1
Q
Spinal areas
A
2
Q
MS
A
CNS Location for dissemination in space: optic nerve, periventricular, juxtacortical/cortical, infratentorial, spinal cord
3
Q
ADEM
A
T cell mediated, demyelinating disease of the CNS
- Fever, headache, encelopathy (must have confusion).
- Can be assoc with + myelin oligodendrocyte glycoprotein (MOG) antibodies.
- diagnosis: Lumbar puncture: lymphocytic pleocytosis and elevated protein
Spinal and/or brain MRI (with and without contrast): typically multiple, bilateral lesions - Tx: high dose IV methylprednisone, and prednisone taper.
Can consider IVIG or PLEX
4
Q
Global developmental delay
A
significant delay in 2 or more domains
5
Q
Isolated dev delay
A
significant delay in one domain
most common is language/speech
6
Q
intellectual disability
A
> 5 years
limitations in intelligence affecting 1 of 3 adaptive domains - conceptual, social, practical
7
Q
Developmental delay workup
A
genetic testing +/- MRI
8
Q
Imaging findings in developmental delay
A
CNS malformations, white matter abnormalities, cerebral atrophy
9
Q
Weakness in infants
A
SMA
10
Q
Weakness in older children
A
DMD
11
Q
SMA
A
- AR, LMN, progressive weakness and atrophy
- diffuse, symmetric proximal muscle weakness LE > UE.
decr or Absent deep tendon reflexes - 5q-SMA - biallelic mutations or deletion on 5q13.2 > deficiency of survival motor neuron protein
Tx: Nusinersen - ASO splicing modifier, Risdiplam
Onasemnogene SMN1 gene replacement.
12
Q
DMD
A
- XLR, dystrophin DMD gene absent, dx prior to age 5, gross motor delay, proximal muscle weakness, hyper CK emia
- progressive weakness > loss of ambulation, respiratory insufficiency, cardiomyopathy.
- Tx: steroids
Becker is partially functional dystrophin leads to more mild phenotype.
13
Q
Physiologic jitters
A
jitters, triggered by stimuli and suppressible.
No autonomic changes, ocular deviation, etc, common in first days of life.
Jitters > stimulus induced, stops with restraint, no eye/autonomic changes. Seizure will be spontaneous, not suppressible and have changes
Assess for hypoglycemia, hypocalcemia, drug withdrawal and hypothermia
14
Q
Shuddering attack
A
rapid shiver of heaad and trunk > facial grimace
- usualy stop within first decade of life
- heightened emotion
15
Q
Breath holding spells
A
6-18 months up until 5
- cyanotic
- can consider iron
16
Q
Infant self stimulation
A
Interruptible, typically when bored or in a car seat
17
Q
Focal seizure in child
A
often prenatal/perinatal insult > doesn’t appear until a few months.
Rhythmic, sterotyped, non-interruptable, no trigger
18
Q
Stereotypies
A
Repetitive, rhythmic, voluntary in initiation
- early childhood, supressible
- triggered by excitement, stress, fatigue
19
Q
Jerking spells, clustered, sudden brief flexion, less responsive, loss of skills, less interactive
A
Infantile spasm
- severe epileptic encephalopathy
3-12 months
Triad: epielpetic spasms (brief axial contractions), hypsarrhythmia on EEG, developmental regression/delay
Sudden contractions of neck, trunk, limbs
clusters
neuroimaging and EEG. Assess for structural causes/tuberous sclerosis
tx: High dose steroids, Vigabatrin
20
Q
Tic disorders
A
repeated, intermittent movements/utterances
- blinking, grimacing, shoulder shrug, head jer, throat clearing
- briefly suppressible
- related to an urge
21
Q
Functional tic
A
Tik tok tic - females 15-25 years old
- explosive onset complex vocal and motor tics.
