General Signs Flashcards
(148 cards)
1
Q
Down Beat Nystagmus (11)
A
Chiari MS Wernicke Brainstem Encephalitis Cerebellar degenration and Obsoclonus Hypomagnesemia Basilar Invagination Lithium Syringobulbia Paraneoplastic GAD
2
Q
Bilateral Facialis (10)
A
GBS Lyme HIV Menigeal Carcinomatosis Sarcoidosis Moebius Melkersson Rosenthal Kennedy Fascioscapulohumeral Amyloidosis
3
Q
Vertical Gaze Palsy
A
Midbrain Infarction Parinaud Pinealoma Hydrocephalus PSP PD CBD Whipple Niemann Pick C Gaucher Tay Sachs bilateral INO
4
Q
Painful Ophthalmoplegia (19)
A
Tolossa Hunt Orbital Pseudotumor Thyroid Ophthalmoplegia is not painful Cavernous Sinus Syndrome Carotid Aneurysma PCA ANeurysma Cavernous SInus Thrombosis Carotid Cavernous Fistula Diabetes Tempo Arteritis Neuplasm Metastasis Apoplex Pituitary Meningioma Sarcoidodi Sinusitis Mucocele VZV Muomycosis
5
Q
DYT 1
A
Torsin A
Oppenheim, Jewish, Action Dystonia
low pnetrance
6
Q
X linked Dystonia Parkinsonism
A
DYT 3 LUBAG Filippinos
7
Q
Dopa Responsive Dystonia
A
DYT 5 girls more than boys SEGAWA Chromosome 14 worsening during the day action leg dystonia progressive Tetrahydrobiopten defect Dopa Sensitive No Dyskinesia later on OCD
8
Q
Paroxysmal Dyskinesia
Kinesogenic
A
dystonia chorea attacks male 80% sound or movement as trigger 1 min up to 100 per day DYT 10 Chr 16 Antiepileptic Tretament CBZ PYT Autosomal Dominant
9
Q
Paroxysmal Dyskinesia
Non-Kinesogenic
A
infancy, longer attacks, less often, 10 min to hours, Chr 2
coffee etchanol tiredness as triger
Azetazolamide, Clonazepam
10
Q
Paraneoplastic cerebellar degeneration (antibodies?)
A
Anti Yo Ri Tr CV2 Ma VGCC
11
Q
paraneoplastic cerebellar degeneration (decription)
A
rapid onset cerebellar syndrome subset with LEMS small cell lung, breast, ovary, hodgkin #1 anti Yo (breast gynaecology) anti Ri: truncal, opsoclonus (breast) --> can lead to dementia SCLC often anti Hu and later paraneoplastic encephalomyelitis Hodgkin: anti Tr
12
Q
Limbic encephalitis
A
Men under 45> possible anti Ma and testis tumor
13
Q
new variant CJD: whats different to sporadic CJD?
A
1) patients are younger (27y)
2) psychiatric and sensory symptoms as first signs
3) no typical EEG changes
4) longer duration of disease
14
Q
Oligodendroglioma mutations
A
if 1p and 19q = good chemotherapy response
15
Q
GBM
A
older patients EGFR upregulated younger p53 mutated more men than women extracranial metastasis possible Temozolamide/ Temodal and Radiation
16
Q
Meningioma
A
women more then men NF2: 22q gene mutations Estrogen receptors psammoma calcification
17
Q
Metastasis to brain
A
Lung Breast Melanoma Colon Kidney Testicle
18
Q
Bloody metastasis:
A
Melanoma
Chorionepitheliioma
Kidney
Lung
Thyroid
19
Q
Medulloblastoma
A
children post fossa Rossette Formation boys more than girls Chromosome 17 loss N-Myc bad prgognosis 72% JC Virus
20
Q
Neuroblastoma
A
adrenal Gland
number one solid tumor in children
Polymyoclonus Opsoclonus
21
Q
Choroid Plexus Papilloma
A
SV40 virus?
22
Q
Hippel Lindau
A
AD VHL Gene
Hemangioblastoma of Cerebellum
also Hemangioma of spinal cord and retina
renal pancreatic cysts
23
Q
Pineal Tumor
A
CSF follow up level of Melatonin after surgery
24
Q
chemotherapy sensitive brain tumor:
A
anaplastic oligodendroglioma
1p/19q very sensitive
25
Radiation injury
1) acute: seizure, worsening of tumor symptoms, ICP
possibly brain edema but not visible on MRI!!
