Neurology III Flashcards
MS (multiple sclerosis):
1) How does it typically present?
2) What is the pathophys?
1) Episodic neurological symptoms causing significant disability; onset <35
2) Demyelination of nerves in CNS
What is the epidemiology of MS?
1) F»>M; most common above and below 45◦ (north of OH/MI border and Denver, south of Australia; uncommon around the equator
2) N European ancestry 1 in 10,000
Asians 1/20,000; rare in native Americans
MS:
1) Etiology?
2) DDxs?
3) Tx?
1) Thought to be autoimmune, influenced by low sunlight exposure/Vit D, +EBV Ab, h/o smoking + genetic susceptibility
2) Lyme ds, syphilis, HIV, stroke, somatization disorders, Vit B12 deficiency, SLE, lymphoma
3) No cure, symptom management is goal
-Refer to neurology
-Glucocorticoids, Interferon beta, MABs, immunosuppressants, anti-inflammatories
MS:
1) Evaluation?
2) LP?
1) MRI + contrast (gadolinium): perivascular and or corpus collosum white matter lesions
2) Elevated oligoclonal bands or IgG index is supportive
How is MS diagnosed?
No single specific diagnostic test; may be clinical, neuroimaging, and/or laboratory criteria
> Clinical Diagnostic criteria:
1) 2 or more documented (self reported or clinician observed) episodes of neurologic symptoms AND focal symptoms/signs of CNS
2) Symptoms must last > 24 hours
3) Interval between episodes must be at least 1 month
MS:
1) What is the imaging diagnostic standard?
2) Why is the imaging method important?
3) What does the imaging standard reveal?
1) MRI of brain and spinal cord looking for plaquesassociated with MS
2) Most sensitive and specific investigation: can dx in absence of reported symptoms
3) MRI reveals plaque formation and multifocal areas of demyelination (often periventricular)
Name and describe the most common type of MS
Relapsing-remitting multiple sclerosis (RRMS) (85% most common type):
1) Episodic flare-ups with near or complete recovery between attacks.
2) During attacks, new symptoms may present, whereas previous symptoms may worsen.
3) Complete recovery or residual deficits may ensue following each bout.
4) Best prognosis
Describe Secondary progressive multiple sclerosis (SPMS)beginning as RRMS
1) Episodic flare-ups with residual/persistent deterioration in neurologic function
2) ∼2% risk per year of RRMS becoming SPMS
Describe Primary progressive multiple sclerosis (PPMS)(∼15%)
Steady decline of neurologic function from onset of disease without episodic flares
Describe Clinically isolated syndrome (CIS) form of MS
Initial bout of neurologic symptoms caused by inflammation and demyelination of CNS for >=24 hrs and cannot be explained by anything else (ex/ infection, toxins…)
MS Tx?
1) Acute attacks: 1 gm IV methylprednisolone daily x 5 days
2) Disease modifying Tx (referral to MS specialty clinics):
-Interferon IM/SQ
-Monoclonal antibodies
MS: What is the prognosis?
1) At 10 years, 2/3 are still ambulatory
-Treatment can preserve function
3) RRMS has best prognosis
1) What does clinical diagnosis of MS require?
2) What can MRIs do for MS?
1) 2 neurologic deficits/objective attacks separated in time
2) Are gold standard imaging modality and may Dx MS diagnosis prior to meeting the clinical criteria
1) What are the treatment of choice for patients with acute MS flare-up symptoms?
2) What have all been shown to decrease the frequency of exacerbations and progression of disease in patients with MS?
1) High dose IV corticosteroids
2) The disease-modifying agents immunomodulators (interferon beta) and immunosuppressive agents
Optic neuritis:
1) Sx?
2) Pathophys?
3) Epidemiology?
1) Unilateral vision loss, pain with eye movements, pale optic disk; vision usually improves in 2-3 weeks; 50% will develop MS
2) Demyelinating disease of optic nerve, inflammation infiltrates nerve
3) F»>M, 20-40, more common the further away from the equator
Optic neuritis:
1) Etiology:
2) DDxs?
