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308 Cards in this Set

  • Front
  • Back
What is destroyed in multiple sclerosis?
Oligodendrocytes are destroyed which causes demyelination of CNS axons.
What is particularly interesting in the CSF of a MS patient?
Oligoclonal bands. IgG/albumin ratio is also usually off.
What are Schwann cells?
Myelin producing cells for the PNS
What are Dawson's fingers?
Areas of demyelination surrounding end venules in a longitudinal section of the brain in MS patients.
What cells mediate MS?
T cell-mediated disease, particularly CD4 T cells.
When does MS usually manifest?
3-5th decades
What is an essential part of the clinical presentation pattern of MS?
Dissemination in TIME and SPACE
What type of optic neuritis is a typical presentation of MS?
Central scotoma due to retrobulbar optic neuritis (loss of central vision in 1 eye)
What do T2 or Gadolinium lesions indicate?
Recent plaques. The presence of some recent plaques and some older plaques can fulfill the dissemination need in the DX of MS.
What is included in the DDx of MS?
Acute Disseminated Encephalomyelitis
Idiopathic transverse myelitis
Sarcoidosis
SACD
Infectious myelopathy (HTLV1)
Tumor
What is used to treat acute exacerbations of MS?
High-dose corticosteroids.
What are some treatments for the prevention of MS exacerbations?
Interferon-beta
Glatiramer acetat
Mitoxantrone
Natalizumab
How does natalizumab work?
Natalizumab blocks VLA4 interaction in MS to impairs the binding of these molecules to VCAM so the immune cells cannot cross the BBB.
Used in MS
What is a S/E of mitoxantrone?
Cardiotoxicity
What are some causes of multiple mononeuropathies?
Vasculitis
Diabetes mellitus
Sarcoidosis
Leprosy
Hypothyroidism
Hereditary neuropathy w/liability to pressure palsies
What is the mutation in DMD?
Mutation in the dystrophin gene at Xp21
What is the inheritance pattern of DMD?
X-linked recessive lethal
What is the clinical presentation of a px w/DMD?
Toe-walking, waddling gait, excessive lumbar lordosis, and calf muscle hypertrophy
What is the presentation of Fascioscapulohumeral dystrophy?
In adulthood.
Inability to whistle (mild facial weakness) and weakness of scapulohumeral muscles.
Slow progression.
What is the mutation associated with FSH dystrophy?
Autosomal dominant of 4q35 chromosome
What is the presentation of mytonic dystrophy?
Congenital: respiratory failure & feeding difficulties, hypotonia and cognitive dysfxn
Adult: mild-moderate; slowly progressive weakness of face, anterior neck, and distal limbs w/difficulty relaxing mm. Frontal balding, temporal wasting, cataracts
What is the mutation associated w/myotonic dystrophy and its inheritance pattern?
DMPK gene on chromosome 19.
CTG repeat disease.
Autosomal dominant
What is oculopharyngeal dystrophy?
Autosomal dominant disorder that appears in 4-5th decades.
Ptosis, dysphagia, possible mild weakness.
GCG repeat on chromosome 14q11
What is the inheritance patterns of mitochondrial myopathies?
Maternal pattern of transmission w/variable linkage.
What are the characteristics of MERRF?
myoclonus, generalized seizures, ataxia, dementia, hearing loss, optic atrophy, muscle weakness
What are the characteristics of MELAS?
encephalopathy, high lactic acid level in serum or CSF, stroke-like episodes
What are the characteristics of Kearnes-Sayres syndreom?
Mitochondrial myopathy.
Progressive ophthalmoplegia, retintis pigmentosa, cardiac conduction abnormalities, myopathy
What are the general characteristics of glycolytic defects?
Dynamic, related to exercise intolerance as exercise-induced cramps, pain or myoglobinuria.
Due to a defect that results in accumulation of a substrate or failure to produce an end product
What is type II glycogenosis?
Acid maltase deficiency
Infantile form=Pompei's disease; presents w/hypotonia, failure to thrive in 1st 3mos
Adult presents in 3rd-4th decade w/proximal weakness
What is type V glycogenosis?
Myophosphorylase deficiency (McArdles)
Exercise intolerance w/muscle pain, cramps, myoglobinuria
What is CPT2 deficiency?
CPT2 is needed to get carnitine into the mitochondria for lipid metaboilsm.