2) early delayed: focal tumor symptoms, Tumor enlargement: pseudo-growth
3) late delayed: coagulation necrosis, white matter, softening, liquification,
symptoms of delayed: tumor progressin like or DEMENTIA
migraine like syndrome: with aphasia, hemiparesis hemianopia CSF protein elevated,
26
Anterior Cavernous SInus Thrombosis
```
chemosis and proptosis
III
IV
VI
and V1
```
27
Posterior Cavernous Sinus Thrombosis
```
spread to inferior petrosal sinus:
VI
IX
X
XI
no proptosis
```
additional involvement of superior petrosal sinuns: V nerve palsy!
28
Sturge Weber
```
port wine nevus
ipsilateral meningeal angioma
MR
seizures
hamiparesis
glaucoma
hemiatrophy
```
29
Optic neuritis first sign of MS in ...%?
First sign in 25%
30
Progression to MS if ON in 5 y? females, males
75% females
| 34% males
31
ON and no MRI findings, how much MS?
22%
32
MBP in MS?
elevated in CSF
33
Weston Hurst
acute hemorrhagic encephalomyelitis
| hyperacute ADEM
34
Antibodies in Interferones?
Betaseron 34% -every 2nd day IGN beta1b
Rebif 24% - 3*/week, beta 1a
Avonex 2-5% - 1/week, beta 1a
35
Kernohan Woltman Phenomenon
Babinski ipsilateral to lesion due to uncal herniation with pressure on Brain Stem
36
ICH acute signs
vomiting
hypertension
bradycardia
37
pontine damage (hemrrhage) how are the pupils?
extreme miosis
38
retinal hemorrhages due to ICP?
Terson Syndrome (in SAH)
39
clinical signs in MOYMOYA
1) sudden weakness, headache seizures
2) prolonged TIA
3) TIA induced by hyperventilation or hyperthermia
4) ICH
5) EEG rebuildup: after hyperventilation 5 min slow waves
40
ICH locations
1) Putamen (50%)
2) lobar white matter frontal, temporal, pareital
3) Thalamus
4) cerebellar hemisphere
5) Pons
death in 30% in 30 days
41
Medication in ICH for blood pressure
STICH Trial:
don't use calcium antagonists, they increase ICP
Use Betablockers or ACE
42
SAH score
Hunt and Hess
```
I asymptomatic (slight headache stiff neck)
II no focal signs, headache, neckstiff, --> SX
```
III drowsiness, confused mild focal
IV stupor coma decerebration
V Deep Coma
43
Rupturing of Aneurysma
In 5 years rupturing:
| 2.5 = >10%
44
cavernous malformation
1% per year bleed
halo of small bleed around
50% brainstem
10% multiple
45
cavernous hemangioma on MRI
pocorn lesion
46
Loss of smell (syndromes 3; other diseases: 6)
1) Kallmann
2) Turner XO
3) Albinos
1) AD
2) PD
3) HD
4) Pick
5) Korsakow
6) MTLE
47
Dropped Head Camptocormia (9)
1) Polymyositis
2) inclusion body myositis
3) ALS
4) MG
5) Nemalin Rod Myopathy
6) local radiation
7) isolated neck extensor myopathy
8) PD
9) MSA
48
Respiratory Muscle involvement
```
ALS
MG
Nemailn Myopaty
Pompe
Mitochondrial
GBS
Truchinosis (Diaphragm)
```
49
Carotid Artery Stenosis - symptomatic
70-99% - CEA
70-99% - CEA > STENT
50-70 % - MAN: CEA > medical
50-70 % - women: medical > CEA
<50%: medical
50
Carotid Artery Stenosis - NON symptomatic
60-99% CEA in men only if Sx less than 3%
women: medical treatment always
51
Zidovudine myopathy vs
| HIV myopathy
zidovudine: RRF
| HIV myopathy: nemailne rod myopathy
52
Seizures in meningitis
Haemophilus influenzae
53
Blood in CSF in meningitis (5)
1) Anthrax meningitis
2) Hantavirus
3) Dengue Fever
4) Ebola
5) Amebic Meningoencephalitis
54
Recurrent Meningitis (5)
1) structural bacterial
2) EBV
3) Behcet
4) Mollaret HSV 1/2
5) Vogt Koyanagi Haradi: meningitis iridocyclitis depigmentation of hair
55
legionella meningitis CSF
normal
56
Cat scratch disease
```
Bartonella henselae
adenopathy
encephalopathy
high fever
seizures
vasculitis in AIDS patients
```
57
Brucellosis meningitis typical features:
1) ICP with papiledema
2) meningoencephalitis
3) lymphocytic pleocytosis aseptic
58
Whipple disease features (6)
1) slowly progressive memory loss
2) ataxia
3) seizures
4) supranuclear ophthalmoplegia vertical
5) myoclonus (palatal)
6) myorhithmia oculomastoidal
59
aseptic meningitis
photophobia
conjunctival redness
hepatitis
what could it be?