3) Tx?
1) MS, post-viral inflammation from upper respiratory tract, assoc with low Vit D, toxins, SLE, sarcoidosis
2) Inflammation, trauma, ischemia, compression of nerve, autoimmune diseases
3) All patients should be referred urgently to neuro or ophtho; IV methylprednisolone
Alcohol Nutritional Neuropathy:
1) Sx and cause of Sxs?
2) Pathophys?
3) Epidemiology?
1) Distal limb paresthesia, progresses to sensory loss or severe pain, distal vibratory loss or absent ankle tendon reflexes, weakness
Thiamine deficiency
2) Direct damage to the nerves by alcohol and malnutrition
3) Common in alcoholics (~1/2)
Alcohol Nutritional Neuropathy:
1) Etiology?
2) DDxs?
3) Tx?
1) Heavy alcohol use and poor nutrition
2) B12 deficiency, infection, trauma, DM peripheral neuropathy
3) Alcohol abstinence, thiamine supplements
Myasthenia Gravis:
1) Presentation?
2) Hallmark Sxs?
1) Intermittent, fatigable weakness of commonly used muscles, acquired antibody-mediated autoimmune disorder (can include paraspinal, esophageal and ocular muscles.) worsens throughout the day, improves with rest
2) Ptosis, diplopia, dysphagia, respiratory and proximal limb weakness
Myasthenia Gravis:
1) Pathophys?
2) Epidemiology?
1) Acetylcholine receptor antibodies block receptors on postsynaptic membranes of neuromuscular junctions. Antibodies form through a T mediated cell process from thymic dysfunction
2) F (20s-30s)>M (50s-60s) 14-20/100,00 in US
Myasthenia gravis (MG):
1) Etiology?
2) DDxs?
3) Txs?
1) Communication between nerves and muscles is interrupted. Worsened by some meds (antibiotics –aminoglycosides, B- Blockers)
2) Myopathy, stroke, ALS, chronic fatigue syndrome
3) Make sure UTD on all vaccines, cholinesterase inhibitors (increase concentration of acetylcholine,) immunosuppressants, corticosteroids, thymectomy
CRPS (Complex Reginal Pain Syndrome):
1) Typical presentaton?
2) Pathophys?
1) Burning pain, swelling and limited ROM localized to one limb with assoc color and temperature changes of skin and nails usually secondary 2 surgery or trauma. Most commonly in the hand. Pain greatly increases with light touch.
2) Pain dysregulation in sympathetic pathways and CNS with likely genetic, inflammatory and psychological contributions
CRPS (Complex Reginal Pain Syndrome):
1) Epidemiology?
2) Etiology?
1) European ancestry, 5/100,000 pts/yr 200,000 pts/yr in US
2) Thought to be injury or trauma to a nerve
CRPS (Complex Reginal Pain Syndrome):
1) DDxs?
2) Txs?
1) Cellulitis, vasculitis, lymphedema, DVT, nerve impingement
2) Early, NSAIDs (Naproxen,) Prednisone, pain management for PT, nortriptyline qhs, gabapentin, nerve blocks
Guillain -Barré:
1) Sx?
2) What does it typically start with?
1) Rapidly progressive paralysis targeting Bilat peripheral nerves (distal limbs) typically ascending, loss of reflexes emergency, can rapidly progress to respiratory compromise. Max paralysis at 4 weeks. Sensation typically remains intact, may have autonomic involvement (arrhythmias, fluctuating BP, GI dysmotility, sweating)
2) Typically starts with parethesias or numbness in hands/feet.
Guillain -Barré:
1) S/S NOT suggestive of GBS?
2) Epidemiology?
1) Asymmetric weakness, early bowel/bladder incontinence, well-defined sensory loss.
