Neurology Flashcards
1
Q
Delirium
A
an acute state of confusion, which may be characterized by
a reduced level of consciousness,
cognitive abnormalities,
perceptual disturbances,
emotional disturbances.
2
Q
Coma
A
sleeplike state in which the eyes are closed and the patient is unarousable even when vigorously stimulated
3
Q
Vegetative state
A
complete unawareness of self and surroundings but preserved sleep-wake cycles and at least partial preservation of hypothalamic and brainstem autonomic functions
4
Q
Causes of Coma
A
50% - cerebrovascular disease
20% - hypoxic injury
30% - toxic, metabolic, infectious
5
Q
3 important questions to evaluate coma
A
Does the patient have meningitis?
Are signs of a mass lesion present?
Is this a diffuse syndrome of exogenous or endogenous metabolic cause?
6
Q
Glasgow Coma Scale - interpretation
A
Brain injury is classified as:
Severe: < 9
Moderate: 9-12
Minor >= 13
Always state the individual score for each aspect of the scale
7
Q
Decorticate posturing
A
Flex elbows and wrists
Addudction of shoulders
Extension of legs
Less severe neuro injury
8
Q
Decerebrate posturing
A
Extension of elbows, wrists, legs
Adduction + internal rotation of shoulder
9
Q
What strongly suggests metabolic cause of coma?
A
Myoclonic jerks
Tremor and asterixis in awake pt
10
Q
Most impt brainstem reflexes
A
Pupilary light
Corneal
Conjugate eye mvmts
11
Q
Loss of pupilary light reflex
A
Brainstem herniation
If only 1 pupil size blown, suggests temporal lobe herniation and impingement on 3rd cranial nerves
Symmetric small = OD w/ opiates
Symmetric large = Cocaine, TCAs
Eyes deviate to one side = large cerebral lesion (look away from paralyzed side)
12
Q
Coma after cardiac arrest
A
No pupilary and corneal reflexes @24 hrs
No motor response @ 72 hrs
Little chance of meaningful recovery
13
Q
Coma w/ focal signs suggests…
A
Structural lesion
Stroke
Hemorrhage
Tumor
Abscess
14
Q
When do LP in unexplained coma?
A
Meningitis
SAH (but neuroimaging normal)
15
Q
When do to LP in headache?
A
concern for meningitis (fever and neck stiffness)
or encephalitis (focal neurologic signs, confusion, altered mental status)
if subarachnoid hemorrhage is suspected but imaging studies are normal
16
Q
Criteria for migraine diagnosis
A
POUND
If you have >=3 of the following:
Pulsatile quality (headache described as pounding or throbbing)
One-day’s duration (episode may last 4-72 hours if untreated)
Unilateral in location
N/V
Disabling intensity (altered usual daily activities during headache episode)
17
Q
Route of admin of drugs for severe N/V in migraine?
A
Intranasal
Parenteral
18
Q
Dementia
A
persistent impairment of intellectual function with compromise in at least three of the following spheres of mental activity:
- language,
- memory,
- visuospatial skills,
- emotion or personality,
- cognition (abstraction, calculation, judgment, executive function)
ONLY dx if tehre is functional impairment
19
Q
Dystonia
A
involuntary, sustained contraction of agonist/antagonist muscles, which often can lead to uncomfortable or even painful twisting, bizarre-looking postures
D blocker drugs can cause this
20
Q
What kind of movement disorder is asterixis?
A
Negative myoclonus
Sudden interruption of sustained muscle constrictions leading to loss of tone
21
Q
Parkinson diagnosis
A
based on three cardinal clinical features:
- bradykinesia,
- resting tremor,
- postural instability
22
Q
Dystonic reactions from D2 receptor blocker drugs most often affect…
A
Ocular muscles Face Jaw Tongue Neck Trunk
Limbs rarely affected
23
Q
Torticollis
A
Cervical dystonia
Abnormal postures of head, neck and shoulders
24
Q
1 cause encephalitis in US
A
HSV
25
#1 cause of bacterial meningitis
S. pneumo
N. meningitidis is #2
26
Rash of meningitis
Petechial
Maculopapular
Purpuric in appearance
Usually spares soles and palms
27
What doesn't the N meningitidis vaccine protect against?
Serogroup B
Is 1/3 of cases of bacterial meningitis in US
28
How do you dx aseptic meningitis?
CSF PCR
or
IgM antibody capture ELISA
29
Viral encephalitis EEG findings
EEG findings include focal delta activity over the temporal lobes, typically occurring between 2 and 14 days after symptom onset; periodic lateralizing epileptiform discharges (PLEDs) also may be noted.
30
When do you brain bx for encephalitis?
Pts who don't respond to acyclovir
31
When do you tx HTN in acute stroke?
When greater than 220/120
OR
There is another acute indication for lowering BP such as ACS, CHF, aortic dissection, hypertensive encephalopathy or AKI
- Lower Bp by 15% over 1st 24 hrs
32
In stroke, If the patient is otherwise a candidate for thrombolytic therapy, what should BP be?
When give it?
Contraindications?
blood pressure must be stabilized and lowered to 130 mm Hg, systolic blood pressure >185 mm Hg, diastolic blood pressure >110 mm Hg, or intracerebral hemorrhage
33
urgent anticoagulation with heparin for stroke?
is not recommended for patients with ischemic stroke unless cerebral venous thrombosis, basilar occlusion/stenosis, or extracranial arterial dissection is suspected.
34
When do you suspect vasculitis as cause of mononeuropathy?
When acute involvement of individual nerves is accompanied by pain
35
Difference in presentation of axonal vs demyelinating polyneuropathy?
Axonal - symmetric distal sensory loss +/- burning, tingling (eg diabetes)
Demyelinating - p/w motor sx (eg Guillain Barre)
36
Most sensitive for diagnosis of meningitis
Jolt accentuation of headache from horizontal mvmt of head
More sensitive than Kernig or Brudzinski sign
37
What HSV is more common causing encephalitis in
- adults
- children?
Adults - HSV 1
neonates - HSV 2
38
How does HSV encephalitis usually happen in adults?
Reactivate latent virus in trigeminal ganglion ---> inflammatory necrotic lesions in temporal cortex and limbic system
39
Intention or kinetic tremors are most characteristic of damage to
Cerebellum
40
What happens to Parkinson tremor when a person sleeps?
Resting tremor STOPS when relaxation progresses to sleep
BUT USUALLY HAVE PILL ROLLING TREMOR AT REST
Most tremors caused by basal ganglia pathology stop during sleep
41
What does hyperacusis tell you about where injury to CN 7 is?
Close to origin from brainstem b/c nerve to stapedius is one of 1st branches of facial nerve
42
Fasiculations occur because
Indicate denervation
Occur through hypersensitivity to Ach acting at denervated NMJ
43
Most common location for lumbar disk herniation
b/n L5 and S1
b/n L4 and L5
44
Common objective sign of S1 radiculopathy
Loss of ankle jerk or achilles tendon reflex
45
Hollenhorst plaques
Cholesterol and calcific deposits in retinal arterioles
46
West syndrome
Generalized seizure d/o of infants characterized by
- recurrent spasms
- EEG pattern of hypsarrhythmia
- retardation
Tx: ACTH
47
Women are more susceptible to symptomatic aneurysms than men in their 40s adn 50s. Where are the aneurysms?
Most especially true of those that are on internal carotid on segment of artery that lies in cavernous sinus
48
Difference between classic vs. common migraine
Classic = has preceeding aura of neuro dysfunction (usually visual)
DOES NOT HAPPEN IN COMMON
49
Difference between ischemic vs. compression injury of CN 3
Ischemic
- usually due to diabetes
- usually spare superficial pupilloconstricor fibers b/c of different vascular supply from rest of nerve
Compression
- usually get pupilloconstrictor fibers first b/c superficial
50
What artery lesions compress CN3?
Posterior communicating
Also but less common:
- SCA
- PCA
51
Trigeminal neuralgia can be associated with...
