1, HTLV-2
papovavirus
JC virus causative pathogen of PML
what chromosome is amyloid precursor protein found on?
21
- amyloid precursor protein is broken down to form B-amyloid protein, which is a major component of senile plaques
sarcoidosis
-granulomatous disease
-neuro: cranial neuropathies most common manifestation of neurosarcoidosis.
- treatment: corticosteriods or immunosuppressive therapy
- diagnosis: clinically but can be confirmed by muslce biopsy or an elevated agniotensin converting enzyme in CSF
Kleine-Levin syndrome
periods of hypyersomnia with periods of normal sleep in between
- during periods of excessive sleep pts wake up and exprerience apathy, irritability, confusion, voracious eating, loss of sexual inhibitions, disorientation, delusion, hallucinations, memory impairment, incoherent speech, excitation and depression
onset 10-20 years of age and goes away in 40s
what is the most sensitve test for myasthenia gravis
demonstration of increased “jitter” by a single-fiber EMG
- using AchR-ab in serum, repetitive stimulation and single-fiber EMG identify all MG cases
MRI of huntingtons would show what?
caudate and cerebral atrophy
- the caudate atrophy alters the appearance and configuration of the frontal horns of the lateral ventricles. also in general the ventricles are diffusely enlarged
Lewy body demential core features
VH, fluctuating cognition, parkinsonism
What are the core features of Parkinson’s disease?
resting tremor, rigidity, postural instability and hpyokinesia/bradykinesia
Classic parkinsonian tremor
4-6 per second pill rolling tromor
resting tremor
What is the most replicable finding in PD?
- loss of pigmented cells in the substania nigra and other pigmented dopaminergic tracts
- must be a loss of at least 80% of dopamanergic cells bodies before the clinical symptoms manifest
is PD considered a synucleinopathy?
yes
- abnormal accumulation of alpha synuclein protein in the brain
Lewy body
an eosinophilic cyotoplasmic inclusion that consists of a dense core surrounded by a halo of radiating fibrils, the primary structural component of which is alpha synuclein
- found pervasively in the substania nigra of pts with PD
carbohydrate-deficient transferrin
- test used to detect heavy levels of alcohol consumption -not a good screening test but useful in monitoring abstinence or increased alcohol consumption
wilson’s disease
- autosomal recessive
- disorder of copper metabolism
- many pts present in childhood
treatment: copper chelation w/ d-penicillamine
primary torsion dystonia
- commonly seen in individuals w/ Ashkenazi Jewish descent
- action induced dystonia of the leg or arm
- dystonic movements spread over the trunk and other limbs
- cognition generally intact
Stem occlusion of MCA
- contralateral hemiplegia
- conjugate eye deviation toward the side of the infact
- hemianesthesia
- homonymous hemianopsia
- global aphasia (if dominant hemisphere)
- hemineglect (if non-dominant hemisphere)
Upper division MCA infarct
- Broca-type aphasia
pure alexia without agraphia
left posterior cerebral artery territory
- splenium of corpus collosum
- can write but cannot read their own writing
- speech, auditory comprehension, and repetition are intact
marfans syndrome
autosomal dominant
connective tissue desase associated with defects in fibrillin
polymyalgia rheumatica
aching in proximal and axial joints, proximal myalgias, significant morning stiffness
- associated with temporal arteritis
what is used for migraine prevention
fda approved: depakote, topiramate
off label: desipramine , gabapentin
rupture of AVM causes what?
subarchnoid hemorrhage
- majority are anterior circulation
HD
60% begin with motor signs
15% with behavioral signs
25% with both
- ability to generate saccadic eye movements and their speed is often the earliest sign
- clumsiness and fidgetiness that evolves into chorea
- dysarthria and dysphagia
what do you see in pathology of HD
prominent neuronal loss and gliosis in the caudate nucleus and putamen, along with regional and more diffuse atrophy
what is used to reducing chorea in HD
tetrabenazine
stiff person syndrome
- in 80% develops as nonparaneoplastic phenomenon in association w/ diabetes and polyendocrinopathy, and antibodies to glutamic acid decarboxylase
- fluctuating rigidity of the axial musculature with superimposed spasms. primarily affects lower trunk and legs but can extend to arms shoulder neck
- usual precipitating factors to muscle spasms are emotional upset and stimuli
- rigidity disappears during sleep or general anesthesia
- paraneoplastic form associated with breast/lung/hodgkin w/ autoantigen being amphiphysin-treatment: treatment of tumor and corticosteriods
Treatment: IV IG and gaba agents for symptomatic relief
what does the awake mind require
reticular activating system with its way stations in the brain stem and thalamus as well as an intact cerebral cortex
cervical radiculopathy
symptoms: pain in the neck with radiation to the arm
myelopathy often causes hyperreflexia true?
