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119 Cards in this Set
- Front
- Back
- 3rd side (hint)
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hypoxia
sensitive sites |
cortex - layers 3-5-6
basal ganglia purkinje cells |
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histology
hepatotoxic encephalopathy i.e. Wilson dis. |
alzheimer type II Astrocytes
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brain hemorrhage
frequent place |
1. putamen/int. capsula
2. lobar (parietal/frontal) 3. thalamic 4. cerebellar hemisphere 5. pons |
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Foster-Kennedy Synd
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optic atrophy ipsi
pappilledema contra anosmia |
meningioma orbital, frontal
basal cranium tumor |
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Amynoglycosides
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for Gram (-)
DO NOT cross BBB Ototoxicity |
Streptomycin Neomycin
Amikacin Tobramycin Gentamicin Kanamycin |
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DD Asterexix
=negative myoclonus |
metabolic encephalopathy hepatic / renal
medial frontal cortex wilson PHT intox - CBZ, VAL, PBL |
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Neuroblastoma
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child tumor
extra dural, ++extracranial biopsy: "rosettes" adrenal cells, neuroendocrine metastases++ opsoclonus / myoclonus ~PCD |
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MG & AB
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aminoglycosides (presyn)
quinolone (pre&postsyn) colistine polymixin B D-penicillaine macrolides (erythro/azithromycin) tetracycline (doxyline) |
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AED
~idiosyncratic skin erruption |
aromatic cycle
PHT CBZ LMT PHB/Primidone |
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Fabry dis
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alpha-glactosidase A def
x-linked angiokeratoma eye/skin CRF cardiac complication painfull axonal neuropathy - burning extremities thrombotic events! |
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- MMN -
Multi-focal Motor Neuropathy |
yg adult 20-40
~ALS BUT no UMN motor dem neuropathy +/-asym distal- finger extension/atrophy EMG= CONDUCTION BLOCKS anti-GM1 -->80% CPK moerately elevated treatment= IVIG, cyclophosphamid (!!NO steroid) |
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Kearn-Sayre Synd
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- mitochondrial dis
- yg adult, <20 - ophtalmoparesis bilat, !No diplopia ptosis bilat proximal weakness - Ataxia, dementia, E+ - deafness, night blindness< retinitis pigmentosum - AV block - Prot CSF increased - endocrine - short stature, hypogonadism |
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MLD
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- Arylsulfatase A def
- AR, Chr 22 - Cherry red spot - WM lesion (psy/dem) + dem neuropathy |
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ALD
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- X-linked
- increased VLCFA serum - Addison: hypo NA/Gluc/art tension hyper K, Ca skin pigmentation - polyneuropathy dem&axon |
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brain biopsy
Rosenthal fibers |
- Pilocytic Astrocytoma
- Alexander dis (child leukodyst) - (fucosidosis) |
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cytotoxic drug
--> AXONAL SENSORI>motor polyneuropathy |
- Taxanes (taxol)
- Cisplatin.Carboplatine - Vincristin |
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no BBB
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- Periacqueducal Gray (CRZ)
- Area Posterma (medulla) - Pineal - neurohypophyse (post) - choroid plexus - median eminens - (laminar terminalis-vascular organ, subfornical, subcommisural organ |
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Anatomy
cerebellar peduncles |
Superior:
- mostly efferent to midbrain (red nucleus) and pons - afferent anterior spinocerebellar tract - leg propioception Middle: descending cortico-ponto-cerebellar tracts Inferior: afferent - olivocerebellar,vestibuloscerebellar tract - posterior spinocerebellar tract (hand propioception) |
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eye movement and OKN
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smooth pursuit < IPSI parieto/temp/occipital
saccadic pursuit < CONTRA frontal ! OKN= cortical integrity slow phase= smooth, rapis phase= saccade |
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respiration patterns
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cheyne-stokes --> diencephalon
Hyperventilation, kussmaul (Neurogenis) --> midbrain Apneustic --> pons Ataxic/biot --> medulla |
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LHON
Leber hereditary optic neuropathy |
- mitoch. dis. (3 mutations)
- 85% male 20-30 - Bilat acute/subacute/chron - retibal ggl cell degen --> central scotoma - ! flurosceine normal 50% |
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ALS
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- 90% sporadic male>fem
