Neurology III

Question 1

MS (multiple sclerosis):
1) How does it typically present?
2) What is the pathophys?

Answer 1

1) Episodic neurological symptoms causing significant disability; onset <35
2) Demyelination of nerves in CNS

Question 2

What is the epidemiology of MS?

Answer 2

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

Question 3

MS:
1) Etiology?
2) DDxs?
3) Tx?

Answer 3

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

Question 4

MS:
1) Evaluation?
2) LP?

Answer 4

1) MRI + contrast (gadolinium): perivascular and or corpus collosum white matter lesions
2) Elevated oligoclonal bands or IgG index is supportive

Question 5

How is MS diagnosed?

Answer 5

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

Question 6

MS:
1) What is the imaging diagnostic standard?
2) Why is the imaging method important?
3) What does the imaging standard reveal?

Answer 6

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)

Question 7

Name and describe the most common type of MS

Answer 7

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

Question 8

Describe Secondary progressive multiple sclerosis (SPMS)beginning as RRMS

Answer 8

1) Episodic flare-ups with residual/persistent deterioration in neurologic function
2) ∼2% risk per year of RRMS becoming SPMS

Question 9

Describe Primary progressive multiple sclerosis (PPMS)(∼15%)

Answer 9

Steady decline of neurologic function from onset of disease without episodic flares

Question 10

Describe Clinically isolated syndrome (CIS) form of MS

Answer 10

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…)

Question 11

MS Tx?

Answer 11

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

Question 12

MS: What is the prognosis?

Answer 12

1) At 10 years, 2/3 are still ambulatory
-Treatment can preserve function
3) RRMS has best prognosis

Question 13

1) What does clinical diagnosis of MS require?
2) What can MRIs do for MS?

Answer 13

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

Question 14

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?

Answer 14

1) High dose IV corticosteroids
2) The disease-modifying agents immunomodulators (interferon beta) and immunosuppressive agents

Question 15

Optic neuritis:
1) Sx?
2) Pathophys?
3) Epidemiology?

Answer 15

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

Question 16

Optic neuritis:
1) Etiology:
2) DDxs?
3) Tx?

Answer 16

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

Question 17

Alcohol Nutritional Neuropathy:
1) Sx and cause of Sxs?
2) Pathophys?
3) Epidemiology?

Answer 17

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)

Question 18

Alcohol Nutritional Neuropathy:
1) Etiology?
2) DDxs?
3) Tx?

Answer 18

1) Heavy alcohol use and poor nutrition
2) B12 deficiency, infection, trauma, DM peripheral neuropathy
3) Alcohol abstinence, thiamine supplements

Question 19

Myasthenia Gravis:
1) Presentation?
2) Hallmark Sxs?

Answer 19

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

Question 20

Myasthenia Gravis:
1) Pathophys?
2) Epidemiology?

Answer 20

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

Question 21

Myasthenia gravis (MG):
1) Etiology?
2) DDxs?
3) Txs?

Answer 21

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

Question 22

CRPS (Complex Reginal Pain Syndrome):
1) Typical presentaton?
2) Pathophys?

Answer 22

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

Question 23

CRPS (Complex Reginal Pain Syndrome):
1) Epidemiology?
2) Etiology?

Answer 23

1) European ancestry, 5/100,000 pts/yr 200,000 pts/yr in US
2) Thought to be injury or trauma to a nerve

Question 24

CRPS (Complex Reginal Pain Syndrome):
1) DDxs?
2) Txs?

Answer 24

1) Cellulitis, vasculitis, lymphedema, DVT, nerve impingement
2) Early, NSAIDs (Naproxen,) Prednisone, pain management for PT, nortriptyline qhs, gabapentin, nerve blocks

Question 25

Guillain -Barré: 1) Sx? 2) What does it typically start with?

Answer 25

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.

Question 26

Guillain -Barré: 1) S/S **NOT** suggestive of GBS? 2) Epidemiology?

Answer 26

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

Question 27

Guillain-Barré: 1) Etiology? 2) DDxs?

Answer 27

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

Question 28

Guillain -Barré: 1) Txs? 2) Prognosis?

