CNS Flashcards
Describe cervical myelopathy signs
Myelopathy (UMN signs below the level of lesion) - due to cord compression
Radiculopathy (LMN signs at level of lesion) - due to nerve root compression
Elevated 14-3-3 protein
Creutzfeldt-Jakob disease
Nonspecific marker of neuronal cell death
Anti-Hu antibody
Paraneoplastic syndrome (SCLC, patients who smoke)
Anti-NMDA receptor antibody
Anti-NMDA receptor encephalitis - associated with ovarian teratomas
Median age 21, 4x greater in women
Low alpha-synuclein; round, eosinophilic inclusions of it
Parkinson dementia if in basal ganglia/midbrain, dementia with Lewy bodies if in cerebral cortex
Low hypocretin/orexin
Narcolepsy type 1
Creutzfeldt-Jakob disease - signs
- Rapidly progressive dementia
- Myoclonus provoked by startle
- Mood symptoms (e.g. depression)
- Hypersomnia
CSF noninflammatory, contains elevated 14-3-3 protein (marker of neuronal cell death), elevated tau
Test for abnormally folded proteins using real-time quaking induced conversion (RT-QuIC) assay - mix abnormally folded proteins with normally folded proteins and watch for increased fluorescence following conformational changes in normal proteins
Most common sites of hypertensive hemorrhage in descending order
- Basal ganglia (putamen)
- Cerebellar nuclei
- Thalamus
- Pons
- Cerebral cortex
Cerebellar hemorrhage - signs
- Occipital headache (may radiate to neck/shoulders
- Neck stiffness (extension of blood into 4th ventricle)
- Nausea/vomiting
- Nystagmus
- Ipsilateral hemiataxia of trunk (vermis) and/or limbs (hemispheres) as corticopontocerebellar fibers decussate twice
What hemorrhage and damage would cause pinpoint pupils?
Large pontine hemorrhage - damage to descending sympathetic fibers
What is a useful screening test for hemineglect?
Drawing a clock - requires both sensory and motor components
Malignant hemispheric infarction
When an ischemic stroke causes:
1. Massive cerebral edema - from enthothelial dysfunction and breakdown of BBB –> mass effect, ICP, brain herniation
2. And/or hemorrhagic transformation - from blood extravasation from injured cerebral vessels into brain parenchyma (larger infarcts –> greater risk)
What is the difference between viral and fungal (Cryptococcus)/tuberculous meningitis on CSF?
Both have slightly elevated protein and lymphocytic predominance, but viral has normal glucose
Diagnostic confirmation of Cryptococcus
After CSF, India ink stain or polysaccharide antigen testing
How does Botulinum toxin work?
Cleaves SNARE proteins, preventing ACh release from neuron at neuromuscular junction
Botulinum vs myasthenia gravis
Myasthenia gravis spares pupillary function, autonomic dysfunction is less prominent, and symptom progression typically less rapid
Both are descending weakness
Cauda equina vertebral levels and corresponding effect
L2-sacrum + coccygeal nerve:
S1-S2: absence ankle reflex
S2-S4: saddle anesthesia
S3-S5: bowel, bladder, or sexual dysfunction (e.g. urinary straining)
Can have asymmetric lower extremity weakness/sensory loss
Conus medullaris vs cauda equina
- Conus medullaris is L1-L2
- Severe pain limited to lower back (vs radiating into leg)
- Numbness symmetric and limited to perianal area
- Weakness symmetric
- UMN findings such as hyperreflexia (vs absent ankle reflex)
Who is at increased risk of internal carotid artery dissection?
- Connective tissue disease (Ehlers-Danlos)
- Smoker
- Uncontrolled HTN
- Oral contraceptives
Why does internal carotid artery dissection only present with partial Horner syndrome?
Ptosis and miosis but not anhidrosis - sympathetic fibers for facial diaphoresis travel along the external carotid artery
Dementia with Lewy bodies
Dementia plus >=2 of the following:
1. Visual hallucinations
2. Parkinsonism
3. Fluctuating cognition
4. REM sleep behavior disorder
Blurred optic disc margins
Papilledema
Jervell and Lange-Nielsen syndrome - presentation
Congenital long QT syndrome
Inheritable sensorineural hearing loss due to mutations affecting endolymph production
Presents in childhood with profound bilateral deafness and episodes of arrhythmia-induced syncope during stress
What causes relative afferent pupillary defect?