- 10% familial, AD Chr 21 SOD mutations - cramps ~physical stress - classic --> begin Asym distal weakness (drop wirst/foot) - atypic --> "man in the barrel" - biopsy: fiber grouping |
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taupathies
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- AD
- FTD +- PD (ch17) +- ALS (guan cplx) - Pick - CBD - PSP |
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synucleinopathies
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- PD
- DLBD - MSA |
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C-MAP in repetitive nerve stimulation
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NL --> MG
Decrement 2-5 hz -> MG & LEMS Increment 20-50 hrz --> LEMS/botulinum/organophosphate hypoCa, HyperMg |
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Muscle biopsy
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type I = red, O2, marathon
--> myotonic dystrophy, ~myopathy type II = sprint, glycolyse, lactate --> steroid myopathy: Normal CPK+EMG |
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LEMS
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- 60% paraneoplasic (50% SCLC)
- Ca-channels Ag - prox weakness limbs !! No ptosis or diplopia - dysautonomy - hyporeflexy - low amplitude SFEMG - treat: D-aminopyridin (DAP) |
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Migraine prophylactic treatment
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First line High efficacy Beta-blockers
Tricyclic antidepressants VAL, TPX Low efficacy Verapamil NSAIDs SSRIs Second line High efficacy Methysergide Flunarizine MAOIs Unproven efficacy Gabapentin/Lamotrigine |
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Cluster Headaches prophylactic treatment
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- Calcium Channel Blockers
- lithium - VAL, TPX - Methysergide |
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BERA
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I - CN8- auditory n.
II - cochlear nuclei in medulla/pons III - SUperior Olive in pons IV - Lateral Lemniscus V - Inferior colliculus in midbrain MGN in thalamus Heshel Gyrus |
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PME
progressive myoclonus epilepsy - cognitive decline - ataxia (cerebelaar signs) - E+ (absence/GTCS) |
- Unverlight-Landberg (~Baltic). mut cystatin B
- Lafora Body - Lipofuscinosis= neuroanl Ceroid ( adult= Kuffs dis, juvenil= Batten dis) - Syalidosis with cherry red spot - MERF - DRPLA - Gaucher |
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Stiff Person Synd
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- chronic/progressive/fluctuating rigidity, spasms, especially in axial
- anti-GAD, autoimmune - anti-amphyphisin --> breast CA/paraneo - anti-Gephyrin --> CA/ paraneo |
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DD Acanthocytes
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- neuroacanthocytes
- A-beta lipoproteinemia (bassen-Kornsweig) - Mc-Leod dis (+muopathy, cardiac, CPK elevated) - Halloverden-Satz (AD, pank2 mut, 'eye of the tiger' MRI) (- hepatic dis.) |
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Lyme infection stages
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1- Weeks - erythema chronico-migrans, hepatosplenomegaly
2- Months - 'disseminated': aseptic menigitis, radiculitis, (multi)cranial nerve palsy 3- White Matter Encephalitis |
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caloric reflex
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~brainstem/vestibular
Unilat - cold--> ipsi dev, contra Ny - warm --> contra dev, ipsi Ny Bilat - cold --> down dev - warm --> up dev |
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Apo E4 ~
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- +80male= 100% AD
- head trauma - MS - CJD - early onset ALS-bulbar - Cerebral Amyloid Angiopathy |
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Parinaud
= Dorsal Midbrain Sd (tectum) <pineal body Tu i.e.pinealoma !Child+parinaud+alpha-FP in CSF => Germinoma |
- Up(>down) gaze palsy 'sundown'. Skew deviation
- near light dissociation - retraction-convergence Ny |
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drugs ~ E+
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- Li++
- AB, Imipenem (carbapenem, beta-lactam) - Aminophyllin - Lidocain - Tricyclic (elatrol/amitriptilin, anafranil/clomipramin) >> SSRI/SNRI - amphet Methylphenidate/ritalin - antipsych++ Clozapin/leponex - Bupoprion (zyban) ++ |
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hypothalmus centers
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ANT = PS preoptic nucl, reduce Temp, vasodilat
POST = OS, increase Temp, vasoconstrict LAT = 'hungry' feeding center MED = 'thirst','anorexia' satiety center |
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Tics DD
treatment - neuroleptic (risperidon) - (BTX, tetrabenzine, beta-Block) |
- Tourette (treat:Clonidin alpha-Ag, SSRI for OCD,BZD, TriCyc)
- neuroacanthocytes, - Hallervarden-Spatz - Wilson - HD - CJD - Duchenne muscular dyst. - Autism, Down, fragileX |
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Complex Regional Pain Sd
or RSD- reflex sympathetic dystrophy, causalgy |