Weill disease
Leptospira
rat urine
60
mucormycosis
fungal
in DM
nasal paranasal sinuses
hemorrhgaic infarcts
61
genes in hereditary spastic paralegia HSP
1) spastin
| 2) Atlastin (before 10 y)
62
juvenile onset ALS
ALS2 gene
slow progressive
anarthria dysphagia
63
definition of PLS primary lateral sclerosis
ALS patient without any lower motor signs for 5 years
64
SMA subtypes
1) Wernig Hoffmann Type I 50% SMN-1 early death
2) II and III Kugelberg Welander
in form III cramps typical
65
Kennedy
```
CAG repeat
x linked
androgen receptor
Gynecomastia
Oligospermia
testicular atrophy
```
66
Spastic symptoms in other diseases
- ataxia
- leukodystrophy
1) SCA 1 bulbar and extrapyramidal
2) SCA 2 hypokinetic rigid slow saccades
3) SCA 3 Machado Joseph portugese pure spastic syndrome no ataxia but PD like ATAXIN 3
4) Friedreichs (GAA rpeat, AR)
5) Krabbe beta galaktosidase
6) MLD
7) ALD (x linked)
8) Bassen KOrnzweig
9) alpha tocopherol transferase deficiency
67
differentiate plexopathy:
| radiation vs tumor invasion
radiation:
from upper side
Myokimia
Painless
tumor invasion:
from lower side
Horner
painful
68
Causes for posterior cord syndromes: (6)
1) B2
2) Cu deficiency
3) Folate def
4) HIV vacuolar
5) HTLV
6) syphilis
69
B12 deficiency
1) cognitive, psychiatric
2) optic neuropaty
3) orthostatic hypotension
4) Axonal peripheral neuropathy
5) subacute combined degeneration (post and lat column)
Cobalamin low
Howell jolly bodies
(NO can mimmick!)
70
cu deficiency (casues 3; signs 4)
causes: ZN ingestion; bariatric Sx, Celiac disease
1) anemia neutropenia
2) sensory ataxia
3) pyramidal signs
4) axonal PN
71
Viral meningitis with low glucose (4)
1) Mumps
2) HSV-2
3) Lymphocytic choriomeningitis LCMV
4) VZV
72
negri bodies
rabies
73
acute anterior poliomyelitis (5)
1) Polio virus RNA
2) Coxackie virus
3) WNV
4) Japanese encephalitis
5) Echovirus 70 71 (hemorrhgaic conjunctivitis)
74
Rasmussen
CMV
| HSV1
75
HIV neuropathy treatment
Lamotrigine
76
tongue tremor (cause)
Mercury poisoning
and ataxia cerebellar syndrome
granular cell layer in cerebellum disappears
concentric constriction of visual field
77
Lewy Body Dementia pathology
Lewy Bodies in cortex: ubiquitin and synuclein
no HALO like in PD!!!
no tangles or plaques
78
Huntington disease facts
1) young onset patients inherited gene from FATHER
2) late onset patients from MOTHER
Chr. 4; CAG repeat, 11-34 normal
anticipation
caudate putamen atrophy
PET: caudate
small eurons in BG affected before bigger ones
```
memory spared
Blinking frequency increased
Tongue protrusions
slow eye movements slow pursuit
No volitional saccades possible
```
TX: Halidol NOT DOPA
79
chorein gene
neuroacanthocytosis 9q
80
Polyglycosan Body Disease
adult-onset AR
progressive neurogenic bladder
gait difficulties (i.e., spasticity and weakness) mixed upper and lower motor neuron
sensory loss predominantly in the distal lower extremities
cognitive difficulties (often executive dysfunction)
GBE1 glycogen brancher enzyme defect
81
HD childhood begin:
Westphal variant: rigidity PD like
82
PD genetic without Lewy Bodies
Park 2 parkin
Park 6 PINK1
Park 8 LRRK +/-
83
genetic PD
sleep benefit
Dopa sensitive
early onset
84
PArk 8 LRRK-2
no lewy bodies +/-
no tremor
jews
85
parkin 6
PARK 2
50% of all young onset PD
no dementia
no Lewy Bodies
86
von Economo
no tremor
depression
105 dopa resposnsive
87
vocal cord palsy and PD?
