Weakness, paralysis, spinal cord compression

Question 1

Causes of spinal cord compression

Answer 1

spinal injury (MVA), malignancy (lung, breast, prostate, myeloma) infection (epidural abscess)

Question 2

Symptoms of spinal cord compression

Answer 2

gradually worsening, severe local back pain,

back pain worse in the recumbent position/ at night

Early signs: symmetric lower extremity weakness and hypoactive absent deep tendon reflexes

Late signs : bilateral babinski reflex, decreased rectal sphincter tone, paraparesis/paraplegia with increased DTR and sensory loss

Question 3

Management of spinal cord compression

Answer 3

emergency MRI IV steroids vs antibiotics neurosurgery, radiation oncology consult

Question 4

when to use intrathecal chemotherapy?

Answer 4

only for leptomeningeal (dura, arachnoid and pia mater)

metastasis due to certain solid and hematological cancers (breast, lymphoma, leukemia)

But not used to tx acute compression

Question 5

Guillane barre syndrome presentation is:

Answer 5

produces flaccid quadriparesis, no hypokalemia or non anion gap metabolic acidosis. CSF fluid with no WBCs, high protein and normal glucose.

See ascending symmetrical muscle weakness and absent DTR after infectious illness. 10% of pts may have arm or facial weakness instead of leg weakness.

80% complain of paresthesias of hands and feet but sensory exam is NORMAL. May have pain in back or extremities.

Question 6

Todd’s Palsy

Answer 6

focal weakness of hand arm or leg after seizure.

Question 7

Toluene inhalation presentation

Answer 7

cognitive impairment, flaccid weakness and absent DTR see non anion gap metabolic acidosis and RTA type 1 and hypokalemia.

Question 8

transverse myelitis

Answer 8

rare inflammatory disorder causing segmental spinal cord injury.

Question 9

cause of transverse myelitis

Answer 9

idiopathic and likely from underlying autoimmune disorder following viral infection (50% of cases)

Question 10

pt presents in spinal shock and with a band like squeezing pain. They will have bowel and bladder dysfunction. After one week they have spasticity, hyperreflexia, Babinski signs and clonus

Answer 10

transverse myelitis

Question 11

spinal shock is

Answer 11

lower extremity weakness, flaccidity, areflexia, and absent Babinski sign sensory level to all primary sensory modalities a

Question 12

Diagnosis of transverse myelitis is via

Answer 12

MRI with contrast shows enhancing cord segments with surrounding edema

Question 13

CSF analysis of transverse myelitis is

Answer 13

elevated protein moderate lymphocytosis and normal glucose and NO oligoclonal bands

Question 14

Treatment of transverse myelitis

Answer 14

supportive care with PT and IV steroids can help with pts who have an autoimmune component of dx

Question 15

prognosis of transverse myelitis

Answer 15

3 month recovery but 40% may have some residual disability

Question 16

Cauda equina syndrome is from

Answer 16

affects lumbosacral roots as they exit the spinal cord and caused by a herniated disk or tumor

Question 17

severe unilateral pain in the saddle region that radiates down to the legs with associated asymmetrical lower extremity weakness and proximal or distal

Answer 17

cauda equina syndrome

Question 18

features that distinguish cauda equina syndrome vs GBS vs transverse myelitis

Answer 18

Cauda equina syndrome - bowel and bladder sphincter weakness

GBS- no bowel or bladder sphinter weakness

Cauda equina will have severe lancinating or shooting pain in back

transverse myelitis no back pain

Question 19

Difference between transverse myelitis from GBS

Answer 19

both can have spinal shock with reflexes and Babinski sign will be absent

GBS –NO bowel and bladder symptoms or a sensory loss below the spinal segment

transverse myelitis –WILL have bowel and bladder symptoms or a sensory loss below the spinal segment

Question 20

Spinal cord infarction will have

Answer 20

back pain weakness, loss of sensation and reflexes and autonomic dysfunction and this happens abruptly in minutes to hours.

Question 21

vitamin B12 deficiency will have

Answer 21

slow progressive weakness, ataxia and neuropsychiatric disturbances.