Autosomal recessive on 1p32
Presents w/myoglobinuria & renal failure
What is malignant hyperthermia?
Autosomal dominant disorder
Severe reaction to anesthesia
Severe muscle rigidity, myoglobinuria, fever, tachycardia, arrhythmias.
What are periodic paralyses?
Myopathies characterized by mutations in muscle membrane ion channels.
Hypokalemic=mutation in msucle membrane Ca channel
Hyperkalemic=mutation in sodium channel
What is polymyositis?
Adult onset.
Present w/weeks-months of proximal weakness & neck flexor weakness.
What is the cause of polymyositis?
HLA restricted antigen specific cell mediate immune response against muscle fibers
What is included in a WU for polymyositis?
Muscle enzymes-->elevated CK, ANA, anti-Jo-1
EMG show irritable myopathy
Bx=inflammation w/invasion of non-necrotic fibers by CD8 T cells and macrophages
What is the tx for polymyositis?
immunomodulation
What is the presentation of dermatomyositis?
Weakness of neck flexors, shoulder girdle & pelvic girdle.
Rash precedes weakness-->heliotrope rash.
Heliotrope rash=purple w/periorbital edema
Gottrens sign over knuckles
SubQ calcifications may be seen kids.
What is the pathophysiology of dermatomyositis?
Vasculopathy of the muscle and other organs.
Humerally mediated microangiopathic disroder w/deposition of IgM C3
What is seen in a WU for dermatomyositis?
CK is elevated.
EMG=irritable myopathy
Bx=perifascicular atophy & perivascular inflammation
What is tx of dermatomyositis?
Immunomodulation w/IVIg & highdose steroids
What is inclusion body myositis?
Occurs in adults over 50
Slowly progressive weakness of both distal and proximal muscles w/prominent weakness of quadriceps & wrist & finger flexors
What is seen in a muscle Bx of inclusion body myositis?
Invasion of non-necrotic fibers by CD8+ T cells. Eosinophilic cytoplasmic inclusions & deposition of amyloid.
What is the tx for inclusion body myositis?
No tx
What can cause viral myositides & what is seen in the disease?
CMV, influenza, coxsackie, EBV, mumps.
CK 500x NL
What bacteria can cause inflammatory muscle disease?
Staph aureus in the tropics. Erythema, swelling, tenderness seen.
What drugs cause necrotizing myopathies?
Cholesterol-reducing drugs (statins, gemfibrozole, clofibrate)
Amphiphilic drugs (chloroquine & amiodorone)
What causes anti-microtubular myopathy?
Colchicine.
What causes drug-induced mitochondrial myopathy?
AZT
What causes drug-induced inflammatory myopathy?
Tryptophan, D-penicillamine, procainamide (RARE)
What causes myopathy secondary to impaired protein synthesis?
Long-term steroid use (prednisone)
What is the incidence distribution of myasthenia gravis?
Peak 1: 2nd & 3rd decades, F>>M
Peak 2: 6th & 7th decade, M>>F
What is the pathophysiology of myasthenia gravis?
Antibodies directed against AChR reduces the number of functional AChRs.
This results in EPP of diminshed amplitude.
Fatigue results
What organ may be enlarged in myasthenia gravis?
Thymus, some may have thymoma.
What is used to test for myasthenia gravis?
Repetitive nerve stimulation
Tensilon testing (edrophonium)
Lab tests for anti-AChR antibodies
CXR for thymus
What is the tx for myasthenia gravis?
Anticholinesterase agents (pyridostigmine)
Immunosuppression
Immunomodulation
Thymectomy for px w/thymomas
What is Eaton-Lambert syndrome?
Antibody mediated paraneoplastic disorder of the presynaptic junction. Autoantibodies directed against presynaptic Ca2+ channels-->reduce amt of ACh released.
Often related to small cell lung CA
What is the clinical presentation of Eaton-Lambert syndrome?
Dry eyes & mouth
Constipation & impotence
How does botulinum toxin work?
Interferes with transmitter release at motor nerve & at presynaptic cholinergic terminals. Appears to affect sensitivity to Ca2+
What is the most common mutation related to DMD?
Deletion of exons of dystrophin gene.
What is the mutation associated with congenital muscular dystrophy?