Multiple sclerosis
52
Lennox-Gasttaut syndrome
Mental dysfunciton
Multiple seizure types
- complex partial seizures usually
1-2 Hz generalized spike wave discharges on EEG
Many children have hx of infantile spasms (West syndrome)
20% of Tuberous sclerosis kids will develop this
53
Hypsarrhythmia
EEG pattern of children w/ paroxysmal flexions of body, waist or neck
54
Landau-Kleffner syndrome
Loss of language and function
| Abnormal EEG during sleep
55
Atypical facial pain vs. trigeminal neuralgia
Trigeminal neuralgia
- paroxysmal lacrimating pain
Atypical pain
- constant, deep pain
- usually bilateral but can be unilateral
- often sensitive to antidepressant meds
56
Olfactory aura preceding seizure - where is the lesion?
Hippocampus or parahippocampal gyrus
57
Epilepsia partialis continua
Condition of persistent focal motor seziure activity
- a focal motor status epilepticus
Distal hand and foot muscles most often affected
Response to therapy is poor
58
Causalgia
Can result from trauma to nerves in extremities
Is a disturbance in sensory perception characterized by
- hypesthesia
- dysesthesia
- allodynia
Bullets can cause this as their high velocity can cause enough energy transmission through adjacant tissues to produce damage to nerve
59
Hypesthesia
Decrease in accurate perception of stimuli
60
Dysestheisa
persistent discomfort
| - usually unremitting burning pain
61
Allodynia
perception of pain w/ application of nonpainful stimuli
62
Musculocutaneous N innervation
Damaged w/ fracture of humerus
Supplies
- biceps brachii
- brachialis
- coracobrachialis muscles
Carries sensory info from lateral cutaneous nerve of forearm
Damage causes:
- impaired flexion at elbow with forearm supinated
63
For subdural hematoma of a few days, what imaging do you use?
Not CT - after few days, blood can be degraded into less dense fluid and difficult to distinguish
- eventually will be hypodense to brain
USE MRI
64
Which brain bleeds are unilateral? bilateral?
bilateral - subdural
unilateral - epidural
65
Tinel sign
sensation of tingling radiating away from percutaneous percussion of a peripheral nerve
Usually happens w/ carpal tunnel syndrome
60% sensitivity
67% specificity
66
Brudzinski and Kernig signs
Indication of meningeal irritation
67
Monrad-Krohn test
Confirm psychogenic upper extremity monoparesis
68
What is most common sign of neuro disease w/ encephalitis lethargica?
Disturbed eye movements
69
Most common sequelae of encephalitis lethargica
Unremitting parkinsonism w/ signs and symptoms similar to in idiopathic Parkinson
UNIQUE: oculogyric crises = episodes where eyes deviate to one side or up
70
Facial injury most likely to develop w/ sarcoidosis
Facial paresis
Weakness on 1 side of face
+
no substantial loss of sensation over paretic side
Maybe decreased sensitivity to touch
71
Sequelae of Schistosoma mansoni
Endemic in tropics
Can cause subacute evolving paraparesis
DOES NOT invade spinal cord
- deposits eggs in valveless veins of Batson which drain intestines and communicate w/ drainage from lumbosacral spinal cord
Develop granulomas around ova that lodge in spinal cord
- granulomatous lesions crush the cord
72
Echinococcosis clinical findings
Usually acquired by ingesting material contaminated w/ fecal matter from sheep or dogs
Children - cerebral lesions more often than adults
= encephalic hydatidosis
73
CSF findings with CJD?
Usually normal
May have elevated protein level
20% of cases may have increase in ratio of IgG : total protein, occasionally w/ oligoclonal bands
74
Most common etiologies of rim-enhancing brain lesions in AIDS pts
Primary CNS lymphoma
- CSF EBV PCR test is highly sensitive and specific for this
Toxoplasma infection
75
What kind of lesions in brain do you have with HIV and CMV encephalitis?
Microglial nodules
HIV = usually nodules around blood vessels throughout brain
CMV = nodules usually subpial and subependymal
76
Where do discharges in herpes encephalitis on EEG typically occur? What kind of discharges?
Over temporal regions
Bilateral, periodic epileptiform discharges
77
How do abscesses in brain usually form?
Hematogenous spread of infection
78
Where do brain abscesses usually start in the brain?
At junction of gray matter and white matter
As infection develops, cerebritis appears and subsequently this focus of infection becomes necrotic and liquefies
79
Syphilis - long term sequelae
General paresis
- slow evolving
Early sx:
- subtle dementia (memory loss, impaired reasoning)
Late sx:
- dysarthria
- myoclonus
- tremor
- seizures
- UMN signs
80
Structural brain changes with neurosyphilis
Meninges are thickened and opaque
Granular ependymitis develops
Degenerative changes throughout cerebral parenchyma
81
Rhombencephalitis - principal targets of this disease?
Pons
| Medulla oblongata
82
Most common sx among those w/ brain abscesses?
Headache (3/4 of pts)
tx w/ surgical resection
83
Most common pathogen for brain abscesses
Aerobic +/anaerobic STREP bacteria (> 1/2)
Bacterioides
Staph aureus in pts w/ penetrating head wounds or undergone neurosurgical procedures
84
Old person + stiff neck + blurry vision + fever + headache
CSF = mild pleocytosis w/ no organisms
All blood + CSF cx negative
best med tx for organism likely responsible?
Could be listeria monocytogenes
Use ampicillin + gentamycin
85
Inclusion bodies in oligodendrocyte nuclei - what does this suggest for an infection?
How to dx?
JC virus = PML
Use MRI to dx
- multiple focal well-defined white matter lesions that DO NOT enhance or have mass effect
86
When are eye muscles affected in guillain Barre?
Miller Fisher variant
87
Guillain Barre vs. polio - how do you telll?
CSF analysis
GB = elevated protein count + normal or slightly elevated abnormal WBC + normal glucose
Polio = small increase protein + lymphocytes
88
Subacute sclerosing pancencephalitis
- causes
- CSF analysis
SSPE
Usually in children
Usually kids had measles
Death usually in 1-3 years
CSF like MS w/ oligoclonal bands (measles-specific antibody)
89
Regional adenitis usually in epitrochelar nodes
What is this assoc w/ ?
What does it look like?
Tropism?
Cat scratch disease
- B. henselae
Can produce self-limited aseptic meningitis
In immunocompetent, can get encephalitis + status epilepticus + neovascular proliferation (bacillary angiomatosis)
MRI may show characteristic increased signal intensity in the pulvinar (part of thalamus)
90
Calcification 2/2 brain tumor on skull xray suggests...
Astrocytoma
Meningioma
Oligodendroglioma
Metastatic tumor
Meningioma -- hyperostosis may develop in bone adjacent to tumor even w/o infiltration of bone by tumor
91
#1 source of metastatic tumors to brain?
Lung
92
Why is melanoma in brain esp bad?
Highly likely to bleed after it metastasizes to brain
Same is true of mets w/ choriocarcionma
93
Precocious puberty / acromegaly + visual field developments + no cancer
Hypothalamic hamartomas
94
Craniopharyngiomas
Epithelial neoplasms arising in sellar and 3rd venticular regions
Can cause HYPOpituitarism + visual field defects (vs hyper in hypothalamic hamartomas)
95
NF-1 clinical sequelae
Cafe au lait spots
Multiple cutaneous + subQ tumors
Bone cysts
Sphenoid bone dysgenesis
Precocious puberty
Pheo
Syringomyelia
Glial nodules
Cortical dysgenesis
Macrocephaly
96
Parinaud syndrome
Loss of vertical gaze, pupillary light reflex, lid retraction, covergence-retraction nystagmus
Usually due to lesion in dorsal midbrain in region of superio colliculus
CAn be caused by pineocytomas
97
Paraneoplastic cerebellar degeneration
Subacute, progresive ataxia, dysarthria, nystagmus
Usually assoc w/ Small cell carcinoma, ovarian carcinoma, lymphoma
50% pts have anti-Purkinje cell bodies (anti-Yo Abs)
98
Fabry disease
- deficient enzyme
- accumulated substrate
a-galactosidase A
Ceramide trihexoside
99
Gaucher's disease
- deficient enzyme
- accumulated substrate
Glucocerebrosidase
Glucocerebroside
100
Krabbe's disease
- deficient enzyme
- accumulated substrate
Gaucher and Krabbe are friends!