- inability to suppress, without urge
22
Q
Tic treatment
A
CBIT behavioral therapy
alpha agonists - guanfacine clonidine
23
Q
Pediatric migraine variants
A
Benign paroxysmal torticolis
BPV
cyclic vomiting syndrome
abdominal migraine
24
Q
BP torticollis
A
recurrent episodes of head tilt, vomiting, fussiness
- 6-8 months
- 1/4 have gross motor delay
25
BPV
recurrent vertigo
- loss of balance, expression of fear, diaphoresis, nausea, vomiting, photophobia, phonophobia
2-4 years
26
Cyclic vomiting
bouts of vomiting at regular intervals
4-7 hears old
triggers: excitement, stress, food products, lack of sleep
treat like migraine
27
Abdominal migraine
severe midline abdominal pain 1-72 hours, typical onset 4-7 years old
Acute: treat with ibuprofen/sumatriptan
Preventative: lke migraine, cyproheptadine, amitriptyline, propranolol
28
Harness placebo in pediatric migraine
magnesium, b vitamins as well as the other treatments
29
Multiple sclerosis
Acute exacerbations of MS are usually treated with high-dose glucocorticoids. Between exacerbations, patients may be treated with disease-modifying drugs (e.g., interferon beta, glatiramer acetate, natalizumab).
30
RAPD
relative afferent pupillary defect
Optic neuritis
31
Scotoma
Absence of visual field
32
Transverse myelitis
Associated with MS
asymmetric paraplegia
band like
unilateral sensory loss
bladder dysfunction
33
INO
A gaze abnormality localized to the medial longitudinal fasciculus (MLF) of the brainstem. Characterized by impaired horizontal eye movements with weak adduction of the affected eye and abduction nystagmus of the unaffected eye. Most cases (70%) are due to multiple sclerosis or stroke.
MLF helps with controlling conjugate horizontal movement (versions) of the eyeball and the adjustment of eye movement with a change in head position.
34
VF defecits
Unilateral vision field loss indicates a disorder of a structure anterior to the optic chiasm. Bilateral visual field loss indicates a pathology at or posterior to the optic chiasm.
35
Optic neuritis
Painful vision loss, central scotoma, color vision defecit (red desaturation), RAPD.
DDx: MS, NMO MOGAD, HSV, Lyme, HIV, syphillis, migraine, retinal disease
Tx: high dose steroids > can progress to IVIG later
36
MS
exacerbation - steroids
DMARDS
- Monoclonal antibodies (e.g., natalizumab, ocrelizumab) have the highest efficacy for reducing exacerbations, but lower efficacy medications (e.g., interferon beta, glatiramer acetate) often have fewer side effects. [7]
37
MS criteria
The McDonald Criteria for both DIT and DIS must both be met to confirm a diagnosis of MS: [7]
Dissemination in time (DIT): the appearance of new CNS lesions over time that can be confirmed clinically, with imaging, or with CSF analysis
Dissemination in space (DIS): the presence of lesions in different regions of the CNS that can be confirmed clinically or in MRI
Oligoclonal bands in the CSF but not in blood support diagnosis of MS.
38
MS spinal tracts
Dorsal column - pressure, vibration, fine touch, proprioception
Ascending lateral Spinothalamic tract - pain and temperature
Anterior spinolthalamic tract - crude touch and pressure
39
MS - natalizumab
An antibody against α4-integrin that decreases lymphocyte invasion of the CNS.
40
MS - Ocrelizumab
Antibodies against CD20; deplete B cells
41
PML
multifocal demyelination in the brain. Caused by reactivation of a latent JC virus in patients who are immunosuppressed (e.g., due to AIDS, hematologic malignancies, posttransplant).
42
LP indications
CNS infection, meningeal spread of cancer, inflammatory CNS Process
C/I - signs of increased intracranial pressure (n/v, positional headache), obstructive hydrocephalus
- bleed risk INR > 1.4 or platelets < 50
anti-platelet/anticoagulant but that varies
43
CSF
0-5 WBC
0-5 RBC
elevated protein can be due to infection, malignancy, inflammation, obstruction.
CSF glucose 0.5-0.8
LOW glucose > suggests malignancy, inflammation, infection (bacterial meningitis).
44
Biopsy brain lesion indications
deteriorating despite treatment, accessible active lesion
Focal mass lesion/seizure, abnormal CSF parameters, immunocompromise has higher yield
diffuse lesions, nontargeted smaller biopsy, pineal/periventricular, comatose > less yield
45
ADEM
T cell mediated, demyelinating disease
- Fever, headache, encephalopathy
- assoc with myelin oligodendrocyte glycoprotein (MOG) antibodies
- imaging: multifocal ring or open ring enhnacing lesions with edema, CSF Possible lymphocytic pleocytosis
Assoc with recent vaccination or URI
Tx: High dose IV Methylprednisolone with predisone taper, consider IVIG and plasmapheresis
46
Autoimmune encephilitis
BILATERAL, symmetric
vs HSV encephalitis (unilateral)
47
Spinal tracts and dessucation
48
Spinal tracts
49
Spinal tracts
50
Lesion DC
DC:
Lesion in spinal cord → Ipsilateral sensory loss below the lesion.