Mutations in laminin-2 (link to the basement membrane of muscle)
What is the inheritance pattern seen in most cases of hereditary ALS?
Autosomal dominant
What genes are mutated in ALS?
SOD1: missense mutations-->acquisition of toxic property
TDP-43 & FUS/TLS-->RNA binding proteins needed for correct splicing
What is SMA?
Spinal muscular atrophy
Affects LMNs
Destruction of motor neurons in anterior horn
What is the inheritance pattern of SMA?
Autosomal recessive
What is Type 1 SMA?
Werdnig-Hoffman disease
Most severe.
Onset before 6mos
Death w/in 1st 2 years
What is Type II SMA?
Intermediate in severity
Onset at 1.5 years
Px never learn to walk
What is Type III SMA?
Kugelberg-Welander
Onset after18 months
Patients able to stand & walk
What is the gene mutated/lost in SMA?
SMN1
There is both SMN1 & SMN2.
Can be deletion of SMN1 or conversion of SMN1 to SMN2.
SMN2 is not SMN1-->lacks exon 7-->not enough protein produces.
More SMN protein-->milder phenotype
What are initial signs of CMT?
stumbling gait, awkardness while running, ankle weakness in childhood.
What is CMT?
Motor sensory neuropathies
What is the difference between CMT1 and CMT2?
CMT1 has reduced nerve conduction velocity and demyelination
CMT2 has normal nerve conduction velocity and not obvious demyelination
What causes most cases of CMT1?
duplication of PMP22
What do mutations in MPZ gene cause?
Similar to PMP22 mutations-->CMT1
What does PMP22 code for?
Protein in compact myelin
What is connexin and what is it related to?
CMT neuropathies
gap junction protein needed for nutrient entrance
What gene is mutated in Huntington's chorea?
huntingtin
CAF expansion--->polyglutamate stretch produced-->protein inclusion.
What is affected in spinocerebellar ataxia?
cerebellar purkinje cells-->uncoordinated walking.
What is affected in spinobulbar muscular atrophy?
motor neurons, polyglutamate expansion in androgen receptor
What is the mutation in Fragile X syndrome?
CCG repeat resulting in extensive methylation of 5' end of FMR gene-->shutdown of FMR expression
What the mutation in myotonic dystrophy?
expansion in 3' UTR of a gene resulting in RNA accumulation
What is the mutation involved in Friedrich ataxia?
GAA expansion in intron that affects splicing of Frataxin pre-mRNA
What are the symptoms of dominant spinocerebellar ataxia?
Gait ataxia, dysarthria.
Specific versions have ocular disorders, extrapyramidal signs, peripheral neuropathy, intellectual deterioration, seizures
What is accumulated in NFTs in AD?
tau and ubiquitin
What is a mutation associated w/AD?
APP mutation causing missplicing to produce amyloid deposit.
What are the dominant genes related to Parkinson's disease?
alpha-synuclein (component of lewy bodies)
LRRK2
UCH1 (ubiquitin terminal ester)
What are some recessive genes related to Parkinson's disease?
DJ1
PINK1
What do lewy bodies consist of?
synuclein
What is Gerstmann-Straussler-Scheinker?
Progressive degenerative disease w/prominent cerebellari nvolvement w/ataxia
Prion disease
What mutations are related to epilepsy?
Mutations in ligand & voltage gaited channels.
What is a myelinoclastic disorder?
Disorder in which myelin breaks down due to some known or unknown exogenous factor.
What is a leukodystrophy?
Disorder in which myelin breaks down due to mutations in genes responsible for myelin metabolism or maintenance.
What are the plaques seen in MS brains?
Active plaques: densely cellular w/lots of lymphocytes, foamy macrophages, few astrocytes
Inactive plaques: paucicellular, densly gliotic, depletion of oligodendrocytes, reduced # of axons & axonal atrophy
Shadow plaques: myelin reduced but not absent, thin myelin sheath on remyelinated axons
What is the clinical presentation of ADEM?
Abrupt onset of neurologic syndrome w/persisent high fever & headache
Many have hx of febrile illness 2-14 days before
What is the neuropath of ADEM?
Macro: edema in brain, possible herniation
Micro: small engorged vv. surrounded by foamy macrophages & lymphocytes, sleeve-like demyelination around vv.
What causes SACD?
B12 deficiency.