Question 22

anterior horn cell (poliomyelitis) injury rather than transverse leukomyelitis thus will have sparing of ascending (pain and temperature and vibration or proprioception) and descending (pyramidal and extrapyramidal motor system and autonomics)

Answer 22

how west nile virus works

Question 23

pt has asymmetric flaccid paralysis and extrapyramidal symptoms with fever and maculopapular rash on chest and extremities

Answer 23

West Nile virus

Question 24

Treatment of west nile virus

Answer 24

supportive and try mosquito prevention programs.

Question 25

diagnosis of west nile virus

Answer 25

IgM serology in pts with only fever. CNS symptoms need LP to rule out other etiologies and confirm diagnosis with IgM antibody titer

Question 26

Guillain Barre syndrome

Answer 26

progressive and symmetrical ascending paralysis and paresthesias and decreased to absent reflexes and possible respiratory distress. This is **a monophasic disease** - meaning _after it hits its nadir at 4 weeks pts will slowly have weakness improve._ They will be stable and autonomically should improve. Rates of full recovery can be slow and 80% are able to walk at 6 months No need to repeat any IVIG after reaching the nadir of weakness. Also avoid steroids - can worsen GBS.

Question 27

post polio syndrome

Answer 27

See progressive weakness, fatigue, muscle aches and joint pain which worsens abruptly \>50. seen in pts who had history of paralytic polio due to age related drop out of existing motor units.

Question 28

ALS is

Answer 28

progressive neurodegenerative dx that happens in men \>40 yrs

Question 29

risk factors for ALS

Answer 29

age, family hx, cigarette smoking

Question 30

ALS pathophysiology

Answer 30

affects **upper** (affects corticospinal tract of upper motor neuron) and **lower** motor (anterior horns of lower motor neuron) neurons with relative sparing of sensory neurons NOTE if you were to get LP and CSF would be normal.

Question 31

ALS presentation

Answer 31

see bulbar, cervical and thoracic and lumbosacral neuronal involvement and this is PROGRESSIVE see asymmetrical limb weakness - hand or foot drop. See bulbar symptoms of **dysarthria, dysphagia and respiratory muscle** weakness and weight loss and muscle cramps or atrophy See **upper and lower motor neuro signs** - spasticity, hyperreflexia, and positive Babinski sign.

Question 32

diagnosis of ALS is

Answer 32

clinically but can get **EMG (electromyogram)** and nerve conduction studies and neuro imaging to rule out other causes

Question 33

Treatment of ALS

Answer 33

riluzole (glutamine antagonist) only drug to improve clinical outcomes

Question 34

median survival for ALS

Answer 34

5 years and respiratory failure is the main cause of death

Question 35

What separates ALS from GBS?

Answer 35

no upper motor neuron signs are seen with GBS

Question 36

asymmetrical limb weakness, no loss of sensory symptoms and see dysphagia and hand and tongue fasciculations

Answer 36

ALS

Question 37

Upper motor neuron signs

Answer 37

spastic paralysis, clasp knife rigidity, **hyperreflexia,** **hypertonia** **no fasciulations** Babinski sign

Question 38

lower motor neuron signs

Answer 38

flaccid paralysis **hypotonia** **hyporeflexia** muscle atrophy **fasciculations**

Question 39

CSF analysis for GBS and meningitis?

Answer 39

Question 40

Diagnosis of GBS syndrome?

Answer 40

diagnosis is made via LP and neurophysiology studies (EMG). CSF for GBS: elevated CSF prtoein with normal WBC (albuinocytological dissociation) and normal glucose. This is seen 1 week after onset of symptoms. EMG and nerve conduction studies will show evidence of polyneuropathy.

Question 41

Prognosis of GBS

Answer 41

generally good. 'Nearly 25% get mechanically ventilated due to respiratory muscle weakness. 20% of severely affected are unable to walk after symptom onset.

Question 42

Treatment of GBS

Answer 42

supportive + plasmapheresis or IVIG which can help accelerate recovery.

Question 43

female with double vision and difficulty swallowing which is worse at end of day. Cannot lift hands above her head and can't walk up or down stairs. See horizontal binocular diplopia and bilateral ptosis. increased generalized and oropharyngeal weakness, respiratory insufficiency and dyspnea which is worse when supine.

Answer 43

features of a myasthenia crisis.

Question 44

Treatment of myasthenia crisis initially and why is this 1st choice?

Answer 44

**Plasma exchange** or **IVIG** = because of rapid onset of action and benefits are transient. Don't pick steroids. These start within a few days and last for 3 weeks steroids are added for providing longer term benefit.