Lesion above medulla (e.g., brainstem or cortex) → Contralateral sensory loss.
51
Lesion ST
Pathway:
Sensory fibers enter the spinal cord and synapse.
Cross to the contralateral side within 1–2 segments.
Then ascend in the contralateral ST tract.
Result: Lesion in spinal cord → Contralateral loss of pain and temperature starting ~1–2 levels below the lesion.
Example: A right-sided ST lesion at T8 → loss of pain and temperature on the left side, starting around T10.
52
Lesions table
53
CST, for MS
Right internal capsule stroke
→ Affects CST above decussation
→ Left-sided hemiparesis
Right lateral CST lesion at T10 (e.g., MS plaque)
→ Affects already-crossed fibers
→ Right-sided lower limb weakness
MS:
MS lesions in the spinal cord (especially cervical and thoracic regions) often involve lateral CST.
Results in ipsilateral upper motor neuron signs: Spastic weakness, Increased reflexes, Positive Babinski sign
54
RAS
The RAS is in the brainstem’s reticular formation and is essential for consciousness, arousal, and autonomic control. Damage impairs wakefulness, while proper function allows selective attention and sleep–wake regulation.
55
Glasgow coma scale
Eye opening response (no response > pain > speech > spontaneous (1-4))
Verbal response (no response > incomprehensible > inappropriate words > confused > oriented (1-5))
Motor response (No response > abnormal extension > abnormal flexion > flexion withdrawal from pain > localizes to pain > obeys commands 1-6))
Total score 15
Comatose 8 or less
3 or less totally unresponsive
56
CN 3
Uncal herniation
CN 3 blown
Eye is DOWN and OUT
Uncal herniation compresses CN III against the tentorial edge.
Parasympathetic fibers → pupillary dilation (“blown pupil”).
Motor fibers → paralysis of most eye muscles → “down and out” deviation + ptosis.
57
CN V
corneal stimulation
58
CN 9 and 10
Pharyngeal gag reflex
59
4 score scale for comatose patients
The FOUR score evaluates coma using Eye, Motor, Brainstem reflexes, and Respiration (0–16). It is particularly useful in ICU and intubated patients, providing a broader neurological assessment than the GCS.
https://next.amboss.com/us/article/7704Mh?utm_medium=chatgpt-plugin&utm_source=chatgpt#Z352e8a2ae09f6e24d7fead6a665ff3bb
60
NIHSS stroke scale
Level of consciousness, orientation, and ability to follow commands
Cranial nerve palsies: e.g., visual impairment, facial droop
Motor and sensory deficits or neglect
Language impairment
Coordination
Scores between are assigned for each category are combined and totals can range from 0 (no impairment) to 42 (most severe).
61
CN 3 palsy > ischemic stroke
Posterior cerebral artery
62
Refractory status epilepticus
In the context of benzodiazepine-refractory convulsive status epilepticus, the anticonvulsant drugs levetiracetam, fosphenytoin, and valproate each led to seizure cessation and improved alertness
63
pupillary light reflex
bilateral edinger westphal nuclei
64
Cerebral perfusion pressure
CPP = MAP - ICP
60-80
ICP is 18-20
65
Monro Kelli doctrine
The Monro–Kellie formula states that brain tissue, blood, and CSF volumes are in a fixed cranial space. If one increases, another must decrease; otherwise, ICP rises dangerously.
66
Mgmt of increased ICP
Monitor with EVD - diagnostic and therapeutic
- HOB > 30, keep Pc02 above 28, CPP > 60
- ropofol, versed, ketamine, fentanyl, dilaudid, oxycodone
- CSF drainage > decompression
- Rocuronium
-hypothermia
- pentobarbital
https://next.amboss.com/us/article/HL0K_g?utm_medium=chatgpt-plugin&utm_source=chatgpt#Zdd278c8a16c5b553e4c97d978f4d2f0c
67
Indications for surgical management of acute neurological disease: mass lesion, ICH, cord compression
Volume > 50 cc
ICP > 22
Mass effect - downward herniation/crowding of basal cisterns
> 5mm midline shift
68
Sedative agents on ICP
69
Subdural hematoma
Crescent
venous
bridging veins
crosses suture lines
70
Epidural hematoma
Middle meningeal
Lemon
71
Aneurysm SAH
Traumatic SAH
Starfish sign
Blood in folds
72
Intracerebral hemorrhage
accumulation of blood with edema around it
Basal ganglia, pons, cerebellum common location for a hypertensive bleed.