Optic nerve injury - most often caused by indirect high-intensity force to orbit and transmission of shearing forces
Vision improves with conservative management in half of patient
Surgical decompression may be required
Loss of light perception is associated with worse prognosis
Abnormal red reflex is seen with what traumatic injury?
Vitreous hemorrhage from head trauma –> decreased visual acuity, relative afferent pupillary defect, and abnormal red reflex
Peripheral vertigo nystagmus
- Never purely vertical
- Inhibited by gaze fixation
- Fatigable (<1 min duration)
- Latency period (2-40 seconds)
How are patients predisposed to acute-closure glaucoma?
Lens is located more forward against the iris, impairing flow of aqueous humor through pupil into anterior chamber, increasing IOP
Women >40 yo, Asians and Inuits, farsightedness
Exacerbation can be due to pupillary dilation:
1. Anticholinergics (e.g. tolterodine)
2. Sympathomimetics
3. Low ambient light
Sulfonamides can trigger ACG due to swelling of structures in posterior chamber
Tourette syndrome - treatment
Counseling unless severe, disabling, or distressing:
Behavioral therapy - habit reversal training
Pharmacological:
1. Vesicular monoamine transporter 2 (VMAT2) inhibitors (e.g. tetrabenazine)
2. Antipsychotic
3. Alpha-agonist (e.g. guanfacine, clonidine) - helpful when ADHD or behavioral issues are also present
Central vertigo from posterior circulation ischemia - signs
- Limb ataxia or dysmetria (e.g. abnormal finger-to-nose or heel-to-shin)
- Postural instability - falling to side of lesion
- Headache - due to trigeminal innervation of much of cerebral arterial vasculature
Subarachnoid hemorrhage - timeline of complications
First day - rebleeding with rapid neurologic deterioration (urgent repair by coiling or clipping will reduce risk of rebleeding)
3-14 days - delayed cerebral ischemia due to vasospasm (caused by vasogenic metabolites from degraded blood) presenting with stroke-like symptoms - reduce risk via nimodipine
2 weeks - communicating hydrocephalus due to degraded blood products interfering with CSF reabsorption
Idiopathic intracranial hypertension - epi, signs, diag
Obese women of childbearing age
Excessive weight gain
Positional headaches worse when flat
Pulsatile tinnitus (due to increased vascular pulsations)
Blurry vision (due to increased pressure on optic nerves)
MRI often with MR venography to rule out cerebral vein thrombosis
Lumbar puncture reveals elevated opening pressure (>250)
Guillian-Barre syndrome
After acute infection (e.g. GI, resp, HIV)
Ascending motor deficits, hyporeflexia, back pain
Sensation classically unaffected (although paresthesias are common)
Dx with clinical, CSF analysis, and nerve conduction studies showing demyelination
CSF - albuminocytologic dissociation (increased total protein, normal CSF cell count)
Guillain-Barre treatment
IVIG or plasmapheresis
Most common artery causing Wernicke (receptive) aphasia
Inferior division of MCA - dominant temporal lobe
Also affects:
1. Right upper visual field
2. Somatosensory association cortex (processing of somatosensory information)
3. Lateral parietal and temporal heteromodal association cortex (integration of different sensory modalities - visual, somatosensory, etc)
These thus lead to difficulty using objects
Most common artery causing Broca (expressive) aphasia
Superior division of MCA - dominant frontal lobe
Also affects:
Contralateral hemiparesis
Nondominant frontal lobe lesion affects…
how a patient conveys emotion through speech (motor aprosodia)
Contralateral weakness due to impact on primary motor cortex
Nondominant temporal lobe lesion affects…
- Ability to comprehend emotional gestures (sensory aprosodia)
- Contralateral homonymous quadrantanopia due to impact on optic radiations
Dominant parietal lobe lesion affects…
- Contralateral sensory loss
- Contralateral inferior homonymous quadrantanopia - involvement of superior optic radiations
Nondominant parietal lobe lesion affects…
Anosognosia - denial of one’s disabilities
Apraxia - inability to carry out learned, purposeful movements
Spinal muscular atrophy affects which cells?