GALACTOcerebrosidease
Galactocerebroside
101
Neimann-Pick disease
- deficient enzyme
- accumulated substrate
Sphingomyelinase
Sphingomyelin
102
Tay Sach's disaese
- deficient enzyme
- accumulated substrate
HeXosaminidase A
GM2 ganglioside
103
Metachomatic leukodystrophy
- deficient enzyme
- accumulated substrate
Arylsulfatase A
Cerebroside sulfate (galactosyl sulfatides)
Can use nerve biopsy to diagnose
104
Type of peripheral neuropathy most commonly developing w/ CRF
Symmetric
Distal
Mixed
Sensorimotor neuropathy
Legs affected 1st usually and most severly
Dialysis causes you to lose B vitamins --> neuropathy (thiamine depletion)
105
Akathisia
Restlessness occuring during daytime
| vs restless leg syndrome is only at night
106
Most damaged areas in spinal cord w/ cobalamin deficiency
Lower cervical
| Upper thoracic
107
Pathology of neuropathy in cobalamin deficiency
Starts as demyelinating lesion
Evolves into axonal loss
(Hyperreflexia --> hyporeflexia
108
Vitamin deficits in tobacco-alcohol amblyopia
B1
B12
Riboflavin
109
Vit E deficiency sequelae
Spinocerebellar degeneration
Polyneuropathy
Pigmentary retinopathy
```
Usually degen in :
Clarks columns
Spinocerebellar tracts
posterior columns
Nuclei of Goll and Burdach
Sensory roots
```
Get ataxia
110
Pickwickian syndrome
Obesity assoc w/ hypersomnia
Ex sleep apnea
111
Features of Alzheimer's disease
Neuronal loss
Fibrillary tangles
Loss of synapses
Amyloid plaque formation
Tangles + neuronal loss most common in hippocampis + adjacent structure in hippocampus
Language deficit (decreased fluency, dysnomia, transcortical sensory aphasia)
112
EEG of Alzheimer's pt
Slowing of posterior-dominant rhythm (8-12hz) of normal adult EEG
113
#1 cause of dementia in gen pop
Alzheimer's disease
114
NPH s/p ventriculoperitoneal shunting - complications?
Subdural hematoma
- reducing intracrainal P due to less CSF may cause brain to pull away from covering meninges and break bridging veins
Infections
115
EEG findings of CJD?
Periodic sharp waves at 1-2 Hz frequency on EEEG
Elevated protein 14-3-3- in CSF
116
Neurosyphillis dx
Monocytic pleocytosis
+ serological tests for syphilis
117
Neuro sx of hypothyroidism
headache
dementia
psychosis
decreased consciouness
118
Neuro sx of Whipple disease
CNS infection can happen w/o GI disease
```
Seizures
Myoclonus
Ataxia
Supranuclear gaze disturbanes
hypothalamic dysfunction
dementia
```
Oculomasticatory myorhythmia (pendular convergence mvmts of eyes in assoc w/ contractions of masticatory muscles) = pathognomonic
Bx of jejunum to dx
119
Where is the substantia Nigra?
Brainstem
120
What is PML?
In immunocomp, usually AIDS pts
Affects subcortical white matter usually in
- OCCIPITAL
- PARIETAL regions
CSF normal
Lesions do not enchance on imaging
See foci of abnormality on MRI
Usually does not cause mass effect - onset is gradual
Most common presenting sx:
- hemiparesis
- disturbances in speech, vision, gait
121
Chorea gravidarum
Involuntary mvmt d/o during pregnancy
Rapid + FLUID but not rhythmic limb and trunk movements
Can appear w/ estrogen use too
Main problem is dramatic change in hormonal environment of brain
At end of preggers, or no more estrogen, movements stop
122
How do dopaminergic drugs affect Huntington's in the beginning stages?
Can unmask chorea
NOT advisable b/c can contribute to premature symptom of chorea
123
Choreiform movement disorders
Huntington
Hereditary acanthocytosis
124
Theory of Parkinson's
substantia nigra pars compacta has decreased dopamine production
Get overinhibition of thalamocortical pathways
125
Ways to tx Parkinson's
Thalamus can eb directly intervened to decrease overinhibition
Globus pallidus interna can be lesioned/stimulated
Lesion/Stimulate subthalamic nucleus
126
Lewy Bodies
IN Parkinson's
IntraCYTOPLASMIC inclusion bodies
Eosinophilic inclusions w/ poorly staining halos surrounding them
127
Effects of language in Parkinson's
Not disturbed
Clarity and volume of speech deteriorate w/ development of hypophonia
Handwritting also gets smaller = micrographia
128
Meige syndrome
Form of focal dystonia characterized by:
```
Blepharospasm
Forceful jaw opening
Lip retraction
Necak contractions
Tongue thrusting
```
Can occur w/
- phenothiazine
- butyrophenone use
- idiopathic (women > men)
129
Spasmodic torticollis
Focal dystonia
Starts early in adult life
Contraction of neck muscles --> painful --> hypertrophy
Standing and walking worsen contractions
130
Manifestation of Wilson's disease
Renal tubular acidosis
Hepatic fibrosis
Heart + lung damage
Brain + liver disease
Dementia
131
Oligoclonal bands on CSF ddx
MS
Syphilis
Lyme
Subacute sclerosis pancencephalitis (SSPE)
132
Pt feels electrical sensation radiating down spine when neck passively flexed - what is this? What does it signify?
Lhermitte sign
Signifies spinal cord disease
Happens in MS pts
133
Composition of oligoclonal bands in MS pts
IgG
K-light chain composition
134
Jolly test
Evoked response involving muscles
Cannot usually be used to test VER in MS
Peripheral nerve shocked 5-15x per second and pattern of A/P is recorded
- Peripheral test normal in MS as is more a CNS disease
135
Canavan disease
Defect in N acetylaspartic acid metabolism
Can get developmental regression ~ 6mo age
Extensor posturing and rigidity
Myoclonic seizures
Increase in brain volume and weight
136
Bladder abnormality w/ MS
Spastic (UMN) bladder
Little or no residual urine in bladder after emptying -- contractility good but distensibility poor
CAN GET MS EXACERBATION WITH EXERCISE! Because of the heat
137
Neuromylitis optica
Bilateral optic neuritis + transverse myelitis
- demyelinate optic N and spinal cord
Transverse myelitis = inflammatory demyelinating lesion transecting most of spinal cord
- paraparesis
- bladder and bowel dysfunction
- sensory deficit
Can have cerebellar involvement, more cerebral involvement
Looks like MS but is not!
138
CSF profile of acute disseminated encephalomyelitis
Often fatal
MRI or CT shows rapidly evolving white matter damage assoc w/ high ESR
CSF:
- increased P
- elevated RBC, WBC
- elevated protein
- glucose NORMAL
139
Adrenoleukodystrophy
Adrenal dysfunction + progressive degenerative disease of white matter
Some types are X linked
- mostly in boys
- survival limited to ~3 yrs
Underlying defect in X linked = ATP binding transporter in peroxisomal system responsible for long chain fatty acid metabolism
LCFA accumulate in adrenals and other cells
Similar to adrenomyeloneuropathy in woemnn
140
Adrenomyeloneuropathy
More damage to spinal cord + peripheral nerves
Paraparesis
Problems w/ bowel and bladder control
Sensory distrubances in legs
141
Pelizaeus-Merzbacher disease
Demyelinating d/o part of disease known as sudanophilic leukodystrophies
Mostly males
affect growth of the myelin sheath
142
Leukodystrophy
disturbance of white matter
143
Sudanophilic
Sudan-staining characteristics of involved white matter
144
Lipid profile:
- absent chylomicrons, VLDL, LDL
- serum and red-cell lipid proteins normal
What is the disease? What is the defect/mutation? What does it look like?