MSA
| also gray discoloration hands
88
Segawa
```
DYT 5
14q
hereditary dystonia
DOpa responsive
OCD
sleep benefit (like genetic PD)
first legs stiff falls
retrocollis
later PD
```
89
DRPLA
dentatorubraopallidolusian Atrophy
Chr. 12
CAG repeat
```
Japan
ataxia
choreoathetosis
dystonia
PD
myoclonus
epilepsy
dementia
```
90
Hemiplegic ALS
MILLS VARIANT
91
Dynactin
Bulbar ALS genetic variant DCTN
92
Retinitis Pigmentosa (6)
1) Refsum Disease
2) Friedreichs
3) Cockayne
4) Bassen KOrnzweig
5) PEO
6) Kearns Sayre KSS
93
SIADH causes (7)
1) trauma
2) meningitis
3) stroke
4) SAH
5) neuplasm
6) GBS
7) CBZ
94
Delirium Tremens symptoms
Start 48-96 hours after last drink (2-3 days)
5% Mortality
```
Confusion
Delusion
Hallucinations
Tremor
Aggitation
Insomnia
Autonomic Features
Epileptic Seizures
Tachykardia
```
95
Delirium Tremens Lab
Hypomagnesemia
Hypokalemia
PCo2 low
Ph Increased (Hyperventilation)
96
Moyamoya
Surgical treatment is an option
97
Vertebral artery dissection
no female preponderance (there is in Carotid Artery)
C1-2 most commmon
98
carotid artery dissection
women
late 30/40s
```
fibromuscular disease
ehlers danlos
marfan
osteogenesis imperfecta
Loeys Dietz syndrome (TGF beta R mutation)
alpha 1 antitrypsin
```
MRI string sign
99
Alzheimer Pathology
1) Neurofibrillary tangles: abnormaly phosphorylated tau; but also others: ubiquitin, cholinesterase, beta amyoid
pattern: layer II enthorhinal cortex, CA1, subicular hippocampus, amygdala, deep layers of neocortex III V VI
- --> correlates with degree and duration of dementia
2) Plaques: BetaA4, congo red; amyloid; periphery: microglial cells;
3) vascular amyloid deposits n the walls of vessels;
4) Granuluvacuolar Degenration: intraneuronal clusters of small vacuoles; basophillic granuels,
HIPPOCAMPUS CA1
nowhere else!
5) HIRANO BODIES: CA1 hippocampus rod like red structures: more common in the GUAM disease
6) SYnaptic loss
7) loss Ncl meynert and coeruleus
100
GUAM
Amyotrophic lateral sclerosis/parkinsonism-dementia complex (lytico-bodig)
```
severe cortical atrophy
neurofibrillary tangles
depopulation of substantia nigra
NO LEWY BODIES
NO AMYLOID PLAQUES
```
but yes, tangles neurofibrillary tangles yes
101
MS pathology
plaques: periventricular; subependymal veins line the ventricles;
spinal cord: where pial veins lie in white matter
optic nerve and chiasma (not optic tract)
102
Vertebral artery dissection
no female preponderance (there is in Carotid Artery)
C1-2 most commmon
103
carotid artery dissection
women
late 30/40s
```
fibromuscular disease
ehlers danlos
marfan
osteogenesis imperfecta
Loeys Dietz syndrome (TGF beta R mutation)
alpha 1 antitrypsin
```
MRI string sign
104
Alzheimer Pathology
1) Neurofibrillary tangles: abnormaly phosphorylated tau; but also others: ubiquitin, cholinesterase, beta amyoid
pattern: layer II enthorhinal cortex, CA1, subicular hippocampus, amygdala, deep layers of neocortex III V VI
- --> correlates with degree and duration of dementia
2) Plaques: BetaA4, congo red; amyloid; periphery: microglial cells;
3) vascular amyloid deposits n the walls of vessels;
4) Granuluvacuolar Degenration: intraneuronal clusters of small vacuoles; basophillic granuels,
HIPPOCAMPUS CA1
nowhere else!