Question 45

what is also added to treatment of myasthenia crisis and why do we like this? what if they cannot tolerate 2nd line treatment then what to do next?

Answer 45

**Plasma exchange (IVIG)** followed by glucocorticoids because they add longer term symptom relief and their onset of action starts 2-3 weeks later with peak effect at 5.5 months Cannot **treat acute myasthenia crisis with steroids at first**. need to IVIG or plasma exchange. If people cannot tolerate steroids, they can be started on **azathioprine, mycophenolate mofetil, or cyclosporine** but have delayed onset of working compared to steroids.

Question 46

do we ever use methotrexate with myasthenia crisis?

Answer 46

No. not indicated for myasthenia crisis.

Question 47

how to distinguish between Horner's syndrome vs Bell's Palsy vs Myasthenia gravis when a patient presents like this?

Answer 47

**Myasthenia gravis:** will not have _an abnormal pupillary response_. Will see abnormal response from extraocular muscles (oculomotor, trochlear and abducens nerve and palate and vagus nerves **Horner's:** will have _abnormal mydiasis with pupil or fixed pupil_. Able to move muscles and smile. **Bell's palsy (facial nerve 7)** - normal pupil response. no extraocular eye muscle abnormalities. _But won't be able to smile._ Able to move eye muscles

Question 48

cervical spondylosis is from

Answer 48

progressive degenerative process of cervical vertebral bodies and discs in adults\>55 yrs seen disc herniation, osteophyte formation and hypertrophy of posterior longitudinal ligament and ligament flava. See spinal canal stenosis and medical spinal cord compression in 5 to 10% of individuals and flexion and extension of neck can exacerbate cord compression while extension - when pt bicycles can reduce diameter of spinal canal and cause inward buckling of the ligamentum flavum.

Question 49

Treatment of cervical spondylosis is

Answer 49

conservative with cervical immobilization for mild myelopathy and surgical decompression is needed for those who fail conservative measures, severe myelopathy or progressive deficits or acute deterioration with cord compression

Question 50

diagnosis of cervical spondylosis is with

Answer 50

MRI and CT for cervical cord compression MRI is needed for better intramedullary detail of the spinal cord while giving CT better images of bone and calcified tissues

Question 51

difference between ALS and cervical spondylitis

Answer 51

ALS - lower motor neuro deficits and fasciculations and cranial muscles. NO sensory deficits Cervical cord compression - see subtle or absent sensory deficits and along dermatome. EMG is needed to distinguish between both disorders

Question 52

pufferfish poisoning

Answer 52

**tetrodotoxin** - stops voltage gated sodium channels and prevents sodium depolization. acts on skeletal muscle through same mechanism and cuases relaxation seen within minutes and starts with perioral numbness, paralysis with loss of reflexes and severe hypotension. can cuase subsequent respiratory distress Give **charcoal within 1 hour of symptom onset and treatment is supportive.**

Question 53

ciguatoxin is from

Answer 53

dinoflagellate poisoning is reponsible for **ciguatera poisoning** via activation of voltage gated sodium channel and causes GI, cardiac, and neuro symptoms. See _nausea, vomiting, and diarrhea before cardiac (complete heart block_) and neuro symptoms. see perioral numbness. See **temperature dysesthias.**

Question 54

saxitoxin is seen with

Answer 54

paralytic shell fish poisoning see saxitonxin in urine.

Question 55

Diagnosis of Myasthenia Gravis is by If this is negative what is done next? Do we still diagnose based off edrophonium test?

Answer 55

serological testing: 85% of generalized MG will have positive _acetylcholine receptor autoantibodies_ 50% of ocular MG will have positive AChR antibodies 40% of pts who are seronegative for AchR antibodies are positive for _muscle-specific tyrosine kinase autoantibodies_ If serology is completely negative, **EMG and nerve conduction study** is done with repetitive stimulation to confirm diagnosis. We no longer do edrophonium and ice pack test due to high false positive rates.

Question 56

4T's of anterior mediastinal masses

Answer 56

thymoma's - most common teratoma - and other germ cells thyroid gland or goiter terrible lymphoma - located in middle or posterior medastinum.

Question 57

Thymoma management

Answer 57

surgery in clinical setting with thymom and thymic carcinomas **Thymectomy** is suggested for people who are \<60 yrs and have generalized myasthenia gravis.