Abetalipoproteinemia (Bassen-Kornzweig syndrome)
Mutation of gene: microsomal triglyceride transfer protein
RBC = acanthocytes
Ataxia
- posterior column + spinocerebellar tract degeneration (like Friedreich)
Vitamin E can help stop progression
145
VHL person has cyst + masses in cerebellar hemisphere on CT. What do you do?
Surgical resection of cerebellar lesions ASAP
Likely hemangioblastomas
- can bleed and produce potentially lethal intracranial hematomas
146
Fragile X phenotype
Females - normal
Men -
hyperextensible joints
prominent thumbs
Can detect abnormal chromosome in fetal lyphocytes + fibroblasts for prenatal screening
147
With PKU, what does the child have dangerously high levels of?
Phenylalanine
148
Railroad track pattern on plain xray of skull - intracranial calcification pattern indicative of?
sturge weber
Calcifications follow gyral pattern of cerebral cortex
Abnormal blood vessels overlying brain allow Ca, Fe across defective blood-brain barrier --> calcifications
149
```
Erythrocytosis +
Cerebellar signs +
Microscopic hematuria +
Hepatosplenomegaly
= ?
```
von Hippel Lindau syndrome
Has:
polycystic liver + kidney disease
reitnal angiomas (telangiectasia)
cerebellar tumors
AD w/ variable penetrance
More men affected
Hemangiomas in bones, adrenals, ovaries
Hemangioblastomas in SC, brain stem, cerebellum
150
Infection causing congenital aqueductal stenosis --> hydrocephalus
Mumps
| Rubella
151
Tuberous sclerosis
| - characteristics
```
Retinal phakomas
Adenoma sebaceum
Periventricular tubers
Ash leaf spots
Shagreen patches
CNS calcifications
Renal tumors
Cardiac rhabdomyomas
Epilepsy
```
152
By 5 years of age, more than 1/2 of pts w/ tuberous sclerosis will have...
Subependymal glial nodules that have calcified
Can get big enough to cause obstructive hydrocephalus
153
Child w/ congenital weakness, hypotonia, muscle atrophy
Werdnig-Hoffman disease
congenital motor neuro disease
Anterior horn cell disease
Life expectancy = weeks - months
154
Older child w/ weakness, hypotonia, muscle atrophy
Kugelberg-Welander disease (like Werdnig-Hoffman but in older kids)
Part of class known as spinal muscular atrophies
Anterior horn cell disease
155
Static encephalopathy
Brain damage stopped by neuro problems persist
156
Static motor disorder
Child w/ this has cerebral palsy
Will have static brain lesion but deficits may evolve as child matures
157
Alcohol abuse in preggers
- fetal abnormalities?
- mech of action?
1) Intrauterine + postpartum growth retardation
2) dysmorphic facies in newborn
3) effects on development of CNS
EtOH teratogenic at high doses
- impairs neuronal migration
Commonly causes
- mental retardation, learning disabilities, hyperactivity, micocephaly (not MACRO)
158
Prenatal CMV infection - problems developing?
Retardation
microcephaly
Seizures
Hearing deficits
Chorioretinitis
Optic atrophy
159
What's a skin paraneoplastic syndrome?
Dermatomyositis! (15% of cases of derm are paraneoplastic)
Lung, ovarian, GI, breast tumors can cause this )not CNS)
Always check for cancer when pt has dermatomyositis
160
HIgh CPK in woman w/ relatives affected by Duchenne dystrophy = ?
High prob that she is carrier of abnormal dystrophin
normal CPK does not rule out carrier status
161
Duchenne dystrophy incidence?
1 in 3,000 male infants
162
Myotonic dystrophy
```
Problems relaxing grip
Hypersomnolence
Premature baldness
Testicular atrophy
Cataracts
Cardiac defect needing pacemaker
```
EMG:
dive bomber pattern
repetitive discharges w/ minor stimulation
163
Polymyalgia rheumatica
ONLY in persons older than 60 yo
```
Inc ESR
Anemia
Wt loss
Malaise
Normal CPK
```
164
Signs of brainstem disease in ALS
ALS has loss of
- anterior horn cells (LMN)
- motor nuclei of brainstem + loss of large motor neurons (Betz cells) (UMN)
Brainstem disease:
Diaphragmatic weakness
Facial fasiculations
Brainstem disease signs early in disease is poor prognosis (<1 yr survival)
165
What indicates graver prognosis in ALS?
Brainstem disease
= early involvement of musculature supplied by cranial nerves
```
Ex:
disturbed swallowing
recurrent aspiration
distrubed ventilatory activity
fasiculations of tongue
```
166
How does EtOH abuse damage brain?
Superior vemis of cerebelllum:
- loses purkinje cells
- atrophy off molecular layer
White matter is cerebellum usually OK
167
Lathyrism
Slow or subacute onset of spastic paraparesis
- too much chickling pea diet (L sativus)
Toxin through to be B-N-oxalylamino-L-alanine = excitatory NT that can induce disease in primae models
Damage in CNS usually in SC tracts (corticospinal, spinocerebellar tracts)
Demyelination in lateral and posterior columns of SC
168
Tick paralysis
Can happen esp w/ australian tick producing holocyclotoxin = interfere w/ presynpatic release at NMJ
Remove tick, get dramatic improvement
169
Risk w/ radiation for Hodgkin disease
Thyroid cancer
Carotid stenosis b/c of accelerated atherosclerosis from ionizing radiation
Surgical tx more difficult b/c radiation causes scarring of tissues around vessels --> harder to get to for surgery
170
Hepatic encephalopathy
| - sx
```
Initially:
Decrease in level of alertness
irritability
Depression
tremor
asterixis
```
```
Later:
Lethargy
Paranoia
Bizzare behavior
Dysarthria
Nystagmus
Pupillary dilatation
```
171
Sturge weber syndrome
```
Port wine spots on faces
Contralateral hemiparesis
Retardation
Seizures
Glaucoma
Leptomeningeal angiomatosis causing intracranial calcifications
Angioma of choroid of eye
```
172
Pseudotumor cerebri
Increased CSF pressure
- not at risk of herniation
Can happen in obese or preggers woman - thought to rise from hormonal problems
W/o tx, increased ICP will produce optic N damage w/ loss of VA
173
reversible posterior leukoencephalopathy
Looks like HTN encephalopathy
Involves more than white matter, occur in anterior frontal regions
caused by cyclosporine and tacrolimus
174
Do you get complete hearing loss on one side w/ one side temporal lobe infarction?
No
Hearing in each ear is represented bilaterally (even at brainstem)
If unilateral hearing loss, eval if loss is conductive or sensorineural
175
Mastoditis can extend...
Supratentorially --> temporal lobe --> fluent aphasia (Wernicke)
Infratentorially --> cerebellum --> ataxia
176
Mechanical trauma hearing loss - what does it look like?
High tone conductive loss
Perforates eardrum
177
Cerebellar damage + severe vertigo
| - where is the lesion?
PICA lesion
Vertigo caused by:
- vestibular nuclei infarction
- cerebellar flocculonodular lobule injury
178
Vertigo on extreme extension or rotation of head --> insufficiency of?
Vertebrobasilar system
179
Transient dizziness + ringing + decreased hearing in one ear
Early hearing loss in this disease is?
Meniere's disease!
- can also have fullness in ear
Lower frequency hearing loss
Thought to be due to distension of semicircular canal and increase in volume of endolymph fluid
180
Presbycusis
High frequency hearing loss
181
Acoustic schwannomas usually tumors on what?
vestibular division of nerve
182
Olfactory cortex located...