5) HIRANO BODIES: CA1 hippocampus rod like red structures: more common in the GUAM disease
6) SYnaptic loss
105
GUAM
Amyotrophic lateral sclerosis/parkinsonism-dementia complex (lytico-bodig)
```
severe cortical atrophy
neurofibrillary tangles
depopulation of substantia nigra
NO LEWY BODIES
NO AMYLOID PLAQUES
```
but yes, tangles neurofibrillary tangles yes
106
MS pathology
plaques: periventricular; subependymal veins line the ventricles;
spinal cord: where pial veins lie in white matter
optic nerve and chiasma (not optic tract)
107
EEG smooth chandelier cells
IPSP in duperfisciel layers
108
Deep pyramidal cells EEG
EPSP superfiscial
109
Vertx sharp wave
onset of sleep, children
110
Lambda waves
during reading, saccades, positive large wave
111
BETS
beningn epileptiform transients of sleep
112
WEBINO
wall-eyed bilateral internuclear ophthalmoplegia (WEBINO)
113
Sulpiride
Modal
114
Essential Tremor Treatment options
1) Propranolol Beta blocker
2) Primidone, Gabapentin, Topiramate
3) Alcohol
4) Benzodiazepine
5) Botox splenius capitis muscles
115
TIA Differential Diagnosis
```
Seizures
Migraine
TGAs
Meningioma
GBM
Metastasic brain tumors
Subdural hematoma
```
116
NMO pathology
demyelination
necrotic and infiltrated with neutrophils and eosinophils
Complement and immunoglobulin deposits of types IgM and IgG
MS: upregulation of AQP4
NMO: Loss
117
Park 2
Parkin 6 :
| no dementia and no Lewy Bodies
118
MSA: nucleus without pathology?
Red Nucleus
119
Lathyrism
pyramidal pattern of motor weakness combined with greatly increased tone in the thigh extensors and adductors and in the gastrocnemius muscles so that the more severely affected walk on the balls of their feet with a lurching scissoring gait. Extensor plantar responses are uniformly present in such cases, and the knee and ankle tendon reflexes are exaggerated and often clonic.
neurotoxin ODAP
Grasspea
120
Entacapone Side effects
Diarrhea 5%
Decoloration of Urine
increases Area under the Curve AUC
121
Epinephrine effect:
1) Increases systolic pressure
2) beta receptors: vasodilatation
beta block: vasocostriction
122
Treatment without Biopsy in Brain tumor? which one?
Brainstem Glioma
123
MGMT
Malignant gliomas: have a repair Gene called MGMT
If MGMT is methylated, chemotherapy response much better
124
Entacapone Side effects
Diarrhea 5%
Decoloration of Urine
increases Area under the Curve AUC
125
Epinephrine effect:
1) Increases systolic pressure
2) beta receptors: vasodilatation
beta block: vasocostriction
126
Treatment without Biopsy in Brain tumor? which one?
Brainstem Glioma
127
MGMT
Malignant gliomas: have a repair Gene called MGMT
If MGMT is methylated, chemotherapy response much better
128
VPA
Na, GABA
129
Keppra
SV2A Block
130
LTG
Na
131
Topiramate
Na, GABAa, AMPA, KAINATE, Calcium
132
Felbamate
Hepatotoxic
| NMDA R antagonist
133
Antiepileptic leading to Leukopenia:
1) CBZ
2) PHT
3) VPA
134
Depression and EPilepsy. Which treatment?:
VPA
CBZ
don't give KEPPRA, TOPAMAX, VGB, Benzos
135
Epilepsy and Porphyria: which drugs are ok?
Keppra
| LTG
136
Antiepileptics which induce liver enzymes:
PHT
CBZ
Barbiturate
NOT VPA
Topiramate Enzyme nducer if above 200mg
137
Pro Osteoporotic
CBZ
Phenytoin
Barbiturates
VPA
all reduce testosterone and increase cholesterole
138
VPA
Na, GABA
139
Keppra
SV2A Block
140
LTG
Na
141
Topiramate
Na, GABAa, AMPA, KAINATE, Calcium
142
Felbamate
Hepatotoxic
| NMDA R antagonist
143
Antiepileptic leading to Leukopenia:
1) CBZ
2) PHT
3) VPA
144
Depression and EPilepsy. Which treatment?:
VPA
CBZ
don't give KEPPRA, TOPAMAX, VGB, Benzos
145
Epilepsy and Porphyria: which drugs are ok?
Keppra
| LTG
146
Antiepileptics which induce liver enzymes:
PHT
CBZ
Barbiturate
NOT VPA
Topiramate Enzyme nducer if above 200mg
147
Pro Osteoporotic
CBZ
Phenytoin
Barbiturates
VPA
all reduce testosterone and increase cholesterole
148
Cyp2c19
Decreased clopidogrel metabolism