Answer 28

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

Question 29

What Tx is contraindicated in acute inflammatory demyelinating polyneuropathy but helpful in chronic inflammatory demyelinating polyneuropathy such as Multiple Sclerosis?

Answer 29

Corticosteroids

Question 30

Cerebral palsy 1) What are some adulthood issues related to it? 2) How common is it? 3) Secondary issues?

Answer 30

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

Question 31

1) What is typically seen in CP pts on PE? 2) Why is it easier to Dx now?

Answer 31

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

Question 32

CP risk factors and etiology

Answer 32

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

Question 33

What is the TORCH mnemonic?

Answer 33

Toxoplasmosis Other infections (HIV, syphilis, etc) Rubella CMV (cytomegalovirus) HSV

Question 34

Cerebral palsy: what do the clinical features have in common?

Answer 34

Most are d/o of movement + secondary abnormalities such as poor balance and sensory deficits

Question 35

List and describe the 4 clinical features of CP. Which is most common?

Answer 35

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

Question 36

List some Common comorbidities not part of core definition of CP

Answer 36

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

Question 37

CP management: 1) Txs? 2) Adulthood management? 3) What is a common cause of death?

Answer 37

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

Question 38

What is an important first step in diagnosing neuropathies?

Answer 38

Distinguish peripheral from central lesions is important first step

Question 39

Polyneuropathy vs. central lesions: Who should you suspect CNS lesions in?

Answer 39

Patients with CN and balance disturbances, speech and visual disturbances, CN palsies, ataxia, bowel or bladder incontinence

Question 40

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?

Answer 40

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

Question 41

Diabetic polyneuropathy: What is the distribution of sensory loss?

Answer 41

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)

Question 42

Peripheral Neuropathies: 1) What are they? 2) Pathophys? 3) Epidemiology?

Answer 42

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

Question 43

Peripheral Neuropathies: 1) Etiology? 2) DDxs? 3) Txs?

Answer 43

1) Repetitive motions, ischemic injury or trauma, DM 2) Autoimmune neuropathies, MS, stroke, G-B 3) Underlying disease/complication. Gabapentin

Question 44

Polyneuropathy HPI/ ROS: Onset, Duration, Timing?

Answer 44

1) Rapid onset – think drug/toxins effects 2) Subacute – GBS 3) Chronic – Diabetic, lead toxicity

Question 45

Polyneuropathy hpi/ros: 1) Location/ radiation? 2) What should make you think of GBS? 3) What should make you think of DN?

Answer 45

1) Distal, proximal, ascending, stocking and glove… 2) Ascending motor 3) Stocking and glove sensory

Question 46

Polyneuropathy: What are some associated Sx?

Answer 46

Systemic disease, recent infection, surgery

Question 47

What does a polyneuropathy exam include? Describe this

Answer 47

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

Question 48

Peripheral neuropathy: What are the 5 routine labs?

Answer 48

1) CBC (anemia) 2) CMP (renal/hepatic/electrolytes) 3) FBG or HA1C 4) Vit B12 5) TSH

Question 49

Peripheral neuropathy: What are the PRN labs?

Answer 49

1) Ethanol, folate, thiamine, phosphorus 2) Lyme 3) RPR - syphilis 4) SPEP/UPEP, 24-hour urine 5) ANA

Question 50

Peripheral neuropathy: specialty labs/work up 1) What is the use of Electrodiagnostic testing?

Answer 50

1) Confirming, but not routine or initial EMG 2) Refer if symptoms worrisome: acute onset, asymmetrical, predominantly motor or autonomic, rapidly progressive

Question 51

Besides Electrodiagnostic testing, what are some other peripheral neuropathy specialty labs?

Answer 51

1) Nerve biopsy 2) Specific antibody testing (MG) Imaging not often required (unlike radicular or myelopathy)

Question 52

Peripheral neuropathy: treatment + f/u + education?

Answer 52

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

Question 53

Treatment of diabetic neuropathy

Answer 53

**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

Question 54

Clinical Recommendations for Polyneuropathies

Answer 54

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.