Olfactory tract --> medial + lateral striae
Medial stria --> anterior commissure --> opposite hemi
Lateral stria --> medial adn cortical nuclei of amygdaloid complex + prepiriform area
Primary olfactor cortex = area 34 of Brodmann
- hippocampal gyrus + uncus
NO FIBERS THROUGH THALAMUS
183
Foster Kennedy syndrome
Ipsilateral optic atrophy + Contralateral papilledema in assoc w/ intracranial tumor
Meningioma of olfactory groove can produce this syndrome
184
Benign Positional vertigo
Usually in middle aged ppl
Possibly due to otolith material in posterior semicircular canal
Attacks of recurrent rotational vertigo
- change in head position
- lying down
- turn head onto side of affected ear
Use nylan-barany or hallpike maneuvers to confirm peripheral cause of vestibulopathy rather than central
- will get rotary nystagmus
- fatigability in sx (decrease them w/ increased provocation)
185
```
Progressive vertigo
Ataxia
Sensory loss
Dysphagia
Hiccups
```
Sx of lateral medullary syndrome
Usually due to distal vertebral artery occlusion
186
Low lying vermis or cerebellar tonils + syringomyelia
Chiari malformation
Posterior fossa abnormally small
Tentorium cerebelli relatively low on cranium
187
Tx syringomyelia
Laminectomy
- reduces damage to SC from pressure that develops
Cyst aspiration
Marsupilization
- slice open adn leaving open cyst
Shunting
188
Spinal shock
Transient phenomenon
Occurs w/ damage to fibers from UMN
Usually within a few DAYS of spinal cord injury
Represents exaggeration of normal stretch reflexes in limbs disconnected from UMN control
189
Lumbar spine injury in MVA - how did it happen?
Extreme flexion
190
Syringomyelia
Pain and temp loss - bilateral
Tactile sensation ok
191
Artery of Adamkieqicz enters at what level of SC?
At risk for occlusion w/ ab aortic aneurysm repair
Supplies lower 2/3 of spinal cord
T10-L1
192
Hemisection of SC - at what level do you get STT effects?
Contralateral 2 levels below the spinal level hemisection
193
Periumbilical area innervated by
T10
194
Charcot joints
Result of cumulative damage from loss of reflexes and diminished pain awareness
Classically assoc with syphilis or diabetes
195
Ulnar nerve supplies (motor)
C8-T1
All interosseus muscles - finger adduction + abduction
Lumbricals on ulnar metacarpals - extend digits
196
Schistosomiasis
Use MRI to see lesions!
Eggs embolize to CNS
```
Itching of skin
Lancinating pains in legs + toes
Paraparesis
Loss bladder and bowel control
Unable to stand
```
S masoni is endemic to Puerto Rico + S. America
- embolizes to SC
S. japonicum also embolizes eggs
197
Lateral Corticospinal tract decusates where?
At junction of medulla and SC
198
Spinal claudication
IS NOT THE SAME AS LEG CLAUDICATIOn
Leg pains b/c shunt blood to leg muscles and pain happens due to ischemia of sensory neurons in SC
199
Spondylolisthesis
Slippage of vertebral elements
200
CSF findings w/ spinal cord infarction or cerebral infarction
Usually normal
Sometimes get elevated protein
201
Achilles reflex
S1, 2
202
Patellar reflex
L3, L4
203
Biceps reflex
C5, C6
204
Triceps reflex
C7, C8
205
#1 cause mononeuropathy multiplex
Diabetes mellitus
206
Friedreich disease
- what it affects
- associations
Defect on chromosome 9
- protein frataxin defect
- AR defect - trinucleotide repeat
Affects:
- Spinocerebellar
- Dorsal columns
- Lateral corticospinal tract
- peripheral neuropathy w/ degeneration of dorsal root ganglia
```
Associations:
HOCM
Diabetes
- can cause visual problems w/ hyperglycemia
Optic atrophy
```
Can see effects at juvenile period
207
Parsonage-Turner syndrome
Acute brachial plexopathy
Acute onset pain in neck, shoulder, or upper arm
3-10 days later: weakness affecting proximal arm muscles
Sensory loss too
Cause unknown; can happen w/ vaccination or viral infection
Can have familial cases w/ AD pattern
208
Riley Day disease
Familial dysautonomia
AR d/o usually in Jewish kids
Small fiber neuropathy affecting mylinated and unmyelinated small fibers
--> impair pain and temp sensation
SNS and PSNS also affected --> autonomic effects
- no tears on crying
- corneal ulceration
- no pupillary reactivity
- poor temp regulation
- lots of perspiration
- blood pressure control abnormality
- dysphagia
- recurrent vomiting
- gastric and intestinal dilation
NO papillae of tongue
NO tx
209
Chronic inflammatory demyelinating polyneuropathy (CIDP)
Like G-B but slower
Affects proximal portions of nerves where they exit SC @ root level
--> causes increase in protein --> inflammatory d/o --> demyelinates
Get paresis, sensory loss proximally and distally
210
Porphyric polyneuropathy
assoc w/ AIP
GI paresis
Ab pain
Constipation
Psychosis
Axonal motor neuropathy
Autonomic instability
Seizures, SIADH
211
Acute Wernicke lesions where?
Periaqueductal and mamillary bodies
- hemorrhagic necrosis
Get autonomic failure
212
Botulinism electophsiologic testing results
Posttetanic potentiation of compound muscle A/P
- decremental response of muscles to repetitive stim at low freq
- incremental response to repetitive stim at high freq
Same profile as Lamber Eaton
213
Acute disseminated encephalomyelitis vs MS
ADEM
- demyelinates brain, brainstem, SC
- monophasic --> only 1 occasion (vs recurrent MS)
MS
- recurrent
214
When do emergency surgery w/ disk herniation?
If hurts cauda equina
| - if see evolving focal motor deficit in legs (eg footdrop) + spihincter dysfunction --> surgery ASAP!
215
Wernickes encephalopathy
Deteriorating level of consciousness
Autonomic disturbances
Ocular motor problems
Gait difficulty
Lethal HYPOTN
Hypothermia
216
Head trauma + periorbital ecchymosis + echymosis over mastoid region + hemotympanum (blood behind eardrum)
Basilar skull fracture
217
Diffuse axonal injury
Pathology:
| diffusely spread axonal swellings of white matter, corpus callosum, upper brainstem that are usually hemorrhagic
218
What should you do for urinary incontinence during status epilepticus?
Condom catheter
Keeps pt dry
allows collection urine
monitor I/Os
219
Parotid surgery for parotid tumor can cause what kind of CN palsy?
Facial Nerve
220
Acute shoulder pain after forceful abduction and external rotation - nerve injured? What happened?
Anterior dislocation of humerus
Axiallary N injured
Artery can be injured too
221
Peripheral neuropathy classifications
Mononeuropathy
Mononeuropathy multiplex
Axonal polyneuropathy
Demyelinating polyneuropathy
222
Mononeuropathy
- distribution/pattern
- examples
Focal
Ex:
Carpal tunnel
Bell palsy
223
Mononeuropathy multiplex
- distribution/pattern
- examples
Asymmetric, multifocal (several noncontiguous nerves)
```
Ex:
Vasculitis
DM
Lymphoma
Amyloidosis
Sarcoidosis
Lyme
Acute HIV
Leprosy
```
224
Axonal polyneuropathy
- distribution/pattern
- examples
Symmetric, distal
Mostly sensory
```
Ex:
EtOH
Drugs
Chronic arsenic exposure
DM
Uremia
B12 deficiency
Folate deficiency
HypoThyroid
Paraproteinemia
Paraneoplastic
chornic HIV
Charcot-Marie-Tooth
```
225
Demyelinating polyneuropathy
- distribution/pattern
- examples
Symmetric, often PROXIMAL
Mostly motor
Ascending spread
```
Ex:
acute arsenic exposure
Guillain Barre
Chronic inflammatory demyelinating polyneuropathy
Paraprotenemia
```
226
DDx dementia
```
Alzheimer
CJD
Delirium
Dementia w/ Lewy bodies
Depression
Frontotemporal dementia (Picks disease)
Mild cognitive impairment
Normal pressure hydrocephalus
Vascular dementia
```
227
Alzheimer's Disease
Gradual memory loss
Preserved consciousness
```
Progression of disease:
aphasia
apraxia
agnosia
inattention
L-R confusion
```
228
CJD
Rapid progression
Prominent myoclonus
EEG: triphasic sharp waves
CSF protein 14-3-3 has good specificity
Diffusion weighted MRI is more sensitive and specific
229
Delirium
Altered level of alertness and attention often w/ globally impaired cognition
Abrupt onset
Fluctuating level of alertness common
230
Dementia w/ Lewy Bodies
Early signs:
Mild parkinsonism
Hallucinations
Delusions
231
Depression
SIGECAPS
```
Sleep changes
Interest lost
Guilty
Energy lack
Cognition/concentration reduced
Appetite decrease
Psychomotor retardation
Suicidal ideation
```
232
Frontotemporal dementia
Picks disease is example
```
Often < 60 yo
Language difficulties
Behavioral disturbances
Impulsive
Aggressive
Apathetic
```
Diminished function in frontal and/or temporal lobes
233
Normal Pressure hydrocephalus
Dementia
Gait ataxia
Urinary incontinence
Psychomotor slowing
Apathy
Can cure w/ ventriculoperitoneal shunting
234
Vascular dementia
Loss of function may be temporally correlated w/ cerebrovascular events
May be assoc w/ silent strokes, CV risk factors
235
EEG pattern of absence seizures
Generalized, symmetrical 3-Hz spike and wave activity on normal background
Finding from hyperventiation of pt during EEG
236
Tx epidural hematoma
Emergent craniotomy
237
Restless leg syndrome
4 cardinal sx:
Uncomfortable sensation or urge to move legs
Discomfort worsens in eve or sleep
Discomfort worse at rest
Discomfort relieved by mvmt of affected limbs
238
Sx of heat stroke
```
Temperature > 40C (105 F)
AMS
HYPOTn
Tachy
Tachypnea
```
Can get rhabdo
239
How to assess for Delirium?