Pathophys: inflammatory demyelination of peripheral nerves
2) Rare: 1-2/100,000; M>F 3,000-6,000/yr in US, increases with age
Guillain-Barré:
1) Etiology?
2) DDxs?
1) Usually preceded 1-3 weeks by infections esp URI, GI (**Campylobacter) or major stressors to the body ( ex/ surgery, pregnancy, cancer)
2) Acute spinal cord disease, brainstem ischemia, MG, toxic and other acute neuropathies
Guillain -Barré:
1) Txs?
2) Prognosis?
1) Close monitoring (1/4 will need intubated), IVIG, plasmapheresis, steroids have not shown benefits
-Monitor/stabilize respiratory function – admission for respiratory difficulty
-IVIG, analgesics
2) 5% die, 75% recover by 1 year (gradually)
Typically takes weeks to months for significant recovery
~ 3% relapse
What Tx is contraindicated in acute inflammatory demyelinating polyneuropathy but helpful in chronic inflammatory demyelinating polyneuropathy such as Multiple Sclerosis?
Corticosteroids
Cerebral palsy
1) What are some adulthood issues related to it?
2) How common is it?
3) Secondary issues?
1) Cog dysfunction, seizures, pressure ulcers, osteoporosis, behavior and emotional issues, speech and hearing impairment
2) 3:1000 live births
-Permanent and non-progressive movement d/o
3) Brain injury that affects muscle tone, movement, posture, & balance
1) What is typically seen in CP pts on PE?
2) Why is it easier to Dx now?
1) Movement d/o – spasticity most common
2) Historically dx made 12-24 months of age, earlier now with perinatal US and post birth MRI to identify a brain injury ~ 6 months
CP risk factors and etiology
1) Prenatal brain injury– TORCH infections (toxins, Rh incompatible, maternal health (preeclampsia,) placental insufficiency, congenital)
2) 92% traced to perinatal brain injury but ~ 8% no clear cause
8% postnatal/adult onset – TBI or infection
What is the TORCH mnemonic?
Toxoplasmosis
Other infections (HIV, syphilis, etc)
Rubella
CMV (cytomegalovirus)
HSV
Cerebral palsy: what do the clinical features have in common?
Most are d/o of movement + secondary abnormalities such as poor balance and sensory deficits
List and describe the 4 clinical features of CP. Which is most common?
1) Spasticity: most common, 80% (hypertonia, hyperreflexia); UMN damage in motor cortex
2) Dyskinesia: muscle contractions and writhing.
3) Dystonia: abnormal tone of voluntary muscles
4) Ataxia
List some Common comorbidities not part of core definition of CP
1) Pain 75%
2) Cognitive 50%
3) Inability to walk, Hip displacement, progressive scoliosis
4) Speech, hearing, blindness
5) Epilepsy
6) Incontinence
7) Behavior or sleep disturbance
CP management:
1) Txs?
2) Adulthood management?
3) What is a common cause of death?
1) Treatments: Intramuscular Botox
Systemic and intrathecal muscle relaxants – baclofen and valium
Selective dorsal rhizotomy
PT/OT
2) Adulthood symptomatic management of seizures, pressure ulcers, osteoporosis, behavior and emotional issues, speech and hearing impairment
3) Aspiration pneumonia is a common cause of death
What is an important first step in diagnosing neuropathies?
Distinguish peripheral from central lesions is important first step
Polyneuropathy vs. central lesions: Who should you suspect CNS lesions in?
Patients with CN and balance disturbances, speech and visual disturbances, CN palsies, ataxia, bowel or bladder incontinence
Diabetic polyneuropathy:
1) What is it the most common form of?
2) What is it:? Where do Sx usually begin?
3) What Sx usually come first?
1) Peripheral neuropathy
2) Disease related to axon length; longer axons are affected first; symptoms begin in the distal lower extremities
3) Sensory symptoms usually precede motor symptoms - typically present with slowly progressive sensory loss and dysesthesias (numbness, burning sensation, and pain in the feet), with mild gait abnormalities, bilateral, symmetric
Diabetic polyneuropathy:
What is the distribution of sensory loss?