Usually seen in the setting of atrophic gastritis.
Paresthesiae of limbs early on.
What is the neuropath of SACD?
Macro: shrunken, discolored posterior & lateral columns in spinal cord in lower C & T spine.
Micro: early lesions show symmetric, spongy vacuolation & degeneration of myelin sheaths in T spine in posterior columns then CST & SCTs. Myelin breakdown leads to axonal degeneration & gliosis.
What is central pontine myelinolysis?
Monophasic demyelinatino of basis pontis.
Setting: rapid correction of hyponatremia, extensive skin burns, alcoholics, liver transplant px.
Rapid onseto f confusion, limb weakness, gaze palsies, dysarthria, dysphagia, hypotension
What is the neuropath of central pontine myelinlysis?
Basis pontis has central gray discolored region.
Active demyelination w/reactive astrocytes and macropahges. rare lymphocytes.
What causes PML?
JC & BK viruses (polyomaviruses)
What is the clinical presentation of PML?
Focal neurologic deficits, dysarthria, limb weakness, visual disturbances, ataxia, personality changes, seizures. Death w/o immune reconstitution
What is the neuropath of PML?
WM has small foci of gray discoloration. Multifocal.
Areas of demyelination w/scant perivascular lymphocytes. Nuclear inclusions present
What is Krabbe's disease?
Galactocerebroside-beta-galactosidase deficiency
Lysosomal.
Autosomal recessive
Onset in infancy w/failure to thrive & vomiting. Death at 1-2
What is the neuropath of Krabbe's disease?
Mod-severe atrophy in narrow gyri & wide sulci (WM is gray & firm due to gliosis)
Extensive loss of myelin & oligodendrocytes. See globoid macrophages which tend to be multinucleated & cluster around blood vessels.
What is metachromatic leukodystrophy?
Arylsulfatase A def.(AR)
Present 1-2yo w/ataxia, spasticity, absent reflexes in LLs. Optic atrophy & seizures. Die at 3yo
What is the neuropath of metachromatic leukodystrophy?
WM is firm & chalky.
Demyelination w/extensive axonal loss. Depletion-loss of oligodendrocytes w/severe gliosis. See brown metachromasia in froze nsections--->accumulation of sulfatide in tissue.
What is adrenoleukodystrophy?
ALDP mutation-->X-linked.
Infant: hypotonia, seizures, failure to thrive, deafness, retinal degeneration
Childhood: loss of skills, dementia, cerebellar disorder
Adulthood: clumsiness, spasticity, dementia.
Very long chain fatty acids accumulate in tissue
What is the neuropath of adrenoleukodystrophy?
NL brain externally.
Firm gray WM w/symmetric caudal-->rostral gradient of severity (frontal better than caudal)
U-fibers spared. Severe demyelination in central cerebral WM & commissures.
Adrenals are atrophic & cortex is replaced by ballooned cells w/striated cytoplasm
What causes Tay-Sachs?
Autosomal recessive mutation in hexosaminidase A deficiency-->accumulation of GM2 ganglioside
What is the clinical presentation of tay-sachs?
NL for first few months then fairulty to acquire coordinated skills, possible seizure dev.
Easily startled. Cherry red spotseen in macula & blindess at ~1yr
Large heads at 2 years
Death at 2-4
What is the neuropath of taysachs?
Gyral atrophy w/loss of WM
Ballooned neurons through CNS & PNS. Foamy neurons.
PAS positive
What are the mucopolysaccharidoses?
Disorders due to defects in genes that metabolize glycosaminoglycans (GAGs) resulting in GAG accumulation in neurons
Hunters and Hurlers
What are the inheritance patterns of Hunters & Hurlers?
Hunters: X-linked
Hurlers: AR
What is the clinical presentation of Hunters?
Mental retardation, iduronidase sulfatase def., dermatan sulfate accumulates
What is the clinical presentation of Hurlers?
Mental retardation, iduronidase sulfatase def., dermatan sulfate accumulates, dwarfism, corneal clouding, skeletal deformities (kyphosis, short neck, coarse facial features)
What is the neuropath of mucopolysaccharidoses?
Macro: dural & meningeal thickening that can cause obstructive hydrocephalus. cut surface of WM can cause small perivascular cavities filled with foamy macrophages.