- No anatomic distribution, bandeau localized
- spontaneous/post trauma - Pain+++, dystrophy +- osteoprosis - locla dysautonomic changes (swelling, red,...) |
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rapid correction hypoNa
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- central pontine myelinolyse
- extrapontine myelinolyse in Ctx+ basal ggl |
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'Man in the barrel' DD
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- Central Canal Sd
- watrershed stroke MCA X ACA - Limb girdle - atypical ALS |
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ICP DD
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- PTC
- toxic: vitA, tetracyclen(doxy), Lead intox - idiosyncrasic: amiodarone, quinolone, oestrogen, phenothiazine - metabolic: steroid, hypo/hyperthyroid, hypoparathyr. - vascular: SVT, - SOL - meningitis - increased prot in CSF: GBS, SLE, spinal Tu (oligo) |
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young HD = Whestphal variant
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- yg/very yg juvenile onset
- rigid-hypokinetic syndrome and hyperkinetic - ! E+ - paternal transmission |
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Brain vasculitis
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- Aspergillosis
- TB - Syphillis - HZV - HIV |
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HIV lesions
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- primary --> vacuolar myelopathy
- HZV myelitis+++, CMV - classic radiculitis - encephalitis - AIDS dementia complex, PML |
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myelitis/myelopathy DD
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- HSV
- CMV - Cervical disk syndromes - Compressive myelopathy (eg, tumor abscess, herniated discs, arteriovenous malformation) - Human T-cell lymphotropic virus type 1 (HTLV-1) associated myelopathy/tropical spastic paraparesis (can co-exist with HIV infection) - Spinal epidural abscess - HIV-1 associated conditions (Kaposi sarcoma, lymphoma, toxoplasmosis in the spinal canal - Mycobacterium tuberculosis) |
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Cerebelum
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- Vermis --> trunk equilib.
- Floculo-Nodulus --> equilib/Ny/Vertigo - Ant Lobe --> walk, leg hypotonus - Post Lobe --> dysmetry |
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SE Thalium
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- Alopecia
- painfull neuropathy |
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Dyphteria
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- exotoxine of corynebacterium diphteriae
- acute phase (5-12d) --> palatin dysfunction, dysarthria, dysphagia (CN9/10 decreased Accomodation <ciliary body paralysis, !!light refl ok ! myocarditis - 5-8 wks --> Dem motor polyneuropathy - model of focal demyelination without inflamation |
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dermatomyoitis
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- anti-Jo1 (polymyositis ++), ANA + associated
- adult --> !!paraneoplastic - muscle biopsy, perifascicular atrophy, B/T cell perivascular infiltrat - affect esophagus, lungs (and heart) - skin, heliotrope rash, photsensible,extensor surfaces, and periungual and cuticular changes, malar erythema - causes: inflam, autoimmun, infectious (virus, borrel, toxoplasma), drug-induced (penicillamine, statin drugs,..) |
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Lysteria
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- contaminated food, GI Si
- Gram (+) --> treat Ampicillin - meningo/encephalitis Si - elderly, immunocompromised, chronic dis, pregnant women, newborn. - in CSF, can stain for diphteria! - 10% brain abcess in brainstem, thalamus |
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cryptococcus
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- immunodep, HIV
- reservoir=pigeons - subacute/chronic headaches, Dementia |
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meningitis ~ age
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- newborn --> strepoB, E.Coli, Lysteria
- Child --> N.meningitidis, S.penumoniae, <5y H.influenzaB - adult --> (N.meningitidis, S.penumoniae) =80%, >50-55 Lysteria |
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SSPE
=subacute sclerosing panencephalopathy |
~ measle encephalopathy, post infection ~yrs
-stade I, mood/personnality changes -stade II, myoclonus+mental deterioration - CSF Nl, !! but IgG increased for rubeola - EEG, widespread cortical dysfunction, periodic complex high burst - MRI,bilat white matter changes |
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MS treatments
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- Interferon beta:
- 30%decr relapse-remiting,decre plques in MRI - neutralizing Ab--> rebif++(SC3/wk), betaferon (SC1/2d), (avonexIM) - Natalzumab Tisabri, IV/mth. ! PML - Glatiramer Acetate Copaxone |
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internuclear ophtalmoparesis
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INO = MLF--> Ipsi: No ADduction, Contra: Ny in ABduction.