73
Diffuse axonal injury
Grade 1 > 3
peripheral to midline to brainstem
High acceleration/deceleration which leads to injury to white matter.
74
GCS
13-15 mild
9-12 moderate
3-8 Severe
75
Hyperosmolar (mannitol/hypertonic saline)
Hyperosmolar therapy (mannitol or hypertonic saline) is used in TBI and other neurologic emergencies to lower ICP, reduce cerebral edema, and maintain perfusion. It is a temporary, supportive measure while definitive management is arranged.
76
Normal ICP
0-15
77
Imaging of intracranial pathology can show
basal cisterns compressed
midline shift
78
ICP
Treatment of elevated ICP starts with conservative positioning and sedation, escalates to hyperosmolar therapy and short-term hyperventilation, and if refractory, requires surgical measures (CSF drainage, decompressive craniectomy) or barbiturate coma
79
Mannitol and HTS side effects
Mannitol: hypovolemia and osmotic nephropathy
HTS: acute renal injury. peripheral vascular injury, metabolic acidosis w/ hypokalemia, pulmonary edema
80
Management of ICH
BP: keep SBP around 140
TBI keep BP > 110 to help with CPP
IV infusions - nicardipine
81
Anticoagulation reversal in setting of ICH
82
Spinal cord syndromes
Anterior cord - bilateral motor/pain and temperature
Posterior cord - bilateral proprioception
Central cord - Bilateral upper extremity weakness, pain/temperature
Brown sequard - unilateral weakness, contralateral pain/temperature
- “Same side motor + vibration, opposite side pain/temp.”
83
SCI
A > D, A is the worst, no motor or sensor yfunction preserved in the sacral segment
84
SCI
CT first line because of speed and availability
MRI gold standard, provides info on ligamentous injury, cord edema, hematoma, prognostic value
MAP Push (85-90) for first 7 days
Early cord decompression < 24 hours
85
Spinal shock
Hyperacute, reversible impairment in spinal cord function
Transient loss of everything below injury
Improve
Not really shock
first few hours
86
Neurogenic shock
Actual shock, especially happens in people with complete lesions above T6, have lost sympathetic tone > unopposed PS tone
Subacute phase (first 2 weeks)
Bradycardia and hypotension
Wide pulse pressure due to peripheral vasodilation
Tx: pressors, inotropes (NE, Epi), Anticholinergics (antropine/glycopyrrolate) to counteract the unopposed vagal tone.
87
Sedatives on ICP
Barbiturates, propofol, etomidate: most effective for reducing ICP directly.
Benzodiazepines, opioids, dexmedetomidine: supportive sedation, reduce agitation/pain, but less direct ICP-lowering.
Ketamine: safe in TBI, useful in hypotension.
Always balance ICP control with CPP maintenance (avoid hypotension).
88
Empiric meningitis coverage Age < 50 without RF
Vancomycin, Acyclovir, ceftriaxone
Covers: S. pneumoniae, N. meningitidis, ± resistant strains
89
Empiric meningitis coverage > 50, or RF
Adults > 50 or with risk factors (e.g., immunocompromised, alcoholism, pregnancy)
Vancomycin
PLUS ampicillin (Listeria coverage)
PLUS ceftriaxone or cefotaxime
90
Antibiotic coverage for meningitis
CSF in bacterial meningitis: neutrophils, high protein, low glucose, ↑ opening pressure.
CSF in viral meningitis: lymphocytes, normal glucose, normal/mildly ↑ protein.
Empiric therapy: always vancomycin + ceftriaxone, with ampicillin added for Listeria coverage in patients >50 or immunocompromised. Broaden to cover Pseudomonas in post-surgical cases.