yes
what are common finding of cervical myelopathy?
leg spasticity, upper extremity weakness or clumsiness or sensory changes in the arms legs or trunk. some pt experience lhermittes sign
lesions C4-C5
affect the C5 nerve root
- pain, parasthesias and sometimes loss of sensation at the shoulder
- weakness in deltoid, biceps and brachioradialis muscle
- biceps and supinator refelxes may be lost
lesions C5-C6
- affect C6 nerve roots
- parastheisias in the thumb or lateral distal forarm
- weakness in the brachioradialis, biceps, or tricpts
- bicep and brachioradialis reflexes may be diminished
C6-C7 lesions
- affect C7 nerve root
- paresthesias-indexm middle ring finger
- weakness tricepts and pronators
- triceps tendon reflex may be diminished
extensor pantar response
babinski sign
histology of picks
ballooned cells and intraneuronal inclusions
criteria for picks disease
loss of insight, easy distractibility, withdrawal, impulsiveness, poor self care, perseveration, featuers of the kluver buy syndrome and dimished verbal output
- FTD males and females affected equally
- FTD makes up 10-15% of neurodegenerative dementias
hyperkalemic periodic paralysis
mutations in the alpha subunit of the sodium channel
- autosomal dominant
- onset occurs in infancy or early childhood
- weakness provoked by potassium exposure
what is used for maintenance prevention for cluster headaches?
methysergide
hemicrania continua
continuous unilateral headache of moderate intensity
- more common in females (2:1) , average onset 28 years
- usually continuous and unremitting puntuated by painful unilateral exacerbations lasting 20 minutes to several days, accompanied by one or more autonomic feature
- pts respond to prophylactic indomethacin
lacunar infarct risk factor to control
hypertension, smoking, diabetes
management of pts with lacunar infarcts
- prevention of microangiopathy, use of platelet antiaggregants
CJD
affects men and women equally, manifests in late middle age
-memory loss, cerebellar or visual oculomotor signs, pyramidal and extrapyramidal signs and a variety of involuntary movements especially myoclonus
- EEG periodic sharp wave complexes
CSF: 14-3-3 protein kinase inhibitors and tau protein
- MRI: hyperintense signal in the basal ganglia
JC virus infects waht cells leading to what ?
myelin-producing oligodendrocytes- resulting in cell lysis and demyelination
what is clinical presentation of JC
focal neurological deficits
seizures
cognitive impairment
psychomotor retardation
inattentiveness
etiologic agent responsible for CNS lymphomac
epstein bar virus
clinical presentation of CNS lymphoma
- progressive personality changes
- seizures
- increased intracranial pressure
what is the major morbidity associated with idiopathic intracranial hypertension?