Anterior horn cells
As do poliomyelitis and ALS
Organophosphate poisoning - signs
Weakness accompanied by multisystem cholinergic symptoms
Diseases of neuromuscular junction
- Botulism
- Organophosphate poisoning
- Myasthenia gravis (improves with rest)
- Lambert-Eaton
Labyrinthitis
Inflammation of labyrinth and vestibular nerve –> acute-onset hearing loss and vertigo, +/- nausea/vomiting
If no hearing loss, it is called vestibular neuritis
Usually follows viral syndrome, may last few days
Main manifestation of vestibular schwannoma
Sensorineural hearing loss
Sometimes imbalance and tinnitus
True vertigo not typical because slow growth allows for development of compensation
Foramen magnum meningioma - signs
- Compression of brainstem at level of medulla - hypoglossal nerve dysfunction (tongue deviates toward side of lesion between strong part pushes weak side); bilateral corticospinal tracts run through medulla –> UMN signs (Babinski, spastic gait)
- Impaired CSF outflow –> increased ICP
Time course of following strokes:
Cardiogenic emboli
Flow limitation or embolization from carotid artery atherosclerosis
Lacunar stroke
Hemorrhagic
Cardiogenic emboli: multiple infarcts cause patchy neurologic findings - onset abrupt and maximal at start
Flow limitation or embolization from carotid: ipsilateral ocular and/or cerebral hemisphere ischemia - cerebral edema over hours-days, not rapid
Lacunar stroke: quite small, severe focal symptoms depending on area, not global
Hemorrhagic: minutes-hours, initially focal but soon ICP –> decreased consciousness
Dandy-Walker syndrome
Congenital atresia of foramina of Luschka and Magendie –> prevents CSF outflow into subarachnoid space –> hydrocephalus
Flattened gyri and sulci indicate…
Generalized cerebral edema due to increased hydrostatic pressure of hydrocephalus, allowing CSF to flow to parenchyma
Atypical Parkinsonian disorders (Parkinson-plus)
- Multiple system atrophy
- Progressive supranuclear palsy
- Corticobasal degeneration
Wide-based gait
Cerebellar or sensory ataxia
1. Cerebellar - stumbling/lurching ipsilaterally
2. Sensory - high-stepping, stamping gait; worsens in dark
Narrow-based, scissoring gait
Bilateral leg weakness with spasticity from UMN lesion (e.g. cerebral palsy, spinal cord injury)
Waddling gait
Myopathic weakness - proximal more than distal usually
Weakness of pelvic girdle muscles –> excessive pelvic tilt that shifts with each step
Putaminal hemorrhage
Involves adjacent internal capsule:
1. Contralateral hemiparesis and hemianesthesia (corticospinal and somatosensory fibers in posterior limb)
2, Conjugate gaze deviation toward side of lesion (damage of frontal eye efferents in anterior limb)
Brainstem stroke
Contralateral motor deficits with ipsilateral cranial nerve deficits (i.e. crossed signs)
Medulla involvement: tongue deviation, contralateral paralysis, contralateral loss of position sense (dorsal column medial lemniscus)
Saccular aneurysms vs Charcot-Bouchard
Saccular - spontaneous subarachnoid hemorrhage - severe headache with maximal intensity within hour + meningeal irritation
Charcot-Bouchard - Intraparenchymal hemorrhage of small penetrating arteries (same places as lacunar infarcts)
Elevated ICP tends to cause what eye palsy?
Bilateral abducens nerve palsies - long intracranial course makes it vulnerable to compression or stretching at skull base
Parinaud syndrome
Seen with pinealoma
- Limitation of upward gaze
- Light-near dissociation (pupillary light reaction impaired while the near reaction (accommodation) remains intact)
- Eyelid retraction