91
HSV encephalitis (different from meningiti)
90% HSV1
Amygdala and hippocampus affected
Headache, fever, confusion, focal deficit, seizures
Tx: Acyclovir 14-21 days
Secondary autoimmune encephalitis complication (NMDAR)
92
VZV neuro disease
- can cause similar encephiltis to HSV
- Ramsey Hunt syndrome - reactivation of geniculate ganglion of CN 7 (fcacial) >> vasculitis, vasculopathy (pediatric stroke), myelitis, post herpetic neuralgia (shingles)
93
Congenital CMV
microcephaly, sensorineural hearing loss, seizures, intellectual disability
- calcifications, ventriculomegaly, reduced sulci, cerebellar hypoplasia
94
Other herpes viruses on immunocoprised hosts
EBV - recurrent encephilitis
CMV - retinitis, myelitis, polyradiculitis
HHV6 - very rare, seen in people with profound T cell depletion
95
Powassan virus
Positive, single strand RNA enveloped FLAVIVIRUS
- tick borne encephalitis
ixodes scapularis (deer tick) bites people, whereas ixodes cookei and marxi rarely do
- NE, great lakes, SE canada
- Peak spring to fall
- testing is serologic/abx on serum or CSF
- encourage tick bite prevention
- 1-4 week incubation
- CSF pleucytosis (lymphocytic), normal glucose
- widespread lesions and cerebral edema
-Fever, headache, nausea, vomiting
Altered mental status, confusion, seizures, focal neurologic deficits
Severe cases → meningoencephalitis with long-term sequelae (memory loss, motor dysfunction).
blood transfusion case - don't donate blood for at least 4 months
96
Flaviviruses
Flaviviruses are +ssRNA, enveloped, icosahedral viruses that often spread via arthropods (mosquitoes, ticks) and cause diseases ranging from encephalitis (Powassan, TBEV, West Nile) to systemic viral illnesses (dengue, yellow fever, Zika). HCV is a notable exception, spread parenterally.
97
Enterovirus D68 or Acute flaccid myelitis
- poliomyelitis not caused by poliovirus
- Spinal cord gray matter > acute flaccid paralysis
- Associated with enterovirus (D68)
- Sudden onset after viral prodrome
- Acute asymmetric limb weakness (often upper extremities). Reduced or absent reflexes (LMN pattern). Cranial nerve involvement possible (e.g., facial weakness, dysphagia). Sensation usually preserved (helps distinguish from transverse myelitis).
EV D68 - respiratory droplet spread
- cough, sneezing, rhinorrhea, myalgias, severe asthma exacerbation
AFM with EV D68
- acute flaccid weakness, non enhancing poliomyelitis on MRI, dorsal pons non enhancing lesion, ventral root enhancement.
Diagnosing: clinical and radiographic
EV: respiratory support, AFM: IVIG may help, corticosteroids may help
Avoid touching face, wash hands
98
AFM vs GBS
GBS → Peripheral, symmetric, ascending, areflexic.
TM → Central cord lesion, sensory level, UMN signs develop.
AFM → Anterior horn gray matter, asymmetric flaccid paralysis, sensation spared.
If you see asymmetric sudden limb weakness after a viral illness with normal sensation and MRI gray matter lesions, think AFM.
If you see symmetric ascending weakness with sensory/autonomic features and CSF protein elevation without WBCs, think GBS.
99
AFM vs TM
100
Nipah
- cycle between animals/humans
- Negative SS RNA virus
- fruit batfox
- direct contact with pteroptus body fluid
- contact with infected pigs
- human to human
Incubation 4-14 days
- severe flu lke illness > encephalitis > CNS vasculitis
- wash hands, avoid bat areas
101
Hendra virus
similar to Nipah but no human to human
- horse case
- Incubation 9-16 days
- Severe flu like illness then encephalitis
- wash ahnds, avoid bat areas, avoid sick horses, vaccinate horses
102
Nipah and hendra
PCR diagnosis
IgM or IgG ELISA
Supportive
Ribavirin and remdesivir
103
Chikayunga virus
Positive, enceloped, RNA virus
mosquito bourne
daytime
can also be through blood transfusion, maternal-fetal.
3-7 days, abrupt high fever, widespread maculopapular rash, severe JOINT pain
Neuro: HA, meningoencephalitis, progress to GBS
Dx - HIGH VIREMIA - NAAT/RT-PCR
No specific treatment
GBS - IVIG or PLEX
Mosquito repellent precautions
high viremia means infected people avoid bites too
ChikV vaccines
104
Herniations
Subfalcine → ACA compression → leg weakness.
Central transtentorial → midbrain/pons → coma + posturing.
Uncal → CN III palsy + hemiparesis, ± Kernohan’s notch (ipsilateral weakness).
Tonsillar → medullary compression → death.
ASC Midterm
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