Neuro Potpourri

Содержание

1. Neuro Potpourri
2. Brain trick
3. Neuro goes to the movies
4. Myasthenia GravisNeuropathiesALSRestless Legs SyndromeNeuro Potporrui
5. Chronic autoimmune neuromuscular disease Characterized by varying
6. Prevalence estimated at 14-20 cases per 100,000
7. Caused by defective neuromuscular transmissionAcetylcholine is the neurotransmitter involvedBoth anatomic and physiologic changes involvedMyasthenia Gravis
8. Myasthenia GravisIn normal neuromuscular transmission, Ach released
9. Myasthenia GravisIn MG, several factors contribute to
10. Myasthenia GravisIn MG, the post-synpatic membrane morphology
11. Myasthenia GravisThymus gland plays a role in
12. Myasthenia GravisAs a result of these changes
13. Myasthenia GravisUnusual distribution of symptoms often presents
14. Myasthenia GravisMuscle weakness, particularly in the extremities,
15. Myasthenia GravisMuscles which control breathing, swallowing (bulbar)
16. Myasthenia GravisSeveral classes of ABX: aminoglycosides, quinolones, macrolidesAntihypertensives: B-blockers and CCBLithiumMagnesium (including Maalox, Mylanta)
17. Myasthenia GravisTensilon test: IV administration of this
18. Myasthenia GravisAntibody testing is utilized to detect
19. Myasthenia GravisEMG testing, using repetitive nerve stimulation,
20. Myasthenia GravisLimited number of clinical trials conducted
21. Myasthenia GravisAnticholinesterase drugs impair the degradation of
22. Myasthenia GravisPrednisone often provides rapid improvement in
23. Myasthenia GravisImmunosuppresive drugs may be considered when
24. Myasthenia GravisThymectomy may offer benefit to certain
25. Peripheral neuropathies can be subdivided broadly into
26. NeuropathyRadial nerve palsyCompression of radial nerve in spiral groove of humerus“Saturday night palsy” or “Honeymoon palsy”
27. NeuropathyCarpal tunnel syndromeCompression of medial nerve between
28. NeuropathyAtrophy of thenar eminence can occur laterRisk
29. Polyneuropathies lead to symmetric sensory, motor or
30. NeuropathyCharcot-Marie-ToothAutosomal dominant inheritanceOften presents with foot deformities
31. NeuropathyDiabetic neuropathy commonly occurs in stocking-glove distributionPositive and negative symptoms may occur alternately
32. NeuropathyToxic neuropathies may result from exposure to
33. Aside from a thorough history, serologic studies
34. Treatment involves addressing underlying causePharmacologic treatment can
35. Acute or subacute, progressive polyradiculoneuropathy, often preceded
36. DiagnosisAscending weakness, primarily motor symptomsMuscles of respiration
37. TreatmentClose monitoring of respiratory functionPreventive measures –
38. Sudden onset of facial paresis caused by
39. Bell’s PalsyAbrupt facial paralysis that gradually worsensEar
40. Routine labs or diagnostic imaging not necessary
41. Amyotrophic lateral sclerosis (ALS) "Lou Gehrig's disease"A-myo-trophic=no
42. ALSIncidence higher in menOnset from teenage years
43. Increasing muscle weakness, especially involving the arms
44. ALSRiluzole is only available treatment; it may
45. May occur as a primary (idiopathic) disorderOccurs
46. RLSIncidence twice as high in femalesCan occur
47. Thought to have genetic componentDopamine is thought
48. Avoidance of caffeine, nicotine, ETOHTreat deficiencies (Fe, Mg, folate)Sleep hygieneExerciseMassagePharmacotherapy with dopaminergics (Mirapex, Requip)RLS
49. Скачать презентанцию

Brain trick

Слайды и текст этой презентации

Слайд 1Neuro Potpourri
Gerald F. Smith, MPH, MS, PA-C
Neurology and Neurophysiology Associates
Philadelphia

University
Spring 2015

Слайд 2Brain trick

Слайд 3Neuro goes to the movies

Слайд 4Myasthenia Gravis

Neuropathies

ALS

Restless Legs Syndrome
Neuro Potporrui

Слайд 5Chronic autoimmune neuromuscular disease

Characterized by varying degrees of weakness

of skeletal muscles

Name literally means “grave muscle weakness”

Myasthenia Gravis

Chronic autoimmune neuromuscular disease Characterized by varying degrees of weakness of skeletal musclesName literally means “grave muscle

Слайд 6Prevalence estimated at 14-20 cases per 100,000 population (36,000-60,000 cases)
Most

likely underdiagnosed
Age of onset in females typically between age 20-30;

males 70-80, with more males affected

Myasthenia Gravis

Prevalence estimated at 14-20 cases per 100,000 population (36,000-60,000 cases)Most likely underdiagnosedAge of onset in females typically

Слайд 7Caused by defective neuromuscular transmission
Acetylcholine is the neurotransmitter involved
Both anatomic

and physiologic changes involved

Myasthenia Gravis

Caused by defective neuromuscular transmissionAcetylcholine is the neurotransmitter involvedBoth anatomic and physiologic changes involvedMyasthenia Gravis

Слайд 8Myasthenia Gravis
In normal neuromuscular transmission, Ach released from motor nerve

terminal in quanta
Travel across neuromuscular junction to receptors
Binding to receptors

causes muscle contraction

Myasthenia GravisIn normal neuromuscular transmission, Ach released from motor nerve terminal in quantaTravel across neuromuscular junction to

Слайд 9Myasthenia Gravis
In MG, several factors contribute to defective neuromuscular transmission

Antibodies

block or destroy Ach receptors on the muscle end-plate

Myasthenia GravisIn MG, several factors contribute to defective neuromuscular transmissionAntibodies block or destroy Ach receptors on the

Слайд 10Myasthenia Gravis
In MG, the post-synpatic membrane morphology is changed, having

lost it normal folded shape

Also, the number of Ach receptors

on the muscle end-plate is reduced

Myasthenia GravisIn MG, the post-synpatic membrane morphology is changed, having lost it normal folded shapeAlso, the number

Слайд 11Myasthenia Gravis
Thymus gland plays a role in MG
10% of MG

patients have thymus tumor (mostly benign)
Lymphoid hyperplasia allows interaction of

B- cells with helper T-cells to form antibodies

Myasthenia GravisThymus gland plays a role in MG10% of MG patients have thymus tumor (mostly benign)Lymphoid hyperplasia

Слайд 12Myasthenia Gravis
As a result of these changes to the post-synaptic

membrane, the effect of Ach is reduced

Resultant effect is greatly

reduced or blocked stimulation of muscle contraction

Myasthenia GravisAs a result of these changes to the post-synaptic membrane, the effect of Ach is reducedResultant

Слайд 13Myasthenia Gravis
Unusual distribution of symptoms often presents puzzling clinical picture
Classic

symptoms include: drooping of eyelids, diplopia, dysphagia, and dysarthria
These symptoms

are sometimes pursued as intracranial in origin

Myasthenia GravisUnusual distribution of symptoms often presents puzzling clinical pictureClassic symptoms include: drooping of eyelids, diplopia, dysphagia,

Слайд 14Myasthenia Gravis
Muscle weakness, particularly in the extremities, is a common

symptom
The muscle weakness becomes more severe as the day progresses
Rest

will often temporarily improve muscle weakness

Myasthenia GravisMuscle weakness, particularly in the extremities, is a common symptomThe muscle weakness becomes more severe as

Слайд 15Myasthenia Gravis
Muscles which control breathing, swallowing (bulbar) may be affected
Myasthenic

crisis occurs when these muscles weaken to the point where

ventilation or eating/drinking is impaired
Crisis can be triggered by infection, fever, or drugs

Myasthenia GravisMuscles which control breathing, swallowing (bulbar) may be affectedMyasthenic crisis occurs when these muscles weaken to

Слайд 16Myasthenia Gravis
Several classes of ABX: aminoglycosides, quinolones, macrolides
Antihypertensives: B-blockers and

CCB
Lithium
Magnesium (including Maalox, Mylanta)

Myasthenia GravisSeveral classes of ABX: aminoglycosides, quinolones, macrolidesAntihypertensives: B-blockers and CCBLithiumMagnesium (including Maalox, Mylanta)

Слайд 17Myasthenia Gravis
Tensilon test: IV administration of this drug, which blocks

degradation of Ach, provides for improvement in muscle weakness
Therapeutic trial

of oral pyridostigmine may be utilized

Myasthenia GravisTensilon test: IV administration of this drug, which blocks degradation of Ach, provides for improvement in

Слайд 18Myasthenia Gravis
Antibody testing is utilized to detect Anti-Ach receptor antibodies
Nearly

75% of patients with generalized MG have serum antibodies to

Ach receptors
Antibody concentration may be increased in SLE, ALS, and inflammatory neuropathy

Myasthenia GravisAntibody testing is utilized to detect Anti-Ach receptor antibodiesNearly 75% of patients with generalized MG have

Слайд 19Myasthenia Gravis
EMG testing, using repetitive nerve stimulation, shows decremental change

in amplitude of muscle response
Not specific to MG
May be normal

in mild cases

Myasthenia GravisEMG testing, using repetitive nerve stimulation, shows decremental change in amplitude of muscle responseNot specific to

Слайд 20Myasthenia Gravis
Limited number of clinical trials conducted to evaluate treatment

of MG
Treatment should be tailored to severity of symptoms
Response to

treatment can be hard to assess, as symptoms fluctuate

Myasthenia GravisLimited number of clinical trials conducted to evaluate treatment of MGTreatment should be tailored to severity

Слайд 21Myasthenia Gravis
Anticholinesterase drugs impair the degradation of Ach (Mestinon)
Allow Ach

to accumulate at the neuromuscular junction
Response is variable
GI side effects

common CNS effects rare

Myasthenia GravisAnticholinesterase drugs impair the degradation of Ach (Mestinon)Allow Ach to accumulate at the neuromuscular junctionResponse is

Слайд 22Myasthenia Gravis
Prednisone often provides rapid improvement in symptoms
Most effective in

patients with recent onset of symptoms
Approx. 1/3 of patients will

become weaker initially

Myasthenia GravisPrednisone often provides rapid improvement in symptomsMost effective in patients with recent onset of symptomsApprox. 1/3

Слайд 23Myasthenia Gravis
Immunosuppresive drugs may be considered when other therapies have

produced sub-optimal responses

Azathioprine, Cyclosporine, and Cyclophosphamide have been used with

varying levels of success

Cost and side effects related to these agents are important considerations

Myasthenia GravisImmunosuppresive drugs may be considered when other therapies have produced sub-optimal responsesAzathioprine, Cyclosporine, and Cyclophosphamide have

Слайд 24Myasthenia Gravis
Thymectomy may offer benefit to certain patient populations, in

particular younger patients earlier in the course of their disease
Thymoma

is an absolute indication
Elective thymectomy in moderate to severe generalized MG

Myasthenia GravisThymectomy may offer benefit to certain patient populations, in particular younger patients earlier in the course

Слайд 25Peripheral neuropathies can be subdivided broadly into mononeuropathies and polyneuropathies
Mononeuropathies

result from injury to nerve along its course or by

compression, angulation or stretching of the nerve
Entrapment neuropathies occur when the nerve course passes through a narrow anatomical space

Neuropathy

Peripheral neuropathies can be subdivided broadly into mononeuropathies and polyneuropathiesMononeuropathies result from injury to nerve along its

Слайд 26Neuropathy
Radial nerve palsy
Compression of radial nerve in spiral groove of

humerus
“Saturday night palsy” or “Honeymoon palsy”

Слайд 27Neuropathy
Carpal tunnel syndrome
Compression of medial nerve between transverse carpal ligament

and deeper structures of wrist
Pain, burning or tingling in distribution

of median nerve

NeuropathyCarpal tunnel syndromeCompression of medial nerve between transverse carpal ligament and deeper structures of wristPain, burning or

Слайд 28Neuropathy

Atrophy of thenar eminence can occur later
Risk factors include repetitive

motions of hands, pregnancy, and secondarily in conditions such as

DM, RA and hyperparathyroidism
Diagnosis is clinical with support from EMG and NCT
Treatment includes ergonomic modification, splinting, NSAIDS, corticosteroid injections, and surgery

NeuropathyAtrophy of thenar eminence can occur laterRisk factors include repetitive motions of hands, pregnancy, and secondarily in

Слайд 29Polyneuropathies lead to symmetric sensory, motor or mixed deficit
Most prominent

distally
May be broadly classified as hereditary, metabolic or toxic
Pain may

be prominent symptom
In approximately 50% no specific cause is identified

Neuropathy

Polyneuropathies lead to symmetric sensory, motor or mixed deficitMost prominent distallyMay be broadly classified as hereditary, metabolic

Слайд 30Neuropathy
