Exam 3 Flashcards
Which portion of the neurological history is unique?
Handedness
The purpose of the HPI is to
Organize the history details into a chronological fashion
HPI parts
Diagnostic process
Where is the lesion
Localization
What is the lesion/disease
Neurological findings by level
Different pathology timelines
Disease specific findings
Helps with the what
Specific signs
Historical symptoms
Constellation of findings
Which tuning fork can be used for vibration or hearing?
256 Hz
Papilledema should raise concern of
IIH
Optic nerve inflammation
Testing CN I
Fragrant smell
Affected in factual trauma, ENT infections, diseases affecting telencephalon
Testing CN II
Visual card
Each eye tested separately
Abscess visual acuity and visual field
Testing CN III, IV, VI
Testing CN V
Facial sensation, jaw closure, tongue sensation of ant. 2/3
Sensory exam of face
Test muscle tone and strength of masseter muscle through palpation
Use cotton swab to test anterior tongue
Testing CN VII, XI
Facial muscle movements
Taste reception of ant 2/3 of tongue
SCM and trapezius strength
Testing CN IX, X, XII
Soft palate movements (IX, X)
Pharynx sensation (IX)
Tongue movements (XII)
Opens mouth and say aaaah, Test gag reflex
Stick out tongue and move side to side
Push tongue against cheeks
Testing CN VIII
Hearing and balance
CALFRAST (CALibrated Finger Rub Auditory Screening Test)
MRC scale
Ankle reflex
S 1/2
Patellar reflex
L3, L4
Biceps reflex
C5/6
Triceps reflex
C 7/8
Deep tendon reflex grading
Interpretation of Deep Tendon Reflexes
Positive frontal signs in anyone older than 1 year is a sign of
Frontal lobe dysfunction
Sensory deficits are caused by lesions
Along the neuroaxis from sensory cortex to peripheral nerves
Light touch tests the _ pathway
Spinothalamic and DCML
Sharp touch tests the _ pathway
Spinothalamic
Temperature sensation tests the _ pathway
Spinothalamic
Vibration sense tests the _ pathway
DCML
Join position tests the _ pathway
DCML
Allodynia
Frank pain from touch
Parasthesia or Dysthesia
Abnormal quality such as burning, tingling or uncomfortable sensation
Transverse cord lesion causes
Trauma
Tumor
MS
transverse myelitis
Small central cord lesion causes
Bilateral cape-like distribution of pain and temp
Large central cord lesion
Widespread deficits
Upper extremities more affected with sacral sparing
Common causes of central cord syndrome
Trauma
Non traumatic and post traumatic syringomyelia
Spinal cord tumors
Anterior cord lesion causes
Trauma
MS
Anterior spinal artery infarct
anterior cord syndrome
Damage to anterolateral pathway causes loss of pain and temp below the level of lesion
Posterior cord syndrome causes
Vitamin B12 defiency
Tertiary syphilis: “tabes dorsalis”
Loss associated with posterior cord syndrome
Loss of vibration and position sense below the level of the lesion
Hemicord lesion causes
Trauma
MS
Lateral compression from tumors
Sensory deficit in Dermatomal distribution indicates
Spinal nerve lesion
A sensory deficit in Dermatomal distribution is causes by
Herniated disc
Spinal arthritis
Tumors or cysts
An isolated peripheral distribution of sensory deficit is associated with
A peripheral nerve lesion (i.e carpal tunnel)
symmetric distal distribution of sensory deficit indicates
Polyneuropathy or peripheral polyneuropathy
MOST COMMON CAUSE OF SENSORY ABNORMALITY
Common causes of peripheral polyneuropathy
Diabetes mellitus
Strokes are either _ or _
Ischemic or hemorrhagic
What is the leading cause of serious long-term disability
Stroke
What is the most common type of stroke
Ischemic
What is the single biggest risk factor causing stroke
High blood pressure
Behavior risk factors leading to stroke
Smoking
Sedentary lifestyle
Unhealthy diet
Non-traditional stoke risk factors
Sleep apnea
Signs and symptoms of a stroke
Sudden onset of symptoms of vascular origin
Severe headache
Confusion
Trouble speaking
Numbness
Trouble walking
Weakness
Lack of balance
Difficulty understanding
Dizziness
Vision changes
Anterior circulation
Ophthalmic artery
Posterior communicating artery
Anterior chorodial artery
Anterior cerebral artery
Middle cerebral artery
Posterior circulation stroke
Vertebral artery
Basilar artery
Posterior cerebral artery
Anterior cerebral artery infarct presentation
contralateral weakness (leg more than arm)
Motor hemineglect
Transcortical motor aphasia
Behavioral disturbance
Deep ACA stroke symptoms
Sphincter dysfunction
Mutism, anterograde amnesia
Corpus callous involvement: alien limb syndrome
Acute complete middle cerebral artery infarct presentation
M1 occlusion
Ipsilateral conjugated eye and head deviation
Hemianopsia
Contralateral hemiparesis
Contralateral hemi hypoesthesia
Cognitive signs always present
Right hemisphere- multimodal neglect
Left hemisphere- aphasia and apraxia
Superior division of MCA presentation
Contralateral hemiparesis (arm>leg)
Transient Ipsilateral eye deviation
Partial contralateral sensory loss
Visual fields impaired
Dominant: Broca’s aphasia or mutism
Non-dominant: confusion, neglect, inattention
Stroke of the inferior division of MCA presentation
M2 inferior division
Contralateral homonymous hemianopsia
Upper quandrantanopia
Mild arm and leg weakness
Dominant: Wernicke’s aphasia
Non dominant: neglect, confusion, dyspraxia
Anterior Choroidal Artery stroke presentation
Pure motor or sensory motor syndrome
Contralateral hemiparesis, hemihyperstesia and upper quadrant without cognitive disturbances
Similar to MCA but not cerebral involvement
Posterior circulation stroke syndrome
Midbrain syndrome
Weber- Ipsilateral paresis of addiction and vertical gaze palsy, contralateral weakness
Claude- oculomotor paresis, contralateral ataxia and tremor
Parinaud- impaired vertical gaze, loss of pupillary response
Posterior cerebral artery stroke presentation
Hemianopsia
Motor symptoms infrequent
Thalamic involvement
bilateral PCA infarcts: Balint syndrome
Clues to posterior circulation syndromes
Diplopia, tilt of vision, vertigo, drunken gate, hiccups, bilateral or crossed motor or sensory symptoms, decreased consciousness and amnesia
Wallenberg’s (dorsolateral medullary syndrome)
Vertebral and PICA infarct
Ipsilateral pain and temp facial deficit
Contralateral pain and temp loss in arm/leg
Dysphasia, dysphasia
Servere nausea, vomiting nystagmus
Ipsilateral Horners
Medial medullary syndrome (Dejerine)
Contralateral hemiparesis
Ipsilateral tongue weakness
Contralateral impaired proprioception and discriminative sensation with preserves pain and temp sensation
Basilar artery infarct
Locked in syndrome (quadriplegia, bilateral facial palsy, horizontal gaze palsy)
Midbrain syndromes
Weber
Claude
Parinaud
Weber syndrome (unilateral ventral)
Ipsilateral paresis of adduction and vertical gaze palsy
Contralateral weakness of the body
Claude syndrome (unilateral tegmental)
Oculomotor paresis
Contralateral ataxia and paresis
Parinaud syndrome (dorsal midbrain)
Impaired vertical gaze
Loss of pupillary response
Posterior cerebral artery stroke
Hemianopsia
Motor symptoms not common
Thalamic involvement
Etiology is emboli
Bilateral infarct: balint syndrome (asilmultagnisia, ocular apraxia, optic ataxia)
Lacunar stroke syndrome
Small subcortical infarct
Pure motor hemiparesis
Pure sensory stroke
Sensory-motor stroke
Dysarthria-clumsy hand syndrome
Ataxic hemiparesis
Most common type of Lacunar stroke
Pure motor hemiparesis
Watershed stroke syndromes
Involve junction of distal regions with two arterial syndromes
Intracerebral hemorrhage
Bleeding into brain parenchyma
Sudden onset focal deficits (contralateral weakness, headache, vomiting, decreased consciousness)
Caused by hypertension
Subarachnoid hemorrhage
Commonly caused by aneurysm rupture
Severe headache
Worst headache of life
Microscopic features of acute cerebral ischemia
Eosinophilic neuronal necrosis
Microscopic features of subacute cerebral ischemia
Reactive astrocytes, macrophages
Microscopic features of remote cerebral ischemia
Cavity
Which neurons are most susceptible to cerebral hypoxia/ischemia
Pyramidal neurons in hippocampal CA1
Cerebellar Purkinje cells
Pyramidal neurons in cerebral cortical layer III/V
Border zone/watershed areas also susceptible
Intraparenchymal hemorrhage
Results from hypertension
Basal ganglia, thalamus, and pons affected
Complications: cerebral edema and herniation
Subarachnoid hemorrhage
Most commonly caused by trauma
Also caused by ruptured Saccular aneurysm
Sudden headache/stiff neck
bloody CSF
High morbidity and mortality
Vascular malformations
Atriovenous malformations (high blood flow)
Cavernous malformation (low flow)
Epidural traumatic vascular injury
Arterial blood
Middle meninges artery laceration secondary to skull fracture
Subdural vascular injury
Venous blood
Tearing of bridging veins
May become chronic
Most common spread of infection to the brain
Hematogenous
Acute bacterial meningitis findings
High neutrophil, high protein, low glucose, bacteria on gram stain/culture
Symptoms of bacterial meningitis
High fever
Headache
Stiff neck
Confusion
Skin rash
Brain abcess
Viral meningoencephalitis
Most common cause of encephalitis
Lymphocytic infiltrates, microglial nodule, neuronophagia
Herpes simplex meningoencephalitis
HSV-1, hemorrhagic, necrotizing temporal lobes
Cowdry A viral inclusions, chromatin margination
Cortical necrosis, leptomeningeal inflammation
CMV meningoencephalitis
Necrotizing encephalitis and ventriculoencephalitis
Cytomegaly “owls eye inclusions”
Rabies meningoencephalitis
Negri bodies
JC virus meningoencephalitis
PML
Reactivation of JC virus
Immunocompromised patients
Demyelination
Nuclear inclusions in oligodendrocytes
Fungal meningoencephalitis
Typically caused by candidiasis
Cryptococcal meningitis
Soap bubble basal ganglia
Parasitic CNS disease
Toxoplasmosis
Immunocompromised patients
Rim-enhancing lesions
Encrusted bradyzoites
Free tachtzoites
Prion disease
CJD: rapid progressive dementia
Caused by prion proteins without DNA or RNA
Transmissible
Spongiform encephalopathy
MS pathology
Autoimmune response against myelin sheath leading to visual disturbances, parenthesias, spasticity, gait disturbances
Increased IgG in CSF
Multifocal white matter plaques
Oligoclonal bands
Acute disseminated encephalomyelitis
Monophasic demyelinating disease that follows viral infection/immunization
Acute autoimmune reaction to myelin
What is a stroke?