One&half = PPRF+MLF --> Ipsi: No AB/ADduction, Contra No ADduction (ABduction ok) |
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fascioscapulohumeral muscular dystrophy
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- AD ch4
- slow prog , ~ arrest - +- asym weakness atrophy face shoulder - !! Deltoid OK till end - ! popeye arm - retinal damage |
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Inclusion Body Myositis
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- spor vs heredit
- most frequen inlfam myop >50 - male>women - proximal and distal, asym 30% - wrist/finger flexors++, knee extensors (quad weakness++), ankle dorsiflexors - biposy, rimmed vacuoles, Tcell infiltrat, fiber necrosis - No treatment |
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Quadriceps weakness/atrophy DD
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- Muscular Dystrophy
Becker, limb-girdle 2B - Inflam myopathy IBM, polymyositis, focal myositis - Nerve disorder femoral neuropathy, diabetic amyotrophy, L3-4 radiculopahty - Lumbosacral plexopathy, neoplastic++ |
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primary demyelination neuropathy
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- Dispersion (>15% duration prox vs distal response)
- 20% decreased motor Velocity - 20-25% decrease motor dist latency - Conduction block= 50% decreased motor Amplitude prox vs dist (!acquired) - F-wave 20% increased latency (31msec Arm/58msec Leg) |
GBS/CIDP
focal--> polyneuropathy/polyradiculopathy diffuce--> CMT1, heredit dem, lukodystrophy, connective tissue disorder entrapment sd |
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primary axonal neuropathy
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- 20% decrease in amplitude
- needle EMG denervation (acute): spont activity: posit sharp waves, fibril, complex repetitive disch reinnervation (chron, 3-4 month) big polyphasic motor unit, decreased recruitment |
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needle EMG topography
ARM |
C5- delt,bic,supra/infraspinatus,brachioradialis (!radial)
C6- pronator teres, FlexCarpiRad,ExtDigit C7- triceps (only!), ExtCarpiRad C8- InterOs(1srtdorsal), ABdDiMin,ExtIndicis (!ulnar) T1- thenar group, ABdPolBrev |
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needle EMG topography
LEG |
L2/3- IlioPsoas
L4- Quad, Adductors L5- TibAnt(peron), TibPost(tibial), GlutMed,(paraspinal) S1- GlutMax, Hamstring, Gastrocnemius |
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polyneuropathy
Metabolic & Systemic |
Diabetes mellitus
Alcoholism Uremia Thiamine deficiency Vitamin B12 deficiency Multiple myeloma Amyloidosis Cryoglobulinemia Hypothyroidism or hyperthyroidism Sarcoidosis Paraneoplastic syndrome, eg, lung cancer Neuropathy associated with critical illness |
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polyneuropathy
Infectious & Inflammatory |
Hansen’s disease (leprosy)
HIV/AIDS Lyme disease Diphtheritic neuropathy Polyarteritis nodosa Rheumatoid arthritis Temporal (giant cell) arteritis Toxic |
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polyneuropathy
toxic drugs |
Metals: lead, mercury, arsenic, thallium
Drugs: isoniazid, phenytoin, pyridoxine, nitrofurantoin, vincristine Industrial agents or pesticides: acrylamide, organophosphates, hexacarbon solvents, methyl bromide, carbon disulfide |
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polyneuropathy
Hereditary |
Charcot-Marie-Tooth disease
Porphyria Friedrich’s ataxia Dejerine-Sottas disease (HMSN type III) Refsum disease (HMSN type IV) |
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polyneuropathy
Mostly motor |
Guillain-Barré syndrome
Chronic inflammatory demyelinating polyneuropathy (CIDP) Porphyria Diphtheritic neuropathy Toxic neuropathy Poliomyelitis Periodic paralysis syndrome |
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~ICU patient
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Critical care Neuopathy
axonal motor !CSF Nl prot good prognostic Acute Quadriplegic Myopathy increased++ CPK +- use myorelaxant, steroid EMG +- myopathic biopsy: myosin atrophy, filament loss (!!steroid myopathy-->fiber II, with Normal CPK/EMG) |
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superficla reflex
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- corneal CN5-7
- gag CN9-10 - abdominal +-T10 - cremaster L1-2-(T12) - anal S2-5 - babinski S1-2<sensor, L4-5<tibial |
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Penicillamine
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- wilson treatment, also mercure poisoning
- Ab --> MG - drug-induced SLE - SE: hair loss, tinnitus, taste change |
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Lithium