Question 58

Lambert Eaten myasthenic syndrome is

Answer 58

paraneoplastic syndrome with small cell lung cancer that presents with slowly progressive proximal muscle weakness and decreased DTR, dry mouth, and ED. See vigorous muscle activation improves with recovery of DTR and muscle strength. Rarely see ocular symptoms.

Question 59

back pain, paresthesias and symmetrical weakness worse in lower extremities and hyporeflexia

Answer 59

Guillain Barre syndrome or acute inflammatory demyelinated polyneuropathy see ascending progressive symmetric weakness or paralysis after a week of URI or GI (campylobacter) - 1/3 won't have prior symptoms. As weakness ascends see weakness with walking, 3/5 strength of lower extremities compared to 4/5 strength of upper extremities in this pt. See bulbar symptoms (ophthalmoplegia, facial diplegia, dysarthria, dysphagia) and respiratory compromise. Back pain is from dx targeting the primary proximal polyneuropathy and pts having mild sensory symptoms such as paresthesias and sensory ataxia. they can develop dysautonomia = tachycardia, diaphoresis and sluggish pupils and absent reflexes.

Question 60

if suspecting acute inflammatory demyelinated polyneuropathy then need to get

Answer 60

LP - in all suspected GBS pts to rule out infectious causes and document elevated CSF protein in normal CSF WBC. See albuminocytologic dissociation. _LP is not diagnostic for GBS_ because it can see abnormal symptoms a week after symptom onset. **Nerve conduction and study and EMG are needed to confirm GBS**.

Question 61

All suspected Guillian Barre Syndrome pts should get this diagnostic test?

Answer 61

LP to rule out underlying infection.

Question 62

How to treat acute GBS? How to treat acute myasthenia gravis crisis?

Answer 62

**IVIG or plasmapheresis** don't pick steroids. Steroids is 2nd choice for myasthenia crisis don't use steroids in GBS.

Question 63

chronic inflammatory demyelinating polyradiculopathy is CIPD

Answer 63

**acquiring inflammatory disorder** characterized by demyelination of peripheral nerves and nerve root. **progressive, symmetric, proximal and distal muscle weakness** along distal sensory loss affecting arms and legs. See diminished or absent deep tendon reflexes. distal sensory loss is normally vibration and proprioception due to larger involvement of myelin fibers. **symptoms occur \>8 weeks** (so different from AIDP--GBS)

Question 64

progressive, symmetric, proximal and distal muscle weakness along distal sensory loss affecting arms and legs. See diminished or absent deep tendon reflexes. distal sensory loss is normally vibration and proprioception due to larger involvement of myelin fibers. \>8 weeks of symptom onset

Answer 64

**chronic inflammatory demyelinating polyradiculopathy** EMG will show **peripheral nerve demyelination** and (reduced conduction velocity and prolonged distal motor latencies). Lumbar puncture will show **albuminocytologic disassociations** in 90% of pts.

Question 65

ciguatera fish poisoning is from

Answer 65

person ingests a reef fish or a grouper, amberjack, moray eel, snapper, barracuda predatory fish ate dinoflagellates that produce cigatoxin and concentrate it in their ograngs that increases potency of their neurotoxin. stable and cannot be altered by cooking and so see 3 hrs after eating or delayed by 2 days.

Question 66

ciguatera fish poisoning affects

Answer 66

three organ systems **GI** - vomiting, diarrhea, abd cramping **Cardiac** - bradycardia, compelte heart block and hypotension **CNS** - perioral paresthesias, pruritis without urticaria, painful dentition or a feeling that teeth are loose, painful urination, and blurred vision are the most specific finding see temperature related dysesthesia.

Question 67

organophophate poisoning:

Answer 67

salivation, bronchorrhea, bronchospasm and weakness and paralysis hallmark test is **anticholinergic challenge with atropine** where lack of response would support diagnosis of organophosphate poisoning.

Question 68

what is scromboid poisoning?

Answer 68

this is where fish is left above 40 (104'F) and this allows bacteria on the fish's skin to convert histidine to histamine when you eat it it causes vasodilatory symptoms including, nausea, vomiting, and palpitations, headaches, and rash and pruritis. Difficult to distinguish between scromboid poisoning and anaphylaxis until after treatment is given. distinction is that rapid improvement of symptoms upon treatment with antihistamine agent which is definitive tx. seen more with self caught fish due to higher chance of improper storage.