Permanent focal brain damage
Vascular etiology
Focal neurological deficit, symptoms or signs
Stroke diagnosis is based on
Neurological deficit and imaging
Most common sites of intracerebral hemorrhage
Cerebral lobes
Basal ganglia
Pons
Thalamus
Cerebellum
intracerebral hemorrhage management
Monitor
Reversal of anticoagulants, platelet replacement
Nutrition, hydration, DVT prophylaxis
Acute arterial hypertension <140
Hyperglycemia and electrolytes treated
Intracranial pressure monitoring
Seizure treatment (NO prophylaxis)
Surgery and decompression for those that are deteriorating or those who have hemorrhage in cerebellum
Most common cause of subarachnoid hemorrhage
Trauma
Most common cause of non-traumatic subarachnoid hemorrhage
Saccular Aneurysm
Subarachnoid hemorrhage symptoms and diagnosis
Worst headache of life
CSF fluid analysis
Subarachnoid hemorrhage management
Monitor
Nutrition and hydration
Hyperglycemia and electrolytes
Acute arterial hypertension management <160
Intracranial hypertension
Hydrocephalus
DVT prophylaxis
Seizure treatment and possible brief prophylaxis
Aneurysm clipping or coiling
Subarachnoid hemorrhage cerebral vasospasm and delayed cerebral ischemia
Leading cause of death and disability in SAH (7-10 days after)
Diagnosed using: TCD, CTA, MRA, angiography
Treat using nimodipine x21 days for all patients with SAH
Emergent stroke care
Document time of onset or last known normal
NIHSS
Four limb pulses
Vital signs
Avoid aggressive control of HTN (only with planned thrombolysis with tPA or tyrombectomy)
Monitor cardiac rhythm
Maintain hydration with normal saline (NO glucose)
Second IV line in thrombolytic patients
Supportive stoke care
Stabilize vitals
Hydration
Nutrition
Mobility
Pharmacological treatment for acute stroke
DVT prophylaxis- Aspirin and Clopidogrel within 24 hours, continue for 21-90 days then switch to single agent
Acute anti coagulation not recommended
Thrombolytic therapy- Alteplase (tPA) within 3 hours but up to 4.5 (do not give if high risk of bleeding)
What do we do about clots that cause stroke?
Mechanical thrombectomy within 24 hours
Cerebral Venous thrombosis
Headache, focal neurological deficits, seizures
Venous infarct: hemorrhagic with edema
Anticoagulation is treatment: heparin, LMWH, warfarin for 6-12 months
Acute dual anti platelet therapy is used for
Minor stroke and TIA for short periods (1-3 months)
Intracerebral hemorrhage
Small vessel disease: arteriolosclerosis (HTN), cerebral amyloid angiopathy (HTN, diabetes)
Large vessel disease: arteriorvenous malformations, dural arteriovenous malformations, cavernous sinus malformations, moyamoya disease
Major causes of stroke
Atherosclerosis
Cardioembolism (commonly caused by a fib)
Small vessel disease
Treatment of carotid atherosclerotic disease
Carotid endarterectomy
Carotid artery stunting
TCAR
Large vessel stoke mechanisms
Dissection
Atherosclerosis
Small vessel disease
Lacunar infarction
Stroke prevention
Risk factor modification and treatment
Anti-throbmbotic therapy (warfarin, direct oral anticoagulation)
Surgery and interventions
Carotid revascularization indications
Symptomatic severe or moderate stenosis (not indicated in symptomatic mild stenosis)
Considered in asymptomatic in asymptomatic moderate to severe stenosis
Not indicates in symptomatic carotid occlusion
CAS and CEA are equivalent
CEA higher MI risk
CAS higher stroke risk, riskier in older people
In asymptomatic severe stenosis, CEA
Intracranial stunting higher risk than medical treatments
Atrial fibrillation stroke prevention
Warfarin, DOACs
CHF stoke prevention
No anticoagulation, use Aspirin
Non-cardioembolic stoke prevention
No anticoagulation, use Aspirin
Patent foramen ovale stroke prevention
No anticoagulation, use Aspirin
PFO closure
Non-Anti-Thrombotic medical therapies for stroke prevention
Statins are effective in strove prevention (target LDL<70)
Lower HTN (SBP <120)
No hormonal replacement
No benefit from lowering homocysteine
_ is the most important medication for stroke prevention
Anti-platelet therapy (single agent)
Stroke prevention for patients with patent foramen ovale
Percutaneous device closure
The principle mechanism for CSF production is
Active secretion by epithelial membranes of the choroid plexus
Where does the spinal cord end
L1 or L2
Common indications for LP performance
Contraindications for LP
Is a CT scan always required before a LP
No
Indications for CT scan before LP
> 60 years
Known CNS
Immunocompromised
Abnormal level of consciousness
Seizure within 1 week of presentation
Focal finding on neuro exam
Suspicion of intracranial mass
Papilledema
suspected subarachnoid hemorrhage
Normal CSF
Clear and colorless
Viscosity similar to water
Turbid CSF
Bacteria
WBC or pus: suggestive of meningitis
Blood: suggest subarachnoid hemorrhage
Red and brown CSF
Blood
Yellow CSF
Jaundice
Xanthochromia (suggests subarachnoid hemorrhage)
CSF SAH vs TLP
CSF sampling tubes 1-3
Tube 1: chemical investigation
Tube 2: microbial investigation
Tube 3: microscopic investigations
Microscopic exam of Normal CSF
Total WBCs: 1-5
62% lymphocytes (if increased, aseptic or viral meningitis
36% monocytes
2% neutrophils (if increased, bacterial meningitis)
Free from blood (if present, SAH and malignancy)
CSF glucose
50-80
normal CSF glucose/ plasma glucose ratio: 0.6-0.7
What does a decreased CSF/plasma glucose ration indicate
CNS septic infections, brain tumors, TB meningitis and sarcoidosis, falsely low due to hypoglycemia or incorrect interpretation
False hypoglycorrhachia: decreased due to glucose saturation in plasma
Normal CSF protein
15-45 (albumin
-Igs produced by CNS lymphocytes
-Transthyretin (amyloidosis)
-structural proteins found in brain tissue
High CSF protein
RCB lysis from traumatic tap
Increased BBB permeability
Increased production by CNS tissue (MS, subacute sclerosing panencephalitis)
Obstruction (tumor, spinal stenosis)
Low CSF protein
Indicates Leak
due to dural tear from sever trauma
Otorrhea
Rhinorrhea
What is the MS panel?