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- vertical down beat Ny
- E+ - extraPyr Si !!- hyperCa !!- leucocytosis - hypothyr - teratogenic --> epstein cardiac/valvular malformation intox--> N+,V+,arrythmia. treat< throphyllin,diamox |
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Valproate SE
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- alopecia
- thrombocytopenia - OMPK - increased weight - teratogenic - pancreatitis - tremor/parkinsonism |
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Myoclonus DD
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- physiol (seep)
- essential benign - E+ (JME), !PME - drugs: PHT,CBZ,DOPA,serotonin Sd - metabolic: hepat,uremic,non-ketog hyerosmo, hypoglycemic - post-anoxic=Lance-Adams Sd; ~5-HT, tryptophan sensitive - degenerative: CJD,AD,CBD |
treat
VAL, BZD, 5-HT antg (tryptophan),... |
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Animal Toxines
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- Puffer fish - tetradotoxin | >block Na channels
- Shellfish - brevetoxin/Saxitoxin | cf perioral paresthesis - Snails - Cignatoxin | - Cobra - bungarotoxin >cf MG, block post-syn receptor Ach - Bee/Wasps - Hymenoptera venins >immunolog reaction - Snake - notexin > block presyn Ach release, necrosis - Black-widow - alpha-latrotoxin >presyn Ach release, tetany |
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Retinoblastoma
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- 30% in1yr affect second eye
- association with pineoblastoma - osteogenic sarcoma - ch13, ~ P53 on ch17 |
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E+ ~ age
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perinatal --> trauma
child --> syndromes 20-35 --> idiopathic 35-60 --> trauma >60 --> sympto: SOL, stroke |
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stroke ethiology
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20-25% cardio-emboli
25% large vessel 20% lacunar 20% cryptogenic 5% rare causes: dissect,vasculitis,fybrodysp |
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spastic paraparesis
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- hereditary Spas Parap, AR spastin mutation
- (lathyrism sensori-motor) - spinal stroke, AVM - Tu (sagital) - HTLV1 >tropical SP. ~CLL - Primary Lateral Sclerosis (PLS) - VitB12 def +post collumn - Copper def - Gerstmann–Sträussler–Scheinker synd (GSS) - nutritional spinal spastic & ataxia |
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winging of the scapula
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- Serratus Ant < long thoracis C5-6(7)
- Rhomboid < dorsal scapular C4-5 - Trapezius <CN11 Accessory |
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eye mouvements
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MR - adduction
LR - abduction SR - primarily elevation, secondarily intorsion, tertiarily aDduction IR - primarily depression, secondarily extorsion, tertiarily aDduction SO - primarily intorsion, secondarily depression, tertiarily aBduction IO - primarily extorsion, secondarily elevation, tertiarily aBduction |
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Von Hippel-Linau Sd
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- AD ch3
- !! hemamgioblastoma in cereb/spinal - retinal angiomatosis - renal cell CA, pheochromocytoma, angioma - pancreas cyst |
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acute dystonic reaction
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- yg male+++ 5-45yr
- orofacial, b uccal, lingual, torticolli - <drugs: antipsych ~2%, - increased risk with antidep, alcohol, cocain - in minute---days - treat: anticholin, BZD |
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Sturge-Weber
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= encephalotrigeminal angiomatosis
- sproadic embryonal - port-wine in face - E+ - glaucoma - mental ret - ! Ipsi leptomeningeal angioma |
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Polycystic Kidney Dis
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- PKD1 in ch16 ~85%, AD (also ar)
- PKD2 in ch4 ~10% anterior circulation anevrysme |
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eryhtema nodusum
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< autoimmune reaction
-! Behcet -! Sarcoidosis - Leprae - infection, IBD,... |
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Moebius Synd
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=congenital Bilat Facial Palsy
- CN VI & VII palsy - Limb abnormalities:clubbed feet, missing fingers/toes - Chest-wall abnormalitie |
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Cogan Synd
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= autoimmune dis of inner ear/eye
