Weakness, paralysis, spinal cord compression Flashcards
spinal cord compression, GBS, demyelination, Todd's palsy and toluene solvent inhalation, myasthenia gravis, ALS,
Causes of spinal cord compression
spinal injury (MVA), malignancy (lung, breast, prostate, myeloma) infection (epidural abscess)
Symptoms of spinal cord compression
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
Management of spinal cord compression
emergency MRI IV steroids vs antibiotics neurosurgery, radiation oncology consult
when to use intrathecal chemotherapy?
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
Guillane barre syndrome presentation is:
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.
Todd’s Palsy
focal weakness of hand arm or leg after seizure.
Toluene inhalation presentation
cognitive impairment, flaccid weakness and absent DTR see non anion gap metabolic acidosis and RTA type 1 and hypokalemia.
transverse myelitis
rare inflammatory disorder causing segmental spinal cord injury.
cause of transverse myelitis
idiopathic and likely from underlying autoimmune disorder following viral infection (50% of cases)
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
transverse myelitis
spinal shock is
lower extremity weakness, flaccidity, areflexia, and absent Babinski sign sensory level to all primary sensory modalities a
Diagnosis of transverse myelitis is via
MRI with contrast shows enhancing cord segments with surrounding edema
CSF analysis of transverse myelitis is
elevated protein moderate lymphocytosis and normal glucose and NO oligoclonal bands
Treatment of transverse myelitis
supportive care with PT and IV steroids can help with pts who have an autoimmune component of dx
prognosis of transverse myelitis
3 month recovery but 40% may have some residual disability
Cauda equina syndrome is from
affects lumbosacral roots as they exit the spinal cord and caused by a herniated disk or tumor
severe unilateral pain in the saddle region that radiates down to the legs with associated asymmetrical lower extremity weakness and proximal or distal
cauda equina syndrome
features that distinguish cauda equina syndrome vs GBS vs transverse myelitis
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
Difference between transverse myelitis from GBS
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
Spinal cord infarction will have
back pain weakness, loss of sensation and reflexes and autonomic dysfunction and this happens abruptly in minutes to hours.
vitamin B12 deficiency will have
slow progressive weakness, ataxia and neuropsychiatric disturbances.
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)
how west nile virus works
pt has asymmetric flaccid paralysis and extrapyramidal symptoms with fever and maculopapular rash on chest and extremities
West Nile virus
Treatment of west nile virus
supportive and try mosquito prevention programs.
diagnosis of west nile virus
IgM serology in pts with only fever. CNS symptoms need LP to rule out other etiologies and confirm diagnosis with IgM antibody titer
Guillain Barre syndrome
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.
post polio syndrome
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.
ALS is
progressive neurodegenerative dx that happens in men >40 yrs
risk factors for ALS
age, family hx, cigarette smoking
ALS pathophysiology
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.
ALS presentation
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.
diagnosis of ALS is
clinically
but can get EMG (electromyogram) and nerve conduction studies and neuro imaging to rule out other causes
Treatment of ALS
riluzole (glutamine antagonist) only drug to improve clinical outcomes
median survival for ALS
5 years and respiratory failure is the main cause of death
What separates ALS from GBS?
no upper motor neuron signs are seen with GBS
asymmetrical limb weakness, no loss of sensory symptoms and see dysphagia and hand and tongue fasciculations
ALS
Upper motor neuron signs
spastic paralysis,
clasp knife rigidity,
hyperreflexia,
hypertonia
no fasciulations
Babinski sign
lower motor neuron signs
flaccid paralysis
hypotonia
hyporeflexia
muscle atrophy
fasciculations
CSF analysis for GBS and meningitis?
Diagnosis of GBS syndrome?
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.
Prognosis of GBS
generally good.
‘Nearly 25% get mechanically ventilated due to respiratory muscle weakness. 20% of severely affected are unable to walk after symptom onset.
Treatment of GBS
supportive + plasmapheresis or IVIG which can help accelerate recovery.
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.
features of a myasthenia crisis.
Treatment of myasthenia crisis initially and why is this 1st choice?
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.
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?
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.
do we ever use methotrexate with myasthenia crisis?
No. not indicated for myasthenia crisis.
how to distinguish between Horner’s syndrome vs Bell’s Palsy vs Myasthenia gravis when a patient presents like this?
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
cervical spondylosis is from
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.
Treatment of cervical spondylosis is
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
diagnosis of cervical spondylosis is with
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
difference between ALS and cervical spondylitis
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
pufferfish poisoning
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.
ciguatoxin is from
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.
saxitoxin is seen with
paralytic shell fish poisoning
see saxitonxin in urine.
Diagnosis of Myasthenia Gravis is by
If this is negative what is done next?
Do we still diagnose based off edrophonium test?
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.
4T’s of anterior mediastinal masses
thymoma’s - most common
teratoma - and other germ cells
thyroid gland or goiter
terrible lymphoma - located in middle or posterior medastinum.
Thymoma management
surgery in clinical setting with thymom and thymic carcinomas
Thymectomy is suggested for people who are <60 yrs and have generalized myasthenia gravis.
Lambert Eaten myasthenic syndrome is
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.
back pain, paresthesias and symmetrical weakness worse in lower extremities and hyporeflexia
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.
if suspecting acute inflammatory demyelinated polyneuropathy then need to get
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.
All suspected Guillian Barre Syndrome pts should get this diagnostic test?
LP to rule out underlying infection.
How to treat acute GBS?
How to treat acute myasthenia gravis crisis?
IVIG or plasmapheresis
don’t pick steroids. Steroids is 2nd choice for myasthenia crisis
don’t use steroids in GBS.
chronic inflammatory demyelinating polyradiculopathy is CIPD
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)
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
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.
ciguatera fish poisoning is from
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.
ciguatera fish poisoning affects
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.
organophophate poisoning:
salivation, bronchorrhea, bronchospasm and weakness and paralysis
hallmark test is anticholinergic challenge with atropine where lack of response would support diagnosis of organophosphate poisoning.
what is scromboid poisoning?
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.