CSF analysis of changes
Increased albumin in CSF
Increased BBB permeability
IgG of CSF can be obtained from
Blood
Local synthesis from plasma cells in CSF
Normal Q Albumin
<9
Normal IgG index
<0.7
What is the CSF index?
Indicator of relative amount of CSF IgG compared to serum
CSF lactate normal value
11-22 (increased in hypoxia, ischemia, bacterial meningitis, head injury, seizure, metabolic disease)
CSF glutamine normal level
8-18 (increased with ammonia)
Primary headache syndromes
Migraine
Tension
Trigeminal autonomic cephalgias
Most common debilitating primary headache
Migraine
Most common non-debilitating headache
Tension headache
What is happening in migraines?
Increased glutamate release
Trigger activates trigeminal system, triggering brainstem activation, vasodilation and changes in cerebral metabolism leading to cortical spreading depression
What defines a migraine?
4-72 hr duration
At least 2: one sided, moderate-sever, pulsating, worse with activity
At least one: nausea, photophobia and phonophobia
At least 5 attacks
Migraine with Aura
Visual (fortification spectrum)
Sensory
Speech or language
At least two: dysarthria, vertigo, tinnitus, hypacusis, diplopia, ataxia, decreased consciousness
No motor or retinal symptoms
Hemiplegic migraine
Migraine with aura
Fully reversible motor weakness
Fully reversible visuals, sensory, and or speech/language symptoms
Can be familial or sporadic
Retinal migraine
Monocular
Chronic migraine criteria
> 15 days per month
Symptoms > 8 days per month
Duration >3 months
Symptoms of migraines
Fatigue and yawning
Mood alteration
Migraine with aura is associated with
small increased risk of stroke
Triggers for trigeminal activation
Bright lights
Odors
Poor vision
Dry eye
Dental, ear or jaw pathology
Migraine treatments
Triptans (avoid if peripheral arterial disease)
Gepants
Divans
NSAIDS
Acetaminophen
Ergots (avoid in pregnancy and peripheral artery disease)
Opioids-avoided
Bitalbital containing- rebound headache likely
Migrane prevention
Antihypertensive- propranolol
Antidepressants- amitriptyline
Anticonvulsants- topiramate
Tension type headache
2 of the following: bilateral location, pressure like, mild-moderate, not aggravated by physical activity
no nausea or vomiting
Photophobia or phonophobia may be present by not both
Tension-Type headache treatment
Antidepressants- Amitriptyline
Physical therapy
Trigeminal Autonomic cephalgias
Activation of posterior hypothalamus
Serve unilateral pain within trigeminal nerve distribution
Ipsilateral CN features
Cluster headache
Severe unilateral sharp pain
At least one of the following: conjunctival injection or lacrimation, nasal congestion, eyelid edema, forehead or facial sweating, mitosis or ptosis
Occurs at least once every other day
Moore common in men
Cluster headache treatment
Sumatriptan or zolmitriptan
100% oxygen
Cluster headache prevention
Verapamil
Paroxysmal hemicrania
Ipsilateral autonomic symptoms
Short duration
Responsive to indomethacin
Red flags that indicate something other than primary headache
Focal neurological signs or symptoms
New onset if age is over 50
Change in character/severity
History of malignancy
Tobacco history
Headache awakening from sleep or worse with valsalva
When to image for a headache
Thunderclap headache
If concerns for aneurysm, tumor, AVM
MRI is most sensitive
Labs: CRP, TSH, B12
Secondary headache
New headache that occurs for the first time in temporal relation to another disorder that is the known cause of the headache
Due to trauma or injury
Non vascular intracranial disorders
Neuropathies and facial pains
Post traumatic headache pathogenesis
Axonal injury, alteration in cerebral metabolism, neuroinflammation, genetic predisposition
Post traumatic headache treatment
Amitriptyline- tricyclic antidepressants
Propranolol- beta blocker
Trigeminal neuralgia is caused by
Idiopathic
Classical: demyelination due to neurovascular compression by abnormal vascular loop
Secondary: MS CP angle tumors
Clinical presentation of trigeminal neuralgia
Severe stabbing or shock like pain for one to several seconds
Pain with light contact in trigger zones in V2 or V3 regions
Painful trigeminal neuropathy
Nearly continuous pain in one of the trigeminal nerve branch distributions
Caused by trauma or post-herpetic neuralgia
MRI is diagnostic
Trigeminal neuralgia treatment
Oxycarbazepine to carbamazepine
Idiopathic intracranial hypertension risk factors
Female of childbearing age
Obesity
Some medications and systemic disorders
Idiopathic intracranial hypertension clinical features
Headache: severe, constant, unresponsive
Pulsatile tinnitus
Retro-ocular pain or pain with eye movements
Papilledema
Bilateral or holocranial pain, exacerbated by position changes (lying down and exertion)
Idiopathic intracranial hypertension diagnosis
MRI (empty sella, optic nerve ectasia, flattening of posterior sclera, stenosis of transverse cerebral venous sinuses)
Lumbar puncture (opening pressure >25)
Ophthalmologic evaluation
Idiopathic intracranial hypertension management
Weight loss
Carbonic anhydride inhibitors, loop diuretics, indomethacin, corticosteroids
CSF shunting
Nonspecific low back pain
No red flags on history
Normal exam apart from pain and tenderness
Pain does not radiate
Musculoskeletal
Self limited
Types of non specific musculoskeletal low back pain
Arthritis
Strain
Spondylolysis
Spondylolisthesis
Treatment of non specific acute back pain
No bed rest
NSAIDS for 2 x 4 weeks
Do we use opioids for non specific acute back pain
Never
Maybe use tramadol for severe pain
Goal of treatment in chronic back pain
Control rather than cure
Intractable back pain
Failed multiple interventions
Require pain specialists
Red flags for back pain
Loss of function
Constitutional symptoms
History of cancer
Bowel or bladder problems
Recent infection
IV drug use
Recent spine procedure
What is the single most import exam technique in the evaluation of back pain
Straight leg raise
The straight leg raise is used to indicate
L4-S1 radiculopathy (reproduces pain)
Reverse straight leg sign
Evaluates radiculopathy associated with compressed nerves L2-L4
Patrick’s test
Determine back, radicular, or hip pain
Waddells sign
Suggest physiological component
Non anatomical pain
Hoovers Sign
Detects non-physiologic (psychogenic) weakness
Cremastic reflex
L1-2
Scratch upper inner thigh
Ipsilateral cremaster contraction
Anal wink
S2-S4
contraction
Bulbocavernous reflex
Contraction of anal ring
Imaging of the spine
Only perform if red flags are present
MRI without contrast is best
CT is the next best option
Should you scan someone with non specific back pain that has been going on for less than 3 months
NO
Who gets early imaging for back pain?