| - 4 questions/things to find out
Need 1 & 2 + 3 or 4
1) Onset
- from family member
- is there evidence of acute change in MS from pt's baseline?
- Did abnormal behavior fluctuate during day, or increase and decrease in severity?
2) Inattention
+ response to : Did pt have difficulty focusing attention?
3) Disorganized thinking
+ response to: Was pt's thinking disorganized or incoherent (rambling, illogical, switch subject to subject)?
4) Altered level of consciousness
- Overall, how would you rate pts level of consciousness?
- alert
- hyperalert
- drowsy, easily aroused
- difficult to arouse
- unarousable
240
CPP =
MAP - ICP
241
Cushing reflex
TRIAD: Increased systolic, bradycardia, irregular respiration
Increase in ICP causes a decrease in CPP
SNS will first kick in - vasoconstrict to increase MAP and tachycardia
Baroreceptors in aortic arch will then detect increase in BP and activate PSNS via vagus
- Causes bradycardia
These all can cause increased P in brainstem --> affect respiratory centers --> apnea
242
Cord compression dx
spinal MRI
243
Charcot Marie Tooth disease
PNS disease, demyelinating
progressive loss of muscle tissue and touch sensation across various parts of the body
244
How long can it take to see blood on CT without contrast?
24 hrs
245
Peripheral demyelinating disorders in kids
Charcot Marie Tooth
| Diptheria
246
3 causes of subacute facial bilateral palsy
Sarcoid
Lyme
Guillain Barre
247
#1 cause of status epilepticus
Epileptics that don't take meds
248
Most likely dx: essential tremor
- next dx step
- next step in therapy
MRI of brain and spine
Primidone, propanolol
Can see cogwheel effect in ET vs cogwheel rigidity in PD
249
Spinal muscular atrophy type I
Caused by excessive programmed cell death
Hypotonic and weak baby but appears alert
---this means it is periph rather than central defect
LMN defects
Pure motor disorder
Extra ocular muscles usually spared
Moro and tonic neck reflexes present
Tongue fibrillations common
Next step dx: molecular testing for SMN1 gene
Tx: supportive
250
Remember these causes of stroke
Ischemia from atherosclerosis
Afib throwing clot emboli
Septic emboli from endocarditis
251
Spasticity vs rigidity
Spasticity is velocity dependent
- if move arm faster, less spastic
Rigidity is NOT velocity dependent
- will get same rigidity w/ fast or slow mvmt
252
MCA stroke of L hemi
Aphasia
Contralateral hemiparesis (right side)
Contralateral hemisensory loss
253
MCA stroke of R hemi
If this is nondominant hemi,
Apraxia
Contralateral neglect
Confusion
Contralateral hemiparesis
Contralateral hemisensory loss
254
ACA stroke
Contralateral lower extremity and face weakness (paresis)
```
Contralateral motor and/or sensory deficits more in the lower limb
Urinary incontinence
Gait apraxia
Primitive reflexes
Abulia (lack of will/initiative)
Paratonic rigidity
```
255
Vertebral/basilar stroke
```
Ipsilateral
Ataxia
Diplopia
Dysphagia
Dysarthria
Vertigo
```
```
Contralateral
Homonymous hemianopia (PCA)
```
256
PCA stroke
Contralateral Homonymous hemianopia
```
Alexia w/o agraphia (dominant hemi)
Visual hallucinations (Calcarine cortex)
Sensory sx (thalamus)
3rd nerve palsy w/ paresis of vertical eye mvmt
Motor deficits *cerebral peduncle, midbrain)
```
257
Lacunar stroke
Internal capsule = pure motor hemiparesis
Thalamus = pure hemisensory loss
Also ataxic hemiparesis due to cerebrllar fibers through thalamus
Pons = dysarthria, clumsy hand
258
Pupillary findings in ICH and corresponding level of involvement
Pinpoint pupils = pons
Poorly reactive pupils = thalamus
Dilated pupils = putamen
259
CT scan of ischemic vs hemorrhagic stroke
Ischemic appears dark
Hemorrhagic looks white
260
When is surgery good for ICH?
Cerebrllar hematomas
Usually not helpful most
261
Shy Drager syndrome
Multiple system atrophy
Parkinsonian sx
autonomic insufficiency (hypoTN, abnormal sweating, impotence, gastroparesis, etc)
Widespread neuro signs (cerebellar, pyramidal or LMN)
ALWAYS CONSIDER when pt w/ parkinsonism experiences orthostatic hypotension or other autonomic sx
262
Where are neurons lost in PD?
Substantia Nigra
Locus ceruleus
263
Huntington pathology
Loss if GABA producing neurons in striatum
264
Dx MS
Clinically
2 episodes of symptoms
2 white matter lesions on MRI
265
Primary generalized Dystonia
GAG deletion of DYT1 (TOR1A gene)
No sensory loss
AD
Work up: MRI brain
Tx: DBS of globus pallidus, pars interna to stimulate
266
What are lewy bodies made of and where do they deposit?
a-synuclein
Nucleus basalis of meynert
Locus ceruleus
dorsal raphe
dorsal motor nucleus of CN 10
267
Does familial PD often have lewy bodies?
No!
But it is early onset
268
Mimics PD
Multiple System atrophy
(shy Drager, striataonigral degeneration)
- less tremor
- symmetrical
Dementia w/ Lewy Bodies
- cognitive dysfunction
269
What can you have impaired temp sensation in?
Riley Day disease
Spinocerebellar ataxia type 3
270
Is parkinsonism of SCA responsive to levadopa?
Yes
271
What on MRI is characteristic of SCA?
High T2 signal in cerebellum
272
How to tell difference between T1 and T2 MRI
T1 - white matter is white, gray is dark
T2 - white matter is dark, gray is bright
273
Anterior cerebral artery supplies
Medial frontal lobe
Anterior thalamus
Anterior limb of internal capsule
Caudate nucleus
Olfactory bulb and tract
274
Middle cerebral artery supplies
Lateral cerebral hemispheres
Choroid plexus of lateral ventricles
Internal capsule
Some basal ganglia
275
Posterior cerebral artery supplies
Thalamus
Posterior internal capsule
Occipital lobe
276
Mechanism tardive dyskinesia
Unknown
Maybe cascade of responses develop in response to blockade of receptors by dopamine agonists
277
What percent of ppl on dopamine receptor antagonists get TD?
1/3
278
What's characteristic of TD?
Spasms of back and neck
279
How long to develop tardive dyskinesia?