visual loss related to optic nerve dysfunction
what are dandy criteria for idiopathic intracranial hypertension
1) signs and symptoms due to increased ICP
2) normal neurological exam except for abducens palsy
3) neuroimaging excluding other cause of ICP
4) normal CSF parameters except elevated opening pressure
- headache, transient visual obsurations, pulsatile intracranial noises or double vision
Episodic memory
Mediated by:
- medial temporal lobes
- anterior thalamic nuclei
- mamillary bodies
- fornix
- prefrontal cortex
- explicit, declarative awareness
- what you ate for lunch
semantic memory
- inferolateral temporal lobes
- explicit, declarative awareness
- knowing the first president
procedural memory
- basal ganglia, cerebellum, supplementary motor area
- implicit or explicit, non declarative awareness
- how to drive (explicit)
- learning how to type your phone number without thinking about it (implicit)
acute and subacute transverse myelitis
isolated spinal cord dysfunction over hours to days (no evidence of compressive lesion)
- presumed to be autoimmune in origin (some pts have this after febrile illness)
- motor and sensory deficits below the lesion
women or men more likely to have psychogenic seizures
women
what is the most common CNS tumor in NF1
optic glioma
MS medication
Interferon B-1b (betaseron)- can cause depression
Glatiramer acetate (copaxone)
subcortical arteriosclerotic encephalopathy (Binswanger disease)
- subcortical vascual dementia
- associated with more extensive white matter lesions and ventricular enlargmeent
- gradual onset of cognitive difficulties
- weakness discordination slurred speech
korsakoff syndrome
lesions in the diencepahol and temporal lobes
anterograde and retrograd amnesia
Marchiafava–Bignami sydnrome
alcohol
- mental and motor slowing, perosnlaity changes, incontinence, dysarthria, seizures hemiparesis
- sucking, grasping and gegenhalten may be prominent
- central portion of corpus collusum
progressive supranuclear palsy
second most common form of neurodegenerative atypical parkinsonism
- vertical supranuclear ophthalmoplegia
- axial rigidity
- peudobulbar palsy
- mild dementia
- akinetic rigid state
juvenile myoclonic epilespy
often 12-18 years old
sudden myoclonic jerks of shoulders and arms that occur after awakening
often develop to tonic clonic seizures
many also have absence seaizures
- sleep deprivation, alcohol intake and fatigue precipitate seizures
benign childhood epilepsy with centrotemporal spikes (rolandic epilepsy)
- onset 3-13 years
- seizure characteristics: sematosensory distrubances of the mouth, preservation of consciousness, excess pooling of saliva and tonic or tonic clonic activty of the face and speech arrest when dom hem affected
- seizures typically occur after sleep
what does an isolated painful abducens palsy represent especially in older pts with vascular risk factors
microvascular ischemia-diabetic sixth nerve palsy
- spontaneous resolution over 8-12 weeks is typical
the abducens nerve is the cranial nerve most commonly affected bilarterally in isolation, what does this most often occur from
trauma and increased intracranial pressure
diabetic third nerve palsy
pupillary sparing
infarction of the centrifascicular oculomotor axons due to diabetic vsculopahty of the vasa nervorum
(pupillary motor fibers located peripherally spared)
herpes simplex virus 1 encephalitis
- most common cause of sporadic fatal encephalitis in US
- treat acyclovir
- fever, disorientation, personality changes, focal or generalized seizures, memory disturbances, motor deficits, and aphasia, olfactory hallucinations
CSF: (most important test): increased pressure, 10-1000 lymphocytic pleocytosis
-PCR CSF
MRI abnoramlities in temporal lobs
polymyositis
symmetrical and affects proximal muscles more than distal
- malaise, fever anorexia
CK should always be elevated!
nonartertic anterior ischemic optic neuropahty
most common cause of unilateral optic nerve swelling in adults older than 50
brain death
-coma: unarousable/unresponsive to noxious stimuli
-no spontaneous respirations
-absence of brain stem reflexes-Pupillary, oculocephalic, corneal, and gag reflexes all must be absent, and there should not be any vestibulo-ocular responses to cold calorics
-electrocerebral silence
-absence of cerebral blood flow
-absence of potential reversible causes
*positive apnea test absolutely necessary for declaration of brain death
Guillain barre
- motor weakness
- areflexia
- paresthesias with minor sensory loss
- increased protein in CSF without pleocytosis
cerebellar hemorrhage signs
abrupt vertigo, headache, vomiting, inability to stand and walk, with absence of hemiparesis and hemiplegia
triad:
1. appendicular ataxia
2. horizantal gaze palsy
3. peripheral facial palsy
- neurosurgical intervention indicated
tolosa hunt syndrome
painful inflammation of cavernous sinus
- severe unilateral headache with extraocular palsies involoving 3, 4,5,6 CN
(one sided head pain with paralysis eye muscles)
- responsive to corticosteriods and immunosuppressants
alzheimers survival expectancy
8 years