High concern for metastatic disease
Spinal infection concerns
Con earns for cauda equine or conus syndrome
Bowel or bladder dysfunction
Significant neurological deficits
Conus and Cauda
Compression of nerve roots and cord in lower spine
Neurological emergency
Bowel and bladder dysfunction
Structural compression from disk, bone, tumor, abcess
Cauda Equina syndrome
Compression of multiple nerve roots below conus
Asymmetric
Saddle anesthesia
Conus Medullaris syndrome
T12-L1
Symmetric
Peri-anal numbness and pain
Bowel and bladder dysfunction
Metastatic cancer in the spine is from
Breast
Prostate
Lung
Thyroid
Kidney
Consider if history of cancer or constitutional symptoms
Spinal cord tumors
Present with neurological findings first
Spinal epidural abscess
Risk with recent spinal trauma
IV drug use
Immunosupression
Fever and mailable, back pain
ESR and WBC elevated
Commonly S aureus
Vertebral Osteomyelitis
Same symptoms as spinal epidural abscess
Pain at night
May not have fever
Acute Lumbosacral Radiculopathy
Compression from body overgrowth or extruded disk (most common)
Zoster
Diabetic radiculopathy
Compression by mass
L5 radiculopathy is most common
If a disk protrudes midline
Sacral roots are affected first
Multiple roots
If disk protrudes lateral
Affect root exiting below
What do you do if MRI doesn’t give answers about radiculopathy
EMG with nerve conduction study
Treatment for acute structural lumbosacral radiculopathy
Treat symptoms
If severe or persistent- steroids
If significant neuro deficits or evidence of nerve damage by EMG- consider surgery
Central Spinal Stenosis
Neurogenic claudication
Leg pain and numbness with walking or standing
Improves with leaning forward or sitting because canal widens
Vertebral compression fracture
Associated with age
Painful
Sciatica
Pain from butt shoots down back of leg
L5 or S1
Irritation of nerve plexus
Ankylosing Spondylitis
Bamboo spine
Potts disease
TB in spine
Shingles
Radiculopathy associated with rash
Spasticity is velocity _
Dependent
Rigidity is velocity _
Independent
Hyperreflexia
Presence of spread or clonus
Seizure
abnormal or excessive synchronization of a population of cortical neurons
Epilepsy
Tendency toward recurrent seizures (2 or more) unprovoked by any systemic or acute neurologic insults
Pathophysiology of seizures
Imbalance between neuronal excitation and inhibition which results in excessive excitation and hypersynchrony within neural circuits
In generalized epilepsy networks are
Widely distributed and involve thalmocortical structures bilaterally
In focal epilepsies networks involve
Neuronal circuits in one hemisphere
Epileptogenessis
Sequence of events that converts a normal neuronal network to a hyper excitable network
Paroxysmal depolarization shift
The essence of seizures
Cellular events in which rapidly repetitive action potentials are not followed by usual refractory period
Leads to intercostal spike
Seizure etiology
Infancy/childhood:
Prenatatal or birth injury
Inborn error of metabolism
Congenital malformations
Childhood/adolescence:
Idiopathic/genetic
CNS infection
Trauma
Adolescence and young adult:
Head trauma
Drug intoxication/withdrawal
Older adults:
Stoke
Brain tumor
Acute metabolic disturbances
Neurodegenerative
What is a provoked seizure
Toxic or metabolic cause within previous 24 hours can be identified
Causes of provoked seizure
Hypoglycemia
Hyponatremai
Alcohol withdrawal
Exposure to illicit drugs
Prescription drugs
Treatment of provoked seizures
Targets provoking factor
Febrile seizures
Children 6 m-3 yrs
Triggered by fever above 101F
Higher risk of developing epilepsy if last longer than 10 min, focal seizure, or reoccurred within 24 hrs
May develop temporal lobe epilepsy
Good prognosis, only need to treat the fever
What type of seizure may present with impaired awareness
Focal
Seizure classification framework
Next steps after new onset seizure
History, physical exam
Blood tests (CBC, electrolytes, glucose, calcium, magnesium, phosphate, hepatic and renal function)
Lumbar puncture (if meningitis/encephalitis suspected)
Blood or urine screen for drugs
EEG
CT or MRI of brain
Seizure counseling
Risk of recurrence of seizure within two years= 21-45%
Increased risk associated with prior brain trauma, EEG with epileptiform abnormalities, brain imaging abnormalities, nocturnal seizure
If no additional risks an anti epileptic medication does not need to be started after first lifetime seizure
Seizure reoccurrence risk
How long do drivers have to wait after seizure to drive
3 months seizure free
Seizure triggers
Nonadherence/ inadequate treatment
Sleep deprivation
Fever or systemic infection
Metabolic and electrolyte imbalance
Stimulant or proconvulsant intoxication
Concussion or closed head injury
Hormonal variations
Stress
After two unprovoked seizures what should you do?
Start anti seizure medication
What is an EEG
An electrophysiological technique for recording the electrical activity arising from the brain
Signals are generated by cortical pyramidal neurons in the cerebral cortex (oriented perpendicularly to the brain surface)
How are EEG electrodes placed?
The 10-20 system
Normal adult EEG frequency
9 Hz
Beta
13-30 hz
Alpha
8-13 Hz
Theta
4-8 Hz
Children, sleeping adults
Delta
0.5- 4 Hz
Infants, sleeping adults
Spikes
3 Hz
Epilepsy
Most common montages in EEG
Bipolar and preferential
Epileptic activity on EEG
Spike and wave complex
Sharp waves
Polyspikes
Mild disruption EEG
Theta
Moderate disruption on EEG
Theta and delta
Severe disruption EEG
Delta
Profound disruption of EEG
Burst-suppression
Triphasic waves
Metabolic encephalopathy
Lateralized periodic discharges
Structural brain lesions
When do you order an outpatient EEG
Patients with episodic paroxysmal events
Patients with new onset seizures
Patients with a known seizure disorder by unclear diagnosis
Patient with unexplained chronic encephalopathy
When should you order an inpatient EEG
Seizure diagnosis, treatment, medication titration or prognosis
Medically refractive seizures
Patients with epilepsy who has failed 2 or more anti epileptic medications needs to be seen by a Epileptologist for an EMU evaluation
Status epilepticus
Active part of seizure lasts more than 5 minutes
Person goes into second siezure without recovering consciousness from the first one
Repeated seizures for 30 min or longer
Non convulsive status epilepticus
Altered mental status
Subtle signs
Suspect in comatose patients who present after a prolonged generalized tonic-clonic siezures
Stat EEG
Non convulsive status epilepticus (NCSE)
Encephalopathic or comatose patient where you suspect NCSE
New onset refractory status epilepticus
Healthy person without seizure history starts having seizures and progresses to status epilepticus
Sudden unexplained death in epilepsy
Unexplained death in someone with epilepsy who was otherwise healthy
Neuro cutaneous syndromes
Abnormalities of neuroectodermal development
Can be inherited or sporadic
Neurofibromatosis
Autosomal dominant mutations in tumor suppressor genes
NF-1 gene: 17q11.2 neurofibromin (more peripheral)
NF-2 gene: 22q12 merlin (more central)
NF type 1
100% penetrance
Hyperpigmented lesions, neurofibromas, lesions in bone, CNS, peripheral nerves, other organs
Cafe au lait macules
Pathogonomonic for NF 1
Other cutaneous manifestations of NF1
Axillary freckeline
Subcutaneous neurofibroma (Schwann cells and fibroblasts)
Plexiform neurofibromas (risk of malignant degeneration)
NF 1 ocular manifestations
Iris hamartomas (Lisch nodules)
Retinal hamartomas
NF 1 neurological manifestations
Optic nerve gliomas (may cause precocious puberty)
Macrocephaly
Abnormal MRI signals (hyper intense lesions)
Behavior problems, learning disability
Spinal cord manifestations
NF1 systemic effects
Renal artery stenosis (secondary hypertension)
Moyamoya, intracranial aneurysms
Skeletal abnormalities
NF 1 diagnostic criteria
2 or more
6+ cafe au lait macules
Freckling in axillary or inguinal areas
First degree relative
two or more Lisch nodules
Bone lesions
Sphenoid dysplasia
Thinning of cortex of long bones
NF 2
Evident later
more CNS tumors
bilateral vestibular schwannomas + other brain and spinal cord tumors (hearing loss, vertigo, imbalance)
NF 2 diagnostic criteria
Bilateral CN VIII tumor
First degree relative with NF-2
NF treatment
No specific treatment
Comprehensive approach
Genetic counseling
Tuberous sclerosis complex
Autosomal dominant (can be sporadic)
TSC1: hamartin on ch 9q34.3
TSC2; tuberin on ch 16p13.3 (more sever)
Disorder of cellular differentiation
Hamartomas
Skin lesions, siezures, intellectual disability
Variable expression
TSC cutaneous manifestations
Ash leaf spots
Hypomelanotic macules (3 or more)
Facial angiofibromas (in nasolabial folds)
Shagreen patch (orange peel appearance)
Ungual fibromas (nails)
Forehead plaques
Neurologic manifestations TSC
Due to disrupted neuronal migration and abnormal proliferation
Intellectual disability
Infantile spasm
Intracranial lesions (atypical glial cells in center with giant cells in periphery)
Tubers
Subependymal nodules
Subependymal giant cell astrocytoma
Ophthalmic manifestations of TSC
Hamartomas of the retina or optic nerve
Optic atrophy
Cataracts
TSC systemic findings
Cardiac Rhabdomyoma
Renal angiomyolipoma
Renal cysts
Renal cell carcinoma
Hepatic angiomas/hamartomas
Pulmonary lymphangioleimyomatosis
TSC diagnosis
Clinical
Fetal ultrasound or MRI
Prenatal molecular diagnosis
MRI brain
TSC management
Multidisciplinary evaluation
Regular screening
Everolimus
Vigabatrin and steroids
Cosmetic surgery
Sturge-Weber syndrome
Encephalotrigminal angiomatosis
GNAQ gene mutation
Sturgeon Weber cutaneous findings
Facial cutaneous angioma (port wine stain)
CNS findings Sturge Weber
Leptomeningial angiomatosis in subarachnoid space
Brain atrophy Ipsilateral to facial lesion
Sturge Weber neurological manifestations
Seizures
Intellectual disabilities
Focal neurologic deficits
Cognitive impairment
Sturge Weber ophthalmologic manifestations
Glaucoma
Congenital enlargement
Homonymous hemianopsia
Sturge Weber imaging
Tram track sign
Leptomeningeal angiomatosis
Cortical atrophy
Sturge Weber diagnosis
Port wine stain plus one of the following:
Seizures
Contralateral hemiparesis
Intellectual disability
Ocular findings
Sturge weber syndrome treatment
Laser of facial nervous
Anti siezure meds
Epilepsy surgery
Prophylaxis
The nerve conduction study only studies
The fastest 20% of fibers (A alpha/beta)
What things are essential for a nerve conduction study?