1 month
If more acute, is acute Dystonia
Need to have for 3 months for dx
280
Tardive dyskinesia due to?
Dopamine receptor blocking drugs
Not caused by dopamine depleting drugs
281
Where is dopamine in basal ganglia?
Substantia Nigra pars compacta
282
What is rate limiting step of dopamine synthesis?
Tyrosine hydroxylase
Converts tyrosine to dopa
283
Parkinson genetics
Mutation in a-synuclein on chromosome 4q21
284
CT vs MRI is better at what?
CT better for bone
MRI better to see soft tissue
285
Sleep is mediated by
Serotonin
Norepinephrine
286
Tx rem sleep behavior disorder
Clozapine
287
Only drugs safe to use for antipsychotics in PD
Clozapine
| Quietiapine
288
What reflex still works in brain death?
Deep tendon
289
What kind of CT do you get for blood? For abscess or tumor?
noncontrast = blood
abscess or tumor = contrast
290
Narcolepsy pathophys
Get into REM sleep too fast
Sleep paralysis because wake up in REM sleep but still paralyzed
Orexin is decreased in narcolepsy (which usually helps in alertness)
291
Cataplexy pathophys
Happens a lot in narcolepsy
In REM sleep, you are paralyzed so you don't act out your dreams in REM. With narcolepsy, you get loss of muscle tone but you're still awake because body wants to get into REM sleep.
292
Rigidity vs. Spasticity --> how to tell difference?
Rigidity is LEAD PIPE rigidity. Still rigid for as long as you move it
Spasticity
- flexors in arm and extensors in leg worse off
- Clasp knife spasticity
293
Bulbar vs. pseudobulbar dysarthria
Bulbar = LMN lesion for CN 12
- can happen in ALS (classic example)
Pseudobulbar dysarthria = UMN lesion for CN 12
294
Density on non-contrast ct of blood, WM, bone, GM, CSF?
Bone > blood > WM, GM, CSF
295
How big to aneurysms have to be to detect on CT?
> 5mm
296
Tx meningioma
Resection
297
Where are astrocytomas in adults?
NOT IN POSTERIOR FOSSA
298
How to test for dysdiadochokinesia in cerebellar damage?
Rapid alternating movements (eg tap heel)
299
What is the frequency on EEG of awake person?
8-13 Hz
300
Lateral medullary syndrome
Wallenberg syndrome
Vertebral artery occlusion
```
DAMAGE:
CN5, 9, 10
Nucleus ambiguous
Lateral STT
Descending SNS fibers
```
PRESENTATION:
Ipsilateral ataxia
Ipsilateral horners
301
#1 cause of lobar hemorrhage in old person w/o HTN
Cerebral amyloid angiopathy
| - deposit beta amyloid in blood vessel
302
Mycotic aneurysm
Very small usually, can be missed on CT
Multiple aneurysms (not fungal etiology)
Occur w/ Gm +/- infections w/ low virulence
Mycotic aneurysms form over cerebral convexities w/ subacute bacterial endocarditis
Bleeding mostly in subarachnoid space
303
Charcot Bouchard aneurysms
Small
In chronic HTN
Mostly in lenticulostriate arteries
Dentate nucleus very prone to forming these
304
Hemangioblastomas are seen in?
Polycystic disease of kidney
| von Hippel LIndau
305
Leptomeningeal angiomas seen in?
Sturge Weber
306
Transcortical motor aphasia
Like Brocas....
But repetition is ok
307
Transcortical sensory aphasia
Like Wernickes...
But repetition is ok
308
Mesial temporal sclerosis
closely related to temporal lobe epilepsy, a type of partial (focal) epilepsy in which the seizure initiation point can be identified within the temporal lobe of the brain.
Mesial temporal sclerosis is the loss of neurons and scarring of the temporal lobe associated with certain brain injuries.
309
Jacksonian march
a phenomenon where simple partial seizure spread from distal part of limb to face ipsilaterally (on same side of body).
They involve a progression of the location of the seizure in the brain, which leads to a "march" of the motor presentation of symptoms
They are unique in that they travel through the primary motor cortex in succession, affecting the corresponding muscles, often beginning with the fingers. This is felt as a tingling sensation. It then affects the hand and moves on to more proximal areas on the same side of body.
310
Where are the reticular neurons of the ascending reticular activating system? What is it for?
Between thalamus AND midbrain
for wakefulness
311
Classification of concussion
grade 1 = no LOC, < 15 mins sx
grade 2 = no LOC, > 15 min sx
grade 3 = LOC
312
How long is the amnesia usually for grade 3 concussion?
Proportional to duration of unconsciousness
313
What imaging is more sensitive to diagnose acute ischemia?
MRI
314
Apraxia
loss of ability to execute or carry out learned purposeful movement
315
Agnosia
Loss of ability to recognize objects, persons, sounds, shapes, or smells
316
Time to tx brain ischemia w/ thrombolytics
Within 4.5 hrs
317
Common labfindings for SAH
Hyponatremia (increased ANP, or SIADH)
EKG - WT prolongation, T wave inversion, arrhythmias
318
#1 place for aneurysm in ADPKD
Anterior communicating
319
Best way to evaluate hyperacute onset of hemiparesis ~ 1 wk after SAH incident
Transcranial doppler
- measures velocity, finding caused by vasospasm
Angiography usually needed to confirm
320
HTN Hx + unilateral weakness + no changes on CT
Lacunar infarct
microatheroma and lipohyalinosis
Most commonly in internal capsule
321
#1, #2 muscles involved for myasthenia gravis
1 - extraocular muscles
2 - muscles of jaw (bulbar muscles)
CPK usually normal in myasthenia gravis
322
Myasthenia gravis vs. primary muscle problem - what lab value is helpful to distinguish?
CPK normal in MG
323
Myasthenia gravis vs. ALS
normal reflexes in MG
324
What test do you use to evaluate spinal stenosis?
MRI
325
Electrolyte risk with immobilization (eg paralysis)
Hypercalcemia
- possibly due to increased osteoclastic bone resorption
- tx w/ hydration + bisphosphonates
326
Medial medullary syndrome
- artery responsible
- syndrome
Vertebral A
ASA
Ipsilateral
paralysis + fasciculations of tongue
Contralateral
paralysis of arm and leg
loss of tactile, vibratory and position sense
Deviation of tongue to injured side
327
Wallenberg syndrome
- Injured artery
- syndrome
PICA
Vertebral A
Lateral medulla injury
Contralateral
:loss of pain and temperature sensation on body
Ipsilateral:
Loss of pain and temp of face
Horner
Dysphagia
Hoarseness
Deviation of uvula to contralateral side
Loss of gag reflex
cerebellar ataxia
Nystagmus
328
Internal carotid stroke
MCA stroke + amaurosis fugax
329
Proximal weakness of lambert eaton vs. polymyositis
Both have proximal weakness
CPK high in polymyositis
CPK normal in LE
Polymyositis - anti Jo1 and ANA
Lambert eaton - anti voltage gated Ca channels
330
Cavernous sinus thrombosis
- symptoms
- how do you tell the difference w/ orbital cellulitus?
CST happens b/c facial/ophthalmic venous sys is valveless
```
Headache
Binocular palsies
Periorbital edema
Hypoesthesia or hyperesthesia in V1/V2 distribution
CAN BECOME BILATERAL
```
Dx w/ magnetic resonance venography
Orbital cellulitus DOES NOT have headache, bilateral cranial nerve findings, or bilateral periorobital edema
331
Progressive supranuclear palsy
Degenerative condition of
- brainstem
- basal ganglia
- cerebellum
Like parkinson's:
- bradykinesia
- limb rigidity
- cognitive decline
NOT like parkinson's
- no tremor
- ophthalmoplegia
332
Herniated disc
Low back pain sciatica presentation
- radiates to thighs adn below knee
Pain worsens w/ sitting
+ straight leg test
333
Spinal stenosis
Pain is posture-dependent
Flexion of spine = widening of canal
Extension - narrowing of canal
Pain exacerbated by standing still, walking
Pain improved by sitting, lying down
Normal arterial pulses
Straight leg test negative
Dx w/ MRI
Tx laminectomy
334
MS HLA?