Micro: neuronal storage that varies with degree of mental retardation
What is the neuropath of AD?
Reduced brain weight with small, thin cerebral gyri & widened sulci
Enlarged third & lateral ventricles
Extracellular amyloid plaques & NFTs
What is the neuropath of dementia w/lewy bodies?
More atrophy in limbic areas.
Lewy bodies in several areas. Neuronal loss in substantia nigra & locus cereleus
What is the neuropath of huntington's chorea?
Cerebral atrophy & reduced brain weight. Atrophy of caudate, putamen, and globus pallidus.
Neuronal loss & gliosis in areas of basal ganglia.
What is the neuropath of Parkinson's dsease?
Midbrain & pons have loss of pigment in substantia nigra & locus cereleus, which both also show neuronal loss.
Lewy bodies must be found for dx.
What is the neuropath of SCA?
Atrophy of cerebellum, pons, and inferior olivary nuclei. Neuronal loss in SC.
What is the clinical presentation of Friedrich's ataxia?
Onset before 15
Ataxia of gait followed by limb ataxia, dysarthria, loss of proprioception & vibration sense in LL, generalized areflexia, exensor plantar response.
What is the most common mutation in Friedrich's ataxia?
GAA repeat
What is the neuropath of Friedrich's ataxia?
SC: degeneration & gliosis in posterior columns, distal degeneration of CST & SCT
Medulla: degeneration & neuronal loss in accessory cuneate & gracile nuclei.
Cerebellum: gliotic WM, cortex usually spared, dentate nucleus has marked neuronal loss & SCPs are atrophied.
DRG have neuronal loss.
What is the neuropath of ALS?
Shrunken, gray anterior nerve roots.
SC can be atrophic
Loss of motor neurons & gliosis in spinal cord & brainstem (LMNs) and motor cortex (UMNs)
SC has degeneration of CST in lateral funiculus. Skeletal muscle atrophies.
What is a small, linear hemorrhage?
Nerve fiber layer hemorrhage
What is a small, round hemorrhage?
Deep intraretinal hemorrhage
What is a large, red hemorrhage with vessels anterior to it?
Subretinal hemorrhage
What is a large, red hemorrhage anterior to the blood vessels?
Preretinal hemorrhage
What is a large, dark hemorrhage?
Choroidal
What hemorrhages come from choroidal vessels?
Subretinal or choroidal
What vessels do preretinal hemorrhages come from?
Disc & retinal vessels
What vessels do nerve fiber layer hemorrhages come from?
Disc & retinal vessels
What vessels do deep intraretinal hemorrhages come from?
Retinal vessels
Where do disc vessels bleed?
Only near the disc.
What is the etiology of a hemorrhage from disc vessels?
Neurologic
What is the etiology of a hemorrhage from retinal vessels?
Systemic disease
What is the etiology of a hemorrhage from choroidal vessels?
Inflammatory or degenerative disease
What is a flat black disc shaped pathology?
Choroidal nevus
What is an elevated black disc shaped pathology?
Melanoma
What is an angular black pathology?
Retinitis pigmentosa
What is a doughnut-shaped black pahtology?
Chorioretinal scar
What is a diffuse balck pathology indicative of?
Inflammatory or degenerative disease
What are the topical beta-blockers?
timolol, levobunolol, carteolol
What are the S/E of topical beta blockers?
Bradycardia, shortness of breath, masked hypoglycemia, increased lipid panel
What are the S/E of topical prostaglandins?
Redness, longlong eyelashes, iris color change (permanent), macular edema, reactive HSV keratitis
What are the S/E of carbonic anhydrase inhibitors?
kidney stones, numbness, malaise, metabolic acidosis
How do fluoroquinolone work?
DNA gyrase inhibitors
What is hypopyon?
layer of WBCs at the inferior aspect of the anterior chamber
What is keratic precipitates?
deposits of inflammatory debris on the endothelial surface of the cornea
What is anterior synechiae?
Inflammatory adhesions between the peripheral iris and the peripheral cornea
What are posterior synechiae?
Inflammatory adhesions between posterior iris and lens
What is arteritic ischemia optic neuropathy related to?
temporal arteritis
What is nonarteritic ischemic optic neuropathy related to?
diabetes and HTN
What are the most common benign ocular tumors in children?
Dermoid
What is the most common benign ocular tumor in adults?
Cavernous hemangioma
What is dacryocystitis?
Infection of the nasolacrimal duct
What is the most common malignant orbital tumor in children?
Rhabdomyosarcoma
What do you see with Horner's syndrome?
TRIAD
Ptosis
Miosis
Anhidrosis
What is a 1st order Horner syndrome?
Central lesion
Could be stroke, tumor or demyelination
What is a 2nd order Horner syndrome?
Preganglionic
Pancoast tumor
Carotid dissection
Surgery
Trauma
What is a 3rd order Horner syndrome?
Cavernous sinus mass
Cluster headache
ICA disease
What does a CNIII palsy indicate?
Aneurysm at junction of PCoA & ICA until proven otherwise
What is caused by giant cell arteritis?
Sudden, profound vision loss
AION
What is this and what is it evidence of?
Hypopyon
Evidence of anterior uveitis or iritis.
What is the white pathology seen here?
Hard exudate
What is the pathology seen here?
Hard exudate
What is the pathology seen here?
Chorioretinal scar
What is seen here?
Keratic precipitate
what is the white pathology here?
Drusen
What is the white pathology seen here?
Soft exudate
What is the white pathology here indicative of?
Retinitis
What disease is seen here?
HSV keratitis
What is seen here?
Multifocal chorioretinal scarring indicating ocular histoplasmosis
What is this?
Vitritis: inflammatory cells in the vitreous behind the lens!
What is this?
Retinal vasculitis
What is this?
Toxoplasmosis
What is this?
Choroidal nevus
What is this red pathology?
Choroidal hemorrhage
What is this red pathology?
Deep intraretinal hemorrhage
What is this red pathology?
Nerve fiber layer hemorrhage
What is this red pathology?
nerve fiber layer hemorrhage
What is this red pathology?
Nerve fiber layer & preretinal hemorrhage
What is this red pathology?
Preretinal hemorrhage
What is this red pathology?
Preretinal hemorrhage
What is this red pathology?
Subretinal choroidal hemorrhage
What is this red pathology?
Subretinal hemorrhage
What is this black pathology?
Malignant melanoma
What is this black pathology?
Choroidal nevus
What is this black pathology?
Pigment crescent
What is this black pathology?
Retinitis pigmentosa
What is the C/D ratio?
0.1
What is the C/D ratio?
0.7
What is the C/D ratio?
0.8
What optic disc pathology is seen here?
Optic atrophy
What is seen here?
Scleral crescent, a normal variant
What pathology is seen here?
0.7 C/D ratio
Disc edema
What pathology is seen here?
0.7
Optic atrophy
What is this white pathology?
Soft exudates/cotton wool spots
What is this?
Central retinal artery occlusion
What is this?
Flare and/or cell
What is this white pathology?
Soft exudate/cotton wool spots
What is this?
Ischemic optic neuropathy
What is this?
PDR
What is this?
Posterior synechiae
What is this?
Retinal artery occlusion
What is this white pathology?
Soft exudate/cotton wool spot
What is this?
Retinitis pigmentosa
What is this?
Retinitis pigmentosa
The ankle jerk reflex is related to what cord levels?
S1, S2
The patellar reflex is related to what cord levels?
L3, L4
The biceps reflex is related to what cord levels?
C5, C6
The triceps reflex is related to what cord levels?
C7, C8
Describe upper brachial plexopathy.
C5-C6
weakness of shoulder abduction
internal rotation of shoulder
elbow flexion
waiter's tip position
Describe lower brachial plexopathy.
Ulnar and median weakness affects intrinsic mm. of hand & wrist flexion
Describe a preferential lumbar plexopathy.
Proximal muscle weakness, loss of patellar reflex.
Describe a preferential sacral plexopathy.
weakness below knee & loss of achilles reflex
LMN signs.
Loss of reflexes
Decreased tone
Muscle atrophy
Fasciculations
Flexor plantar response
UMN signs.
Hyperreflexia
Increased tone/spasticity
Extensor plantar response
Positive Romberg indicates what?
Sensory ataxia
UMN vs. LMN in face.
UMN spares forehead.
Describe cerebellar ataxia.
Incoordination
Hypotonia
Oculomotor abnormalities
Dysarthria
Parietal lobe damage results in what?