Motor nerve end plate
Neuromuscular junction
Muscle
What things must be functional for sensory action potential?
Myelin sheath
Vasa vasorum
Myelin sheath
Sensory nerve conduction study is
Antidromic
Late responses
F waves (proximal nerve conduction)
H reflex (sensory up, motor down)
Repetitive nerve stimulation
Evaluation of neuromuscular junction disorders
The F wave is used to test
Radiculopathy
Gillian Barre
Peripheral neuropathy
Demyelinating neuropathies
The H reflex is used to evaluate
polyneuropathy
Early GBS
S1 radiculopathy
Axonal disease cause
Reduction in amplitude
Demyelinating diseases cause
Normal or mild reduction in amplitude
Prolonged
Reduced conduction velocity
Conduction block and temporal dispersion are characteristic of
Acquired demyelination
How do you test for compression neuropathies
Median-ulnar comparison study
How does temperature affect conduction velocity
Conduction is reduced by 1 m/s for every drop in 1 degree C
Electromyography use
Diagnosis
Localization
Assist in further testing
Prognosis
Abnormal spontaneous activity
Any activity outside end-plate lasting >3 seconds
A fibrillation potential is a marker off
Active denervation
Myotonic discharges
Myotonic dystrophy , myotonia
Fasciculations
Spontaneous discharge of individual motor unit
ALS, radiculopathy, entrapment neuropathies
Indications for nerve conduction study
myasthenia gravis
Infectious anterior horn disease
Rhabdomyolysis
Critical illness myopathy
What allows for determination of primarily neuropathic or myopathies pattern?
Analysis of motor unit action potention
Duration correlates with
MUAP
Amplitude reflects
Fibers closet to the needle
Polyphasia
Measure of synchrony
Neuropathic disorders
Nerves die
More muscles with each nerve
Myopathic disorder
Muscles die
Decreased muscle fibers with each nerve
What is bacterial meningitis?
Acute purulent infection within the subarachnoid space
Most common cause of bacterial meningitis
S pneumoniae
Neisseria meningitides
More common in children and young adults
Group B strep
Common in neonates and elderly
Process of meningitis development
Nasopharyngeal colonization
Nasopharyngeal epithelial cell invasion
Blood stream invasion
Bacteremia
Penetration of BB and invasion of the CSF
Survival and multiplication in the subarachnoid space
Induction of neuronal and auditory cell damage
Bacterial meningitis clinical manifestation
Classic triad: fever, headache, meningismus
Nausea
Vomiting
Photophobia
Rigors, sweating, weakness, myalgias
Kering sign
Brudzinski sign
Exanthem: macular, petechial, purpura
Findings consistent with meningitis
Kering sign
Brudzinski sign
Unchallenged rigidity
Papilledema
Isolated CN abnormalities
Complications of bacterial meningitis
CN complications
Seizures
Hydrocephalis
Subdural effusions
Bacterial meningitis evaluation
Serum electrolytes
urine osmo
CBC
Inflammatory markers
Head imaging
CSF analysis
Treatment for bacterial meningitis
Varies depending on age group
Dexamethasone can help prevent hearing loss in children
Brain abscess
Commonly in frontal lobe
classic triad: fever, headache, focal neurological deficits
Lumbar puncture contraindicated
Antibiotics and surgery
Encephalitis
Inflammatory process of the brain
Parenchymal dysfunction
Clinical manifestation of Viral Meningitis and Encephalitis
Fever
Headache
Meningeal irritation
Malaise
Myalgia
Anorexia
Nausea/vomiting
Lethargy/ drowsiness
Altered level of consciousness
Focal/diffuse neurological signs and seizures
Clues of Viral Meningitis and Encephalitis
Parotitis= mumps encephalitis
Flaccid paralysis, maculopapular rash, tremors= West Nile
Hydrophobia, aerophobia, pharyngeal spasms= rabies
Diagnosis of Viral Meningitis and Encephalitis
CT or MRI
EEG
CSF
Serum electrolytes
Serum and urine osmolality
Diagnostic test of choice for Viral Meningitis and Encephalitis
CSF RT-PCR (enterovirus, HSV)
CSF and serum (arbovirus)
Viral Meningitis and Encephalitis enteroviruses
Poliovirus
Caxsackievirus
Echovirus
Numbered enterovirus
enteroviruses
Fecal oral transmission
common cause of aseptic meningitis and community acquired viral meningitis
Arbovirus
Mosquito born
Flaviviruses: west Nile (+ sense)
Bunyaviruses: La Crosse (- sense)
Togavirusses (+ sense)
West Nile Virus
Enzootic and Epizootic cycles
Can be transmitted human to human (blood, organs)
Typically asymptomatic
Elderly, alcoholics, diabetics at risk
West Nile Neuroinvasive Syndromes
- Encephalitis
- Meningitis
- Acute flaccid paralysis
Presentation: fever, headache, weakness, neck stiffness, GI symptoms, rash
Treatment: supportive care
La crosse encephalitis
Tree hole mosquito, chipmunk, grey squirrel
Most people are asymptomatic
Mostly affects people less than 14
Treat with supportive care
HSV
HSV-1 transmitted through oral secretions
HSV 2- genital infection and aseptic meningitis (genital transmission)
Latent in sensory root ganglia
Reactivated by IV, hormonal, stress
Meningitis is more common with HSV 2 (treat with acyclovir)
Most common cause of acute sporadic viral encephalitis in US
Temporal lobe involvement, fever and focal neurologic signs
diagnosis based on HSV DNA in CSF
Characteristics of Parkinson’s
Akinesia
Hypokinesia
Bradykinesia
Parkinsonism
Tremor at rest-unilateral
Bradykinesia- unilateral
Rigidity-unilateral
Loss of postural reflex
Flexed posture
Freezing/motor blocks
Hallmark finding of Parkinson’s
Bradykinesia
Parkinson’s tremor
Affects distal limb
Low frequency
Occurs at rest
Parkinson’s rigidity
Lead pipe
Cogwheeling
Resistance to passive movement
Parkinson’s posture
Camptocormia
Parkinson’s gait
Shuffling steps
Freezing
En bloc turning
Loss of postural reflexes
Other motor features of Parkinson’s
Speech disorders
Respiratory difficulties
Drooling
Dysphasia
Nonmotor features of Parkinson’s
Cognitive decline
Sleepiness
Fatigue
Sensory
Sleep disorders
Psychiatric
Causes of death in Parkinson’s
Concurrent illness
decreased mobility
aspiration pneumonia
Trauma from fall
Pathology of Parkinson’s disease
Depletion of dopaminergic neurons in the substantia Niagara pars compacta of the basal ganglia
Lewy bodies
In substantia nigra
Can confirm diagnosis of Parkinson’s but not pathogneumoic
Aggregation of alpha sinuclein
When do clinical symptoms of Parkinson’s begin to emerge?
When 60% of dopaminergic neurons are lost
What molecule is being lost in Parkinson’s?