1) Classic “stocking and glove” distribution of sensory loss
2) Numbness may continue to extend proximally in severe cases, affecting the intercostal nerves (the next longest nerve fibers after the arms) and causing sensory loss over the sternum (but not typically motor symptoms like GBS or MG)
Peripheral Neuropathies:
1) What are they?
2) Pathophys?
3) Epidemiology?
1) Disease or injury to peripheral or autonomic nerves (pins and needles, tingling, numbness, weakness.) May be motor, sensory or mixed. Can be 1 nerve, many nerves or affect nerves throughout the whole body.
2) Nerve entrapment, multifocal demyelination
3) < 7% of general pop increases with age, up to 50% of adults with DM
Peripheral Neuropathies:
1) Etiology?
2) DDxs?
3) Txs?
1) Repetitive motions, ischemic injury or trauma, DM
2) Autoimmune neuropathies, MS, stroke, G-B
3) Underlying disease/complication. Gabapentin
Polyneuropathy HPI/ ROS: Onset, Duration, Timing?
1) Rapid onset – think drug/toxins effects
2) Subacute – GBS
3) Chronic – Diabetic, lead toxicity
Polyneuropathy hpi/ros:
1) Location/ radiation?
2) What should make you think of GBS?
3) What should make you think of DN?
1) Distal, proximal, ascending, stocking and glove…
2) Ascending motor
3) Stocking and glove sensory
Polyneuropathy: What are some associated Sx?
Systemic disease, recent infection, surgery
What does a polyneuropathy exam include? Describe this
Screening Neuro Exam
1) Mental Status (?A + O x 3)
2) CN 1-12
3) Motor (proximal and distal,) DTRs, Babinski
4) Sensory – light touch, 2-point discrimination palms/soles, proprioception BLE, vibratory, pin prick
5) Romberg: stand feet together eyes open, then closed x 1 min. ? loss of balance
6) Coordination
7) Gait
Peripheral neuropathy: What are the 5 routine labs?
1) CBC (anemia)
2) CMP (renal/hepatic/electrolytes)
3) FBG or HA1C
4) Vit B12
5) TSH
Peripheral neuropathy: What are the PRN labs?
1) Ethanol, folate, thiamine, phosphorus
2) Lyme
3) RPR - syphilis
4) SPEP/UPEP, 24-hour urine
5) ANA
Peripheral neuropathy: specialty labs/work up
1) What is the use of Electrodiagnostic testing?
1) Confirming, but not routine or initial
EMG
2) Refer if symptoms worrisome: acute onset, asymmetrical, predominantly motor or autonomic, rapidly progressive
Besides Electrodiagnostic testing, what are some other peripheral neuropathy specialty labs?
1) Nerve biopsy
2) Specific antibody testing (MG)
Imaging not often required (unlike radicular or myelopathy)
Peripheral neuropathy: treatment + f/u + education?
Focus on treating underlying etiology
1) Gabapentinoids (GABA, Neurontin, Lyrica)
2) Antidepressants (TCA, SNRI) can help alleviate neuropathic pain
-Screen with monofilament test – at least annually if not every visit
-Patient education about foot care
Treatment of diabetic neuropathy
Optimal control of DM, B-12 levels
Lifestyle, medications
Daily foot checks, foot care, skin care
Diabetic shoes
Manage pain
Manage blood sugar
Gabapentin/pregabalin
SNRI/Duloxetine
Clinical Recommendations for Polyneuropathies
1) Routine initial labs: CBC, CMP, FBG/A1C, TSH, Vit B12, SPEP.
2) Refer for EMG if symptoms acute, progressive, or worrisome.
3) Imaging not routine. MRI may help localize atypical neuropathy in cases of polyradiculopathy.