HLA DR2
50% are this
335
Neurons in tuberoinfundibular pathway secrete what?
Dopamine
336
What path is nigostriatal path?
Substantia nigra --> basal ganglia
337
Lateral pontine stroke
- artery responsible
- symptoms
AICA
Cerebellar:
Ataxia, nystagmus
```
Ipsilateral
deafness, tinnitus
loss of pain and temp on face
Horners
facial weakness, dysarthria
```
Contralateral
loss of pain and temp on body
Jaw weakness, dysarthria
Vertigo, nystagmus, ataxia
338
Medial pontine stroke
- artery responsible
- symptoms
Basilar A
Contralateral
hemiparesis – body and face; dysarthria
Ataxia (usually contralateral)
loss of touch, vibration and position sense
Ipsilateral
face weakness, dysarthria
horizontal gaze palsy, diplopia
Horners
INO
339
Midbrain stroke
- artery responsible
- symptoms
PCA
Contralateral hemiparesis – body and face; dysarthria
Ipsilateral CN III nerve palsy (down and out)
340
What tumors have periventricular structure predilection?
Tuberous sclerosis periventricular tubers
Primary brain lymphoma
341
Subclavian steal syndrome
Stenosis of subclavian artery proximal to vertebral artery (before it branches off)
Exercise of ipsilateral arm causes blood to flow in reverse down vertebral artery to fill subclavian distal to block
Decrease cerebral blood flow
BP in left arm less than right arm
Upper extremity claudication
Tx with surgical bypass
342
Astrocytomas in cerebellum are usually...
cystic
343
Absence vs complex partial seizure
Absence seizure does NOT have:
- post ictal state
Absence seizure CAN be stimulated by hyperventilation (vs not in complex partial) -- makes a 3Hz spike and wave pattern on normal background
344
Cluster headache
Unilateral retrorobital pain starting suddenly
Redness of ipsilateral eye
tearing, stuffed or runny nose
ipsilateral horners
345
Transtentorial (uncal) herniation signs
Compress:
Contralateral crus cerebri -----> ipsilateral hemiparesis
Ipsilateral oculomotor N -------> mydriasis, down and out, ptosis
Ipsilateral posterior cerebral A ------------> ischemic visual cortex ---------> contralateral homonymous hemianopia
Reticular formation -------------> Alt mental consciousness, coma
346
Can you expect fever in any intracranial hemorrhage?
YES!
347
Sudden onset of vertigo, vomitting, occipital headache in person w/ HTN
Cerebellar hemorrhage
348
What do you do to monitor respiratory fxn in person w/ Guillain Barre?
Serial vital capacity measurements
349
What is a pt in tonic clonic seizure at risk for?
Cortical laminar necrosis
- hallmark of prolonged seizures
- can lead to persistent neuro deficits and more seizures
Can see on MRI
350
Basal ganglia hemorrhage - neuro findings
Hemiplegia
Hemisensory loss
Homonymous hemianopia
gaze palsy
Stupor, coma
351
Cerebellum hemorrhage - neuro findings
Neck stiffness
Facial weakness
NO hemiparesis
Stupor/coma from brainstem herniation
352
Thalamus hemorrhage - neuro findings
Hemiparesis
Hemisensory loss
Upgaze palsy
Nonreactive miotic pupils
Eyes deviate Towards hemiparesis
353
Cerebral lobe hemorrhage - neuro findings
Can be assoc w/ seizures
Contralateral homonymous hemianopia (occipital)
Contralateral plegia/paresis (frontal)
Contralateral hemiparesis (parietal)
Eyes deviated AWAY from hemiparesis
354
Pons hemorrhage - neuro findings
Deep coma, total paralysis w/in minutes
Pinpoint reactive pupils
355
Hypertensive lacunar stroke mostly in...
Lacunar arteries
```
Putamen (#1)
Thalamus
Pons
Cerebellum (more HTN hemorrhage!!!!! vs stroke)
Cortex
```
356
What test can detect endogenous depression?
Dexamethasone suppression test
357
Dx alzheimer's disease
Made clinically by assessing:
MMSE
Neurophysch testing
2 or more of the following:
2 or more areas of cognitive deficits
Progressively worsening memory and other cognitive function
no disturbance of consciousness
onset after age 60
absence of other systemic or neurologic disorder causnig progressive cognitive defects
358
Duret hemorrhages
are small areas of bleeding in the ventral and paramedian parts of the upper brainstem, (midbrain and pons)
They are secondary to raised intracranial pressure with formation of a transtentorial pressure cone involving the cerebral peduncles (crus cerebri) and other midbrain structures caused by raised pressure above the tentorium
359
Diffuse axonal injury
is the result of traumatic shearing forces that occur when the head is rapidly accelerated or decelerated, as may occur in auto accidents, falls, and assaults
Can also be due to metabolic causes
Only edema will cause diffuse axonal injury (esp in metabolic)
360
Areas of brain most susceptible to hypoxic injury
Hippocampus
Purkinje cells of cerebellum
4th layer of cortex
361
Dystrophic calcification
Cells are dying and will uptake calcium
362
Primary generalized seizures
- will induce w/ sleep deprivation, hyperventillation
- --usually induce the seizures so you can figure out how much restriction to put on a kid's life (like in absence)
- runs in families
- starts in reticular activating system and spreads
- usually less than 30s (vs > 40s in partial)
- only motor activity impaired
363
When does teratogenicity dose of valproic acid start at?
750 mg
364
Is febrile seizure genetic?
Somewhat!
Always put these children w/ FH of febrile seizures on Tylenol to lower fever
20% Japanese can have febrile seizures
365
Do ppl who have acidosis seize?
No!
Moment you are aciddic, you will stop seizing
366
Resons for intraprenchymal hemorrhage
HTN
Trauma
Aneurysm/AVM
Malignancy, hemorrhage
367
Internal capsule - where are the motor fibers?
Genu - face and arm
| Posterior capsule - more leg
368
How can you tell the central sulcus on MRI?
Omega sign
369
#1 type seizure in adults
Complex partial
370
Which muscles are affected more in UMN lesions?
A pattern of weakness in the flexors (lower limbs) or extensors (upper limbs), is known as 'pyramidal weakness'
371
What does extensor posturing mean?
Lesion above red nucleus
372
Meralgia paraesthetica
aka Bernhardt-Roth syndrome
numbness or pain in the outer thigh not caused by injury to the thigh, but by injury to a nerve that extends from the thigh to the spinal column
involves only 1 nerve: lateral cutaneous nerve of thigh
The lateral femoral cutaneous nerve most often becomes injured by entrapment or compression where it passes between the upper front hip bone (ilium) and the inguinal ligament near the attachment at the anterior superior iliac spine
373
Complex Regional Pain Syndrome (CRPS), formerly Reflex Sympathetic Dystrophy (RSD)
Clinical features of CRPS have been found to be neurogenic inflammation, nociceptive sensitisation, vasomotor dysfunction, and maladaptive neuroplasticity
374
What is first sign of weakness on PE?
Pronator drift!
The very first sign is extensor digiti minimi sign
375
What are most common seizures 2/2 to EtOH?
Trauma-induced
Withdraw
376
What is the following typical of?
Occipital lobe hypometabolism
Medial temporal lobe atrophy and parietal hypometabolism
Atrophy of midbrain
Occipital lobe hypometabolism is most typical of DL
Medial temporal lobe atrophy and parietal hypometabolism are characteristic if AD
Atrophy of midbrain is characteristic of progressive supra nuclear palsy
377
Top 3 places for hypertension stroke
Basal ganglia
Cerebellum
Pons
378
Number 1 cause of sixth nerve palsy
Diabetes
379
What is first nerve affected by increased intracranial pressure?
CN 6 since it has the longest course.
380
Intracranial HTN symptoms
```
Diffuse HA worse in AM
N/V in AM
vision changes
Papilledema
CN deficits
Somnolenece
Confusion
Unsteadiness
Cushings reflex
```
Evaluate via CT or MRI