Dopamine (posterior putamen affected first/most severely)
Causes of Parkinson’s
Multi hit: oxidative stress, mitochondrial dysfunction, excitotoxicity, inflammation, apoptosis
Environmental risk factors for Parkinson’s
Pesticide
Head trauma
Chlorinated solvents
MPTP
diabetes
IBD
Viral infections
Parkinson’s management
Exercise delays progression
Focus of symptom management
Levodopa
Gold standard for Parkinson’s
Precursor to dopamine
Side affect= nausea
Start at lowest beneficial dose
Other dopaminergic agents
Dopamine agonists
NMDA receptor antagonists- Amantadine (dyskinesia blocker)
Levodopa-Enhancing agents- lengthen duration of action
TRAP
Cardinal features of Parkinson’s
Resting tremor
Rigidity
Akinesia
Postural instability
Loss of neurons with Parkinson’s occurs in
Substantia nigra pars compacta
Parkinsonism diseases
Clinical finding (two of 3 criteria)
Bradykinesia
Rigidity
Resting tremor
Dementia with Lewy bodies, Multiple system atrophy, progressive supranuclear palsy, corticobasal syndrome
Red flags for atypical Parkinsonism
Symmetric disease
Rapid progression
Early speech and swallowing difficulties
Parkinson’s Plus syndromes is also called
Atypical Parkinsonism
Lewy body disease
Shared pathology
Abnormal clumping and accumulation of alpha synuclein
Dementia with Lewy Bodies vs Parkinson’s
Dementia with Lewy Bodies: faster progression of dementia, more of a cognitive disorder
Dementia with Lewy Bodies
2 parts of diagnosis:
Dementia ( decline of executive functioning, attention, visuospatial abilities, slower processing speeds)
2 of the following features: fluctuating cognition, visual hallucinations, parkinsonism, REM behavior disorder
Dementia with Lewy Bodies associated features
Anti-psychotic medication sensitivity
Autonomic dysfunction
Increased delirium
Early postural instability
Delusions
Apathy
Depression/anxiety
Dementia with Lewy Bodies Prognosis
Survival for 5-8 years
Failure to thrive, aspiration pneumonia
Dementia with Lewy Bodies Management
Balance motor symptoms vs psychosis
Only antipsychotics: quetiapine, clozapine
Structured activities
Acetylcholinesterase inhibitors: improve cognitive fluctuations, visual hallucinations
Meantime
Multiple systems atrophy
Autonomic dysfunction +
Parkinsonism (MSA-P) and/or cerebellar dysfunction (MSA-C)
Multiple systems atrophy pathology
Accumulation of alpha synuclein in neurons but no Lewy bodies
Multiple systems atrophy prognosis
6-9 years
Multiple systems atrophy clinical features
Autonomic dysfunction
Orthostatic hypotension
Urinary frequency, retention or incontinence
Erectile dysfunction
Cold, hands or feet
Respiratory dysfunction
Constipation
Multiple systems atrophy Parkinsonism
Wheelchair sign
Rapid progression
Jerky tremor
Myoclonus
Dystonia
MSA clinical features
Gait and limb ataxia
Dysarthria
Eye-movement abnormalities
Corticospinal abnormalities: increased, deep, tendon reflexes, Babinski sign
Progressive Supranuclear Palsy
Progressive parkinsonism with recurrent falls, eye-movement abnormalities, and cognitive changes
Progressive Supranuclear Palsy Pathophysiology
Tauopathy
Tau accumulates to cause abnormal neurofibrillary tangles
Progressive Supranuclear Palsy prognosis
6-9 years
Death from pneumonia and sepsis
Progressive Supranuclear Palsy exam features
Parkinsonism
Abnormal facial expression
Nicole dystonia with retrocollis
Slow, spastic, hypophonic speech (growling)
Eye-movement abnormalities
Progressive Supranuclear Palsy impaired ocular movement hallmark feature
Vertical supranuclear gaze palsy
Progressive Supranuclear Palsy exam features
Play abnormalities in poor postural response
Frontal cognitive/behavioral changes (apathy, bradyphrenia, executive dysfunction, impulsivity, applause sign)
Halting speech pattern: progressive, nonfluent, aphasia, or apraxia of speech
Progressive Supranuclear Palsy speech
progressive non-fluent aphasia or apraxia of speech
Progressive Supranuclear Palsy imagining clues
Midbrain atrophy (morning glory sign, Mickey Mouse sign, hummingbird sign)
Corticobasal Syndrome
Progressive parkinsonism with asymmetric motor abnormalities, cortical signs, cognitive impairment
How common is corticobasal syndrome
Least common of Parkinsonism syndromes
corticobasal syndrome pathology
Tauopathy
corticobasal syndrome presentation
***Asymmetric motor abnormalities: parkinsonism, limb dystonia leading to contractures, myoclonus, resting/action tremor
Cognitive impairment: executive and visuospatial impairment, language abnormalities
corticobasal syndrome cortical signs
Apraxia
Cortical sensory sign (neglect, loss of two point discrimination, agraphesthesia, asterognosis
Alien limb phenomenon
corticobasal syndrome imagining features
Asymmetric frontoparietal lobe atrophy
Movement disorders
Impair normal mobility or cause abnormal movements to occur
Can be hyperkinetic or hypokinetic
Wilsons disease
Hepatolenticular degenaration
Treatable genetic disorder caused by defect in copper metabolism
Features: hepatic, neuro, psychiatric
Diagnosis: low ceruloplamin and elevated copper in urine, liver biopsy, genetic testing
Treatment: chelators
Wilsons disease MRI
Giant panda
Kayser-Fleisher rings
Wilsons disease
Tremors
Oscillatory movement caused by alternating contraction of muscle groups
Must determine if shaking is due to tremor or other cause
Tremor at rest
Parkinson’s
Action tremor
Essential tremor
Intention tremor
Cerebellar
If tremor decreases in amplitude when moving the joint it is a _ tremor
Resting
If tremor increases in amplitude when moving the joint it is a _ tremor
Action
Essential tremor
Can be familiar tremor
Gets better with alcohol
Essential tremor presentation
Postural and action tremor
Mostly in upper extremities
Bilateral symmetric tremor
Essential tremor treatment
Primidone
Propranolol
Deep brain stimulation
Intention tremor is seen with
Ataxia (cerebellar disorders)
Can be caused by alcohol, stoke, medications, tumors
Can be genetic
Friedreich’s ataxia
Most common hereditary ataxia
Progressive weakness, neuropathy, skeletal deformities, diabetes, HOCM
Dystonia
Excessive muscle contraction that causes abnormal movements, postures, or tremors
May be primary
Or secondary
Dystonia symptoms
foot dragging
Tremor
Abnormal postures
Excessive eye closure
Dystonic tremor features
Asymmetric
Irregular
Jerky
Abnormal postures
Look for Nystagmus
Dystonia treatments
Oral medications- anticholinegics, baclofen, clonazepam, dopaminergic agents
Chemodenervation- Botox
Deep brain stimulation
Botulinum neurotoxin injection uses
Dystonia
Spasticity
Chronic migraine
Tremor
Dyskinesia
Drooling
Excessive sweating
Chorea
Random flowing movements
Causes of chorea
Sydenham chorea
Drug induced
Thyroid disease
Chorea gravidarum
Huntington disease
Hemiballismus
Huntington disease
Autosomal dominant disorder caused by trinucleotide repeat (CAG)
Death 15-20 years after onset
Main features of huntingtons
Motor function: chorea
Cognitive: dementia, poor judgement, inability to learn
Psychiatric: emotional shifts, depression, personality changes
Huntington treatment
Treat chorea with benazines
Depression: SSRI
Irritability: SSRI
Myoclonus
Fast, jerky, shock like movement
Myoclonus causes
Epileptic
Essential
Metabolic
Medication induced
Serotonin syndrome clinical features
Fever
Myoclonus
Mental status changes
Hyperreflexia with clonus (inducible or spontaneous)
Drugs associated with serotonin syndrome
Triptans
MAO-B
Tramidol
Tricyclic antidepressants
Criteria for diagnosis of serotonin syndrome
Known serotonergic drug
Clonus
Treatment of serotonin syndrome
Stop offending drug (usually resolves in 24 hours)
Cyproheptadine
Neuroleptic malignant syndrome
Dopamine blockade
Dopamine withdrawal
Rapid progression
Fatal if not treated
Neuroleptic malignant syndrome risk factors
High dose of neuroleptics
Abrupt DA agonist withdrawal
Thyroid disease
CNS infection
Metabolic derangements
Drugs that cause Neuroleptic malignant syndrome
Aripiprazole
Haloperidol
Resperidone
Antiemetic agents: Metoclopramide
Neuroleptic malignant syndrome clinical features
Fever
Encephalopathy
Vitals deregulation
Enzyme changes
Rigidity and hyperreflexia
Mental status changes
Dysautonomia
Muscle rigidity
Neuroleptic malignant syndrome lab findings
Evelvated CK
Leukocytosis
Low iron
Abnormal acute phase reactants
Neuroleptic malignant syndrome diagnosis
3 major or 2 major and 4 minor criteria
Neuroleptic malignant syndrome treatment
Stop offending drug
If severe: dantrolene, benzodiazepines
Serotonin syndrome vs Neuroleptic malignant syndrome
Malignant hyperthermia
Exposure to anesthesia
Ryanodine receptor RYR gene
Hyperkinetic emergencies
Myoclonus
Ballism/Chorea
Storms
Acute dystonic reactions
Oculogyric crisis
Malignant vocal tics
Acute tardive phenomena
Withdrawal emergent syndrome
Acute dystonic reactions
Exposure to DA blocker or antiemetic
Emerge within 5 days
Treatment: IV diphenhydramine (Benadryl)
Oculogyric crisis
Fixed upward gaze
Acute akathisia
Most common movement disorder
Distressing side effect from neuroleptic medication
Treatment: stop medication or propranolol
Acute Hemiballism/Hemichorea
Subthalamic nucleus stroke, hyper or hypo glycemia
Self-limiting
Acute onset chorea
Sydenham’s chorea
1-6 months after strep
Reaction to penicillin
Dopamine blockers should not be given for
Parkinson’s patients
Cyproheptadine is used to treat
Serotonin syndrome
Dantrolene is used to treat
Neuroleptic malignant syndrome
Unconscious behaviors
In-born
Automatic behaviors
Learned behaviors (ex.walking)
Involuntary movements
Non-suppressible
Voluntary movements
Planned
Semi-voluntary movements
Induced by inner sensory stimulus, or an unwanted feeling or thought
Can be suppresses (restless legs, akathisia, tics)
Restless Leg Syndrome (Willis-Ekbom syndrome)
Urge to move legs
At rest, at night
Most common in calves
Restless legs is usually primary but can be associated with
Parkinson’s
MS
Neuropathy
Renal dysfunction
Pregnancy
Hypothyroidism
Anemia
Periodic limb movements during sleep
Lead to frequent arousals and daytime sleepiness
Associated with increased cardiovascular disease
restless legs treatment
Walking, exercise, medications
Gabapentin, Pregabalin, dopamine agonists
Tics
Premonitory feelings or sensations
Temporarily suppressible
May be simple or complex
Can be motor or phonic
Primary tic disorders
Transient tic disorder
Chronic tic disorder
Tourette syndrome
Secondary tic disorders
Medication induced
Tardive tics
Rett syndrome
Neuroacanthocytosis
Sydenham’s
Wilsons
Tics general treatment
Treat OCD, ADHD
Drugs may be used
DBS
Akathisia
Inner restlessness with compulsions to constantly move
Can be generalized or focal
Akathisia causes
Medication induced: antipsychotics, anti-emetics, anti-depressants, calcium channel blockers
Some antibiotics
Akathisia treatment
Discontinue offending agent
Stereotypies
Continuous repetitive and ritualistic movements
Often in children with intellectually disability or autism
More rhythmic than tics, occurs when I grossed in other activities
Which movement disorder is always medication induced?