- child till 30yr - after resp infection(Chlamydia) ? - hearing decres/loss, tinnitis, vertigo - keratitis, conjunctivitis, irritis - systemic vsculitis: CRP increased, VS - treat: steroids, immunosupp |
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PRES etiology
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- tacrolimus
- cylcosporin - bevacizumab (avastin=angiogenesis inhibitor) - transpl/immunosupp - malignant hypertension - eclampsia |
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Refsum dis
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- accumulation of phytanic acid <peroxisomal enzyme def
- Peripheral polyneuropathy (motor>sensor) - cerebellar ataxia, anosmia - retinitis pigmentosa (concentric vis defect, night blindness), cataract - ichthyosis |
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reperfusion synd
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- 1wk-1mth after stroke
- Ipsi headaches - contra neurodeficits - E+ |
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Topamax SE
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- paresthesis (carbon anhydrase inh)
- weight loss, anorexia - cognitive disorder- impaired thinking/ word finding, memory - nephrolithiasis - oligohydrosis/ hyperthermia - acute glaucoma >acute myopia |
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Primary Lateral Sclerosis PLS
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- only UMN!
- >45-50 yr - slow prog: begin leg, then arm/face DD spastic paraparesis |
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hereditary lipid dis with neuropathy
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Dem --> MLD, Refsum
Dem+Axon --> Krabbe, ALD Axon --> Fabry, Tangier, Abetalipoproteinemia |
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Neurosyphilis
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- Asymptomatic neurosyphilis: CSF + for FTA/TPHA
- Acute aseptic meningitis (~1yr of infection) +- CN VII, VIII, VI. +- Optic atrophy, Argyll Robertson pupil: accom OK, No light relflex +- myelitis - Meningovascular=endarteritis with perivascular inflammation (7yr) stroke in the yg MCA++basilar+, aortic aNl, anevrysm - Tabes Dorsalis (20-30yr) = slow prog degen posterior columns (dem in cuneus/gracilis) and posterior roots (inflammation+fibrosis). ~propio, vibr, areflexia, ataxia, paroxismal pain ophtalmic - General Paresis, psych (delusion grandiosis), dem (behavioral ~FTD), mood changes, E+ |
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Nonsyphilitic causes of Argyll Robertson pupil
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diabetes mellitus
multiple sclerosis Wernicke encephalopathy Lyme disease sarcoidosis herpes zoster tumor periaqueductal gray hemorrhage periaqueductal gray |
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Thyroide
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HYPO
~MG myopathy/myalgia, neuropathy, slow relax cramps +myxoedema HYPER chronic thryrotoxic myopathy (CPK nL, extraoccular myopathy 'graves' periodic paralysis ~hypoK fibrilation/fasciculation |
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Lead intox
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- radial palsy, ! foot/wrist-drop (adult)
- encephalopathy (child w/ psych changes, cog decline) - axon motor neuropathy - microcytic anemia - hyperpigmentation - GI Si, blue gum |
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Neurosyphilis
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- Asymptomatic neurosyphilis: CSF + for FTA/TPHA
- Acute aseptic meningitis (~1yr of infection) +- CN VII, VIII, VI. +- Optic atrophy, Argyll Robertson pupil: accom OK, No light relflex +- myelitis - Meningovascular=endarteritis with perivascular inflammation (7yr) stroke in the yg MCA++basilar+, aortic aNl, anevrysm - Tabes Dorsalis (20-30yr) = slow prog degen posterior columns (dem in cuneus/gracilis) and posterior roots (inflammation+fibrosis). ~propio, vibr, areflexia, ataxia, paroxismal pain ophtalmic - General Paresis, psych (delusion grandiosis), dem (behavioral ~FTD), mood changes, E+ |
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Nonsyphilitic causes of Argyll Robertson pupil
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- diabetes mellitus
- multiple sclerosis - Wernicke encephalopathy - Lyme disease - sarcoidosis - herpes zoster - tumor periaqueductal gray - hemorrhage periaqueductal gray |
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Chorea DD
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bbbb
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Thyroide
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HYPO
~MG myopathy/myalgia, neuropathy, slow relax cramps +myxoedema HYPER chronic thryrotoxic myopathy (CPK nL, extraoccular myopathy 'graves' periodic paralysis ~hypoK fibrilation/fasciculation |