Either side: impairment of sensation on contralateral side of body, decreased 2 pt discrimination, sensory inattention, extinction, sensory agnosia
Dominant side: apraxias (Gerstmanns syndrome=dyscalculia, L-R agnosia, finger agnosia, dysgraphia)
Nondominant side: neglect of opposite side, impaired constructional abilities
What is the result of bilateral temporal lobe lesions?
Profound memory loss
What is the result of lesions to the dominant temporal lobe?
decreased verbal learning
What is the result of lesions to the nondominant temporal lobe?
decreased visual learning
What is the result of frontal lobe lesions?
Hemiparesis
Personality changes
Motor difficulties (motor cortex)
Describe Broca's aphasia.
Damage to dominant inferior frontal gyrus.
Nonfluent speech but good comprehension
Associated w/contralateral hemiparesis
Describe Wernicke's aphasia.
Lesion in dominant superior temporal gyrus.
Fluent speech with poor comprehension.
Poor awareness of problem.
Describe conduction aphasia.
Lesion to arcuate fasciculus, which connects Wernickes & Brocas areas.
Difficulty with repetition.
What is a typical presentation of polyneuropathy?
Stocking-glove loss.
Usually symmetrical
What is a sensory neuropathy?
Loss of neurons at DRGs.
profound sensory loss in extremities, can be painful.
Pes cavus is related to what disease?
CMT
Motor sensory neuropathy
What is Guillain-Barre?
An acquired demyelinating polyneuropathy associated with recent infection.
Results in slowed NCVs
Normal CSF w/increased protein
What is the diagnostic criteria for Guillain barre?
1. Weakness that is ~symmetric in all limbs
2. Paresthesias in feet & hands
3. Areflexia or hyporeflexia by 1 wk
4. Progression over days-1 month
5. Elevated CSF protein w/in 3wks
6. Slowed NCVs
What is seen in dying back distal axonopathy?
Longest & largest axons affected @ nerve terminal
Due to metabolic abnormality
Can be associated w/diabetes, uremia, alcohol, vitamin def., drug tox.
What are the lab features of dying back distal axonopathy?
Normal NCVs-->weakness & sensory loss due to nerve fiber loss
CSF normal
What disorders are associated with a CAG repeat expansion?
Huntingtons
SCA
Spinal bulbar muscular atrophy
What disorders are associated with CGG repeat?
Fragile-X
What disorders are associated with CTG repeat?
Myotonic dystrophy
What disorders are associated with GAA repeat?
Friedrich ataxia
What is seen here?
Active MS plaque with accumulation of T cells near vessels.
What disease could this be?
ADEM
What is seen here?
Adrenoleukodystrophy.
See ballooned neurons.
What is seen here?
Perivascular lymphocytes.
Adrenoleukodystrophy
What is this?
ALS
What is this an image of?
Amyloid plaques of AD
What is the disease seen here?
Central pontine myelinolysis.
Loss of myelin in basis pontis.
What disease is this?
Friedrich's ataxia with posterior column loss (gracile>cuneate)
What is seen here?
Atrophy of caudate & putamen related to Huntingtons
What is seen here?
Paucicellular, densely gliotic inactive MS plaque
What are these related to?
Globoid macrophages related to Krabbe's disease.
Pathognomic.
What are these?
Lewy bodies
DLB
What is this indicative of?
metachromatic dystrophy
Should be violet, but is brown to sulfatide accumulation.
What is seen here?
Accumulation of ganglioside in neurons related to mucopolysaccharidoses.
What is seen here?
Nuclear inclusions seen in PML
What are the possible causes of distal axonopathies?
Diabetes
Uremia
Amyloidosis
Alcohol
Thiamine deficiency
Phenytoin toxicity
What conditions can affect DRG cell bodies causing neuronopathies?
Cancer (paraneoplastic)
Sjogrens
Vitamin B6 toxicity
Cisplatin drugs
HIV (rare)
Drusen indicate what?
May be age-related macular degeneration.
Unrelated to systemic disease.
Hard exudates indicate what?
HTN, diabetes, venous occlusion, macular degeneration
Soft exudates indicate what?
Severe systemic occlusive microvascular disease
HTN, collagen disease, AIDS
Myelinated nerve fibers indicate what?
Normal variant
List 3 mechanisms used by antiseizure drugs.
Enhance GABAergic transmission
Inhibit glutamatergic transmission
Block voltage-gated Na+ and Ca2+ currents
List the drugs that work by slowing the rate of recovery of voltage-gated sodium channels from inactivation.
Hydantoins
Iminostilbenes
Carboxylic acids
Lamotrignine
What are the iminostilbenes?
carbamazepine
oxcarbezpine
List the drugs that work by allowing more Cl- to enter thus hyperpolarizing the neuron.
Barbiturates
Benzodiazepines
What are the barbiturates?
Phenobarbital
Primidone
What are the benzodiazepines?
Diazepam
Clonazepam
What is primidone metabolized to?
Phenobarbital and PEMA
What drugs reduce the current through T-type Ca2+ channels in thalamic neurons?
Succinimide (ethoximide)
Carboxylic acids
What drugs inhibit the metabolism of GABA?
Vigabatrin
Gabapentine
Tiagabine
What is a side effect of zonisamide and what is this drug used for?
Antiseizure drug.
Also causes weight loss.
How does topiramate work?
Blocks some Glu receptors
Enhances GABA activity
Blocks sodium channels
Also causes weight loss
What is a drug specific for absence seizures?
Ethosuximide
What is used to treat LGS seizures?
Valproate
Benzos
What is the treatment for status epilepticus?
Diazepam followed in 30min by phenytoin or fosphenytoin.
May need to use phenobarbital.
What antiseizure drug can cause skin rashes?
Lamotrignine
What drugs increase phenytoin metabolism?
Phenobarbital
Carbamazepine
What drugs decrease phenytoin metabolism?
Chloramphenicol
Dicumarol
Cimetidine
Sulfonamide
Isoniazid
What drugs inhibit the metabolism of carbamazepine?
Erythromycin
Isoniazid
Propoxyphene
What are some unique S/E of phenytoin?
Gingival hyperplasia
Hirsutism
Megaloblastic anemia
Inhibition of antidiuretic hormone release
Birth defects: clefts!
What antiseizure drug causes spina bifida?
Valproate
What drugs do NOT work in absence seizures?
Phenytoin
Carbamazepine
What antiseizure drugs are also used to treat mania?
Carbamazepine
Oxcarbazepine
Gabapentin
Valproate
Topiramate
What are the nondepolarizing neuromuscular blocking drugs?
-iums
Tubocurarine
Metocurine
Gallamine
What is a depolarizing neuromuscular blocking drug?
Succinylcholine
What are some S/E of neuromuscular blocking drugs?
Do not enter CNS-->no anesthesia
Depolarizing: fasciculations
Nondepolarizing: release of histamine-->vasodilation, increased secretions
What is dantroline?
Myotropic spasmolytic
Interferes w/Ca2+ release from sarcoplasmic reticulum
What are centrally acting spasmolytics?
Baclofen
Diazepam
How does baclofen work?
GABAb agonist
What is tizanidine?
Centrally acting spasmolytic
How do local anesthetics work?
Reversibly block conduction along axons by blocking Na+ channels
What are the local anesthetics?
-caines=esters
i before -caines=amides
How are esters metabolized?
Plasma ChE
How are amides metabolized?
Liver
What are S/E of local anesthetics?
CNS effects-->agitation, confusion
Cardiovascular depression
What are the anticholinergic dilating agents?
Atropine
Scopolamine
Homatropine
Cyclopentolate
Tropicamide
What is an advantage of anticholinergics over adrenergic agonists as dilating agents?
Anticholinergics dilate w/cycloplegia.
Cycloplegia paralyzes the ciliary muscle
How do beta blockers treat glaucoma?
Decrease aqueous production.
How do alpha-2 agonists treat glaucoma?
Decrease aqueous production & increase aqueous outflow
How do prostaglandins treat glaucoma?
Increase uveoscleral outflow.
How do carbonic anhydrase inhibitors treat glaucoma?
Decrease aqueous production.
What is an S/E of amiodarone?
Corneal deposits
What is an S/E of placquenil?
Macular toxicity
What is an ocular S/E of topiramate?
Bilateral ACG
What are ocular S/E of topical steroids?
Increased IOP
Cataract
Increased susceptibility to infection
Activation of HSV
Corneal perforation
What are cholinergics used for in the eye?
OAG-rare
ACG
Miosis
Myesthenia