Akathisia
Clinical symptoms of MS
Optic neuritis
Inter nuclear opthalmoplegia
Upper motor neuron weakness
Sensory disturbances
Ataxia
Tremor
Bladder/bowel dysfunction
MS diagnosis is based on
Clinical and supporting evidence (specifically MRI)
Important diagnostic principle for MS
Dissemination in time and space
Attack/flare followed by improvement
Clinical pattern of most MS patients
Relapsing remitting
Can also be primary progressive
RAPD
Commonly seen in MS
Clinically isolated syndrome which may prompt testing to determine if MS is present (MRI)
Common locations for MS scars
Optic nerve
Periventricular
Juxtacortical lesion
Dawson’s fingers
Spinal cord
If a patient with MS is given a contrast MRI _ will be present
Enhancing lesions
Internuclear Opthalmoplegia
Can occur with MS
Can occur while on medications leading to a medication change
Acute Disseminated Encephalomyelitis
Childhood illness with antecedent illness
Autoimmune disorder after trigger
Monophysite illness
Complete recovery
smudgy lesions on MRI
Neuromyelitis Optica Spectrum Disorders
Aquaporin 4 antibody
Can cause optic neuritis
MRI does not show clear demyelination
Lacks dissemination in space
Artery of Adamkiewicz
Large branch of anterior spinal artery
Can be disrupted leading to anterior cord syndrome
How do you confirm cord compression or anterior spinal artery infarct
CT
MRI
Posterior cord syndrome is commonly caused by
Syphilis- tabes dorsalis
Vitamin B12 defiency
MS
HIV associated vacuolar myelopathy
Diagnosis of extra-medullary vs intramedullary
Early sacral involvement is due to a _ lesion
Extra-medullary
A _ lesion spares sensation in perineal and sacral areas
Intramedullary
Hemisection cause _ syndrome
Brown-Sequard
Weakness in grip strength
Loss of fine motor skills
Radiating pain and tingling
Cervical spondylomyelopathy
degenerative disc disease
herniated disk
Tumor
Epidural abscess
Epidural hematoma
Paraneoplastic syndromes
Occur when cancer-fighting agents (such as antibodies) of the immune system attack part of brain, spinal cord, peripheral nerves of muscles
Types of paraneoplastic syndromes
Optic neuritis
Meningitis
Encephalitis
Neuropsychiatric manifestions
Epilepsy
Sleep disorders
Myelopathy
Neuropathy
LGL1 antibodies
Disruption of protein protein interactions
Decrease in AMPAR
NMDAR antibodies
Cross linking and internalization
Reduced NMDAR density
GABAbR antibodies
Functional blocking of target agent
Blocking of receptor signal
Anti NMDA antibody in serum and antibody
Anti-NMDA receptor encephalitis
Young individuals
Viral prodrome (can occur after HSV encephalitis)
Personality changes
Treat with steroids (look for ovarian teratoma)
Often seen with Anti-NDMA receptor encephalitis
Limbic encephalitis
Affects temporal lobe
New seizures, personality changes
Mild pleocytosis
Presence of oligoclonal bands
GAD65 Ab + in serum and CSF
Autoimmune encephalitis (must look for tumor)
Subacute onset of cerebellar changes (no CNS tumor)
Paraneuplatic process determined due to high antibody
Hodgkin lymphoma detected
Cerebellar ataxia ((DNER receptor antibody)
Poor response to immunotherapy
When to consider PNS/autoimmune disorder
Encephalitis after ruling out infections
Uncontrolled siezures
New onset psychiatric disturbances in someone with no psych history
Rapidly progressive cognitive decline
Diagnosis of PNS/autoimmune disorder
MRI brain
EEG
Serum antibody tests
CSF analysis to look for inflammation and antibodies
EMG
Look for tumor (CT, PET, ultrasound, colonoscopy, mammogram, tumor markers)
Nutrients involved in conversion of glucose into energy
Thiamine
Riboflavin
Niacin
Pyridoxine
Pantothenic acid
Magnesium
Manganese
Iron
Phosphates
Lipoid acid
Important cofactor for Citric acid cycle
B vitamins
People at risk for nutritional deficiency
Malabsopative states
Vitamins that affect cognition
B12
Niacin
Thiamine
Vitamins that affect the posterior columns
B12
Copper
Vitamins associated with neuropathy
Vitamin B12
Niacin
Vitamin B12 deficiency can cause
Wernicke-Korsakoff syndrome
Thiamine deficiency (B12)
Wernicke’s encephalopathy
-ocular abnormalities, gait ataxia, mental status changes
Korsakoff syndrome
-anterograde and retrograde amnesia
Beriberi-moderate chronic deficiency
-peripheral neuropathy
***lab tests for thiamine levels are unreliable
Treatment: IV thiamine
Inborn errors of folate (B9) metabolism
Defective transport of folate
Defective intracellular utilization due to enzyme deficiencies
MTHFR deficiency
Folate defiency
Autosomal recessive
Developmental delay, hypotonia
Testing- hyperhomocysteinemia
Treatment- folate, cobalamin, methionine
Congenital folate metabolism
Autosomal recessive
Testing-low CSF folate
Treatment- 5-formyltetrahydrofolate
Subacute combined degeneration
Degeneration of dorsal and lateral columns
Due to vitamin B12, folate, or copper deficiency
Vitamin B12 (cobalamin) deficiency
Check levels in serum (MMA or homocysteine)- may be deficient if level is <258
May see stomatitis
May develop from pernicious anemia
Treatment- cyanocobalamin
Vitamin B9 (folate) deficiency
Important for DNA synthesis, oligodendrocyte growth and myelin production
Maternal deficiency leads to neural tube defects in infant
Testing- red blood cell folate
Treat- 1 mg folate daily
Copper defiency
Associated with gastric disease, surgery, or excess zinc
Testing- low serum copper, ceruloplasmin
Treatment- elemental copper, stop zinc
Nutritional neuropathy
Stocking glove sensory changes
Abrupt Guillain-Barré syndrome
B1, B3, B6, B9, B12, copper deficiencies
B6 toxicity
Niacin (B3) deficiency
Pellagra- dermatitis, diarrhea, dementia
(Think of alcoholic encephalopathy that does not improve with thiamine or benzodiazepines)
Treatment- nicotinic acid
pyridoxine (B6)
-microcytic hypochromic anemia, stomatitis, dermatitis, peripheral neuropathy with deficiency
Sensory ganglionopathy with toxicity
Treat deficiency with oral supplementation
Vitamin A (retinol)
Visual function
Toxicity- intracranial hypertension
Vitamin D
Proximal myopathy
Replace with Vitamin D2 or D3
Vitamin E
Deficiency due to malabsorption
Ataxia, myopathy
Treat with supplementation
When do you think about nutritional defiency
Cognition or psychiatric symptoms
Sensation
Balance
Weakness
Vision
People with high risk of malnutrition or malabsorption
Tests for cognitive or psychiatric symptoms
B12, folate, thiamine
Sensation issues
B12, B6, thiamine, folate
Balance issues
B6
Weakness
B12
Vision
B12, folate
Highest yield nutritional work up
B12, folate, thiamine, B6
Increased intracranial pressure is caused by
Mass effect
Increased CSF
Decreased CSF reabsorption
Increased blood volume
Idiopathic: hypervitaminosis A, tetracyclines
Cerebral edema
Hydrocephalus
Herniation
Cingulate herniation
Herniation under flax cerebri
Anterior cerebral artery compression
Descending transtentorial herniation
Unilateral:
Ipsilateral blown pupil
Contralateral hemiparesis
Occlusion of posterior cerebral artery
Coma and kernohan phenomenon
Duret hemorrhage
Bilateral:
Inferior displacement of brainstem
Duret hemorrhage
Cerebellar tonsillar herniation
Herniation into foramen magnum
Brainstem compression resulting in coma/death
Transcranial herniation
Post surgical/post traumatic cranial defect
Increased edema post decompressive hemicraniectomy
Mushroom cap appearance
Cortical brain compression and infartction
Contusions of cranial margin
Most common tumor in intracranial space
Metastasis- adenocarcinoma
Pilocytic astrocytoma
Common in children
Germinoma usually affects
Young males
Schwannoma
Glioblastoma IDH-wildtype
Most common adult intraparenchymal brain tumor
Age >40
Rapid growth, short survival
Resection followed by chemo and radiation
EGFR gene
Astrocytoma IDH-mutant
Usually occurs in mid-30s
Commonly in supratentorial frontal lobes
Treatment: resection, radiation/chemo
+IDH antibodies, ATRX mutation
Oligodendroglioma IDH-mutant 1p/19q codeletion
35-45 yrs
Mostly in frontal lobes
Resection, radiotherapy followed by PVC
Perinuclear clearing
First branch point for differentiating infiltrating gliomos
IDH mutant or wild type
Meningiomas
66yrs is median age
Arachnoid cell origin
Risk factors: exposure to ionizing radiation
Resection, radiation therapy
Whorls, nuclear pseudoinclusions
Vestibular Schwannoma
Benign tumor
Located in cerebellopontine angle
If bitlateral- NFM type 2
Surgical resection, focal radiation
Antoni A areas, verocay bodies
Hemangioblastoma
Von Hippel-Lindau syndrome
Surgical resection
PitNET/adenoma
Endocrine excess/deficits
Manage with medications
Adamantinomatous craniopharyngioma
5-15 years, 46-60 yrs
Derived from rathke pouch
Endocrine deficits, visual issues, hypothalamic syndrome
Wet keratin, CTNNB1 mutations
Pilocytic Astrocytoma
Common in children
Typically cerebellar (cystic lesion with enhancing neuronodule)
Mutations in MAPK pathway- KIAA1549::BRAF
Medulloblastoma
Located in posterior fossa
More common in children
Resection and chemo/radiation, high nuclear to cytoplasm ration
Diffuse midline glioma H3 K27-altered
5-11 yrs
Midline location
Perivascular pseudorosettes
Ependymal tumors
Glial neoplasms
Usually in 4th ventricle
Surgical resection, radiation
Pineal gland tumor
Germinoma is most common
Young male patient
Peripheral neuropathy results from injury to
Either axon or myelin sheath
Wallerian degeneration
Axon loss and balling of myeloid
Caused by trauma, infarct due to diabetes, drugs or neoplasm
Segmental demyelination
Injury to Schwann cell or myelin results in demyelination
Axon remains in tact
Acute and chronic conditions
Gillian Barre syndrome
Inflammatory neuropathy
Immune mediated demyelination
Preceded by infection
Plasma exchange to remove antibodies
Chronic Inflammatory Demyelinating polyneuropathy
Most common acquired inflammatory peripheral neuropathy
Evolves over years, relapsing and remitting
Time course and response to steroids distinguishes from GBS
Onion bulb histology
Vasculitits associated neuropathy
necrotizing arteritis, Perivascular inflammation, hemorrhage and hemosiderin
Diabetic neuropathy
Charcot-Marie Tooth
Most commonly inherited peripheral neuropathy
CMT1-demyelinating and hypertrophic
CMT20 axonal
Muscle fiber type is determined by
Innervating neuron
Common causes of toxic myopathies
Statins
chloroquine/hydroxychloroquine
Corticosteroids
Skeletal muscle atrophy
Denneravation, disuse, old age, primary myopathy,
Neuropathy vs Primary myopathy/dystrophy
Dermatomyositis
Autoimmune damage to small blood vessels
Muscle weakness, skin changes (Gottrons papules, heliotrope rash)
associated with malignancy
Polymyositis
Inflammatory myopathy
Similar myalgia and weakness as Dermatomyositis but no skin changes
Cell mediated with CD8+ endomysial lymphocytic inflammation and macrophage infiltration
Myofibular necrosis
Immune mediated necrotizing myopathy
Subacute muscle weakness
Increased CK levels
Anti-HMG-CoA reductase, Anti-SRP autoantibodies
Random necrosis and regeneration
Inclusion body myositis
Slowly progressive proximal and distal weakness
Milk evelvations in CH
rimmed vacuoles with basophilic granular material
Mitochondrial dysfunction
Refractory to immunosuppression
Duchenne vs Becker
Duchenne- no dystrophin
Becker- reduced/uneven dystrophin
Inherited myopathies
Chronic progressive external ophthalmoplegia
Sign of mitochondrial myopathies
Mitochondrial myopathies
Metabolic myopathies
RYR1 mutation may be associated with malignant hyperthermia in ion channel myopathies
High CPK
Myopathy
Fatigue
Neuromuscular junction
Coma
State of unconsciousness in which mental processes are impaired and a person cannot be roused
Primary injury
Injury that occurs immediately
Secondary injury
Results from primary injury ex. Inflammation
Diastatic fracture
Suture splits
Physical exam findings associated with skull base
Battle’s sign
Raccoon eyes
Epidural hematoma
Bleeding from meningeal artery
Due to skill fracture in the pterion leading to bleeding
Brief of consciousness, come to, skull fracture due to trauma causing laceration, hematoma expands and pushes on temporal lobe of brain, pupil enlarges, contralateral hemiparesis, midbrain becomes compressed, RAS shuts off, coma and death
Subdural hematoma
Bleeding from bridging cortical vein
Not associated with fractures, associated with severe TBI (sudden stop, sudden acceleration leading to tearing of the bridging cortical vein)
The location of a traumatic subarachnoid hemorrhage is
Cortical
Coup lesion location
Contusion is under point of impact
Contrecoup lesion
Contusion remote from site of impact
Contusions are
A brain bruise
Concussion imaging
Normal brain imaging
TBI severity
GCS 15-13 mild
GCS 12-9 moderate
GCS 8-13 severe
When you find spine trauma you must
Look for the second level of injury
How is spinal cord injury scored
ASIA score
Monro-Kellie Doctrine
Normal contents of the skull: brain, blood CSF
CSF production ~0.3 ml/min
Normal ICP 5-15 mmHG
Cerebral edema increases which part of the skull components
Brain
Most important ion for the control of ICP
Sodium (hyponatremia)
Goal cerebral perfusion pressure
60-70mmHg
Goal ICP
< 20-22 mmHg
Etiology of acute intracranial hypertension
Increased blood volume
Intracranial mass
Increased brain (hyponatremia)
Increased CSF
Which nerve is most sensitive to injury for high ICP?
CN 6
Intracranial pressure monitoring is needed for
GCS <8/9
Lateral and tonsillar herniation
Are both iodinated and ferromagnetic agents toxic to the kidneys?
No
Which imaging modality is sensitive to acute hemorrhage?
CT
Which imaging modality is useful for demyelinating lesions
MRI
Which imaging modality is nephrotoxic?
CT (iodinated contrast)
Chronic hemorrhage is best visualized on
MRI
Lobar hemorrhage
Deep hemorrhage
Cerebellar hemorrhage
Pontine hemorrhage
Intraventricular hemorrhage
Mixed hemorrhage
Epidural hemorrhage
Subdural hemorrhage
Subarachnoid hemorrhage (aneurysmal)
Subarachnoid hemorrhage (traumatic)
Subfalcine herniation
Transtentorial herniation
Uncal herniation
Tonsillar herniation
Upward herniation
Most common tumor of the brain
Metastasis
Most common primary tumor
Glioblastoma
Extra-axial
B12 and copper myelopathies are examples of
Intramedullary, intradural pathology
A meningioma and Schwannoma are examples of
Extramedullary, intradural pathology