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Lead intox
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- radial palsy (adult)
- encephalopathy (child w/ psych changes, cog decline) - axon motor neuropathy - microcytic anemia - hyperpigmentation - GI Si, blue gum |
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Alopecia
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- Arsenic intox
- thalium - Val - Li - penicillamine |
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Cherry red spot in macula DD
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Metabolic Storage Diseases:
Mucopolysaccharidosis Hurler's Tay-Sachs/Sandoff's (GM2) Farber's disease GM1 gangliosidosis Niemann Pick's disease Lysosomal Storage Diseases Congenital Developmental Diseases : Leber's Hereditary/ Familial: Hallervorden Spatz disease leukodystrophy, Krabbe's Degenerative: Metachromatic leukodystrophy Vascular: central retinal artery occlusion Drugs: Quinine toxicity Dapsone toxicity Poisoning: Carbon monoxide Methanol |
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retinitis pigmentosa
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Kearn-Sayre
Abetalipoproteinemia refsum syphillis, toxoplasmosis usher synd (-deafness+RP) SCA+++ FA Kuffs, Batten ceroid lipofuscinosis |
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myotropism parasites
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- Trichinosis in periorbital/tongue/diaphragm
- Cysticercosis in gastrocnemius - Toxoplasmosis (- HIV) |
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cataract DD
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- CTX
- MD - MArfan - Homocysteinuria |
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Whipple
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< troheryma whipplei
- no speciGI, malabsorption, arthralgy, fever - Ataxia - Cognitive changes, Dementia - hypothalamus Si - Myoclonus, E+ (SP,CP,CTCS) - 50% Ophtalmoparesis w/ diplopia<supranuclear BOTH horiz&vert! - !! Occulomasticatory Myorythmia - D in jejunnum biopsy,!! PAS+in CSF - treat: Rocephin. TMP-SMX |
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Arsenic intox
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- encephalopathy
- sensorimotor polyneuropathy, areflexia - transverse white line in fingernails - D in hair/urine |
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livido reticularis
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< amantadin
- vasculitis, Hep C - Sneddon Synd= LR+APLA+strokes |
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Mollaret triangle
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~increased rubral tremor "palatine myoclonus"
- dentate nucleus - red nucleus - inferior olive |
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Botulism
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- exotoxin in presyn NMJ
- BTX-A inhibit release Ach vesicule in SNAP-25 - intox 12-36h till 4 d - gastro Si, constip++ - diplpia, ptosis, ophtalmoparesis CN6++ - ! No Accomodation, reduced light reflex - bulbar Si then face/neck then trunk/limbs w/ reduced reflex - DD MG, GBS |
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increased Jitter < SFEMG DD
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- MG
- LEMS - Botulism - ALS - Myopathy, polyneuropathy - hypoCa++, HyperMg++ |
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neurotoxic
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organophosphate,tricresylphophate --> inh AchEsterase.
acute 'SLUDGEM' delayed motor polyneuropathy< corticspinal motor neuronal death hyperthermia--> purkinje cells methylmercury-->tremors, gingivitis, and psych/behavior change, 'pink disease' + visual disturbance (eg, scotomata, visual field constriction), ataxia < granular cell , paresthesias (early signs), hearing loss, dysarthria, mental deterioration, muscle tremor, movement disorders < bsal gglia nn tox Manganese -->Parkinson-like syndrome, psych <basal gglia, |
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Triplets repeat dis
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CAG ---> HD,DRPLA,Kennedy,SCA
CGG ---> FRAXA/FRAXTAS GAA ---> FA CTG ---> MD,SCA8 GCG ---> OPMD |
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Friedrich Ataxia (FA)
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- AR ch9; GAA repeat
- No frataxin (in mb mitoch) - puberty++, 25% adult onset - prog gait/limb ataxia - !!No reflex, decre proprioception <-- n. dorsal of clarck in spinocerebellar - Py Si, Optic atrophy, decr hearing - pes-cavus, scoliosis, cardio --> death, DM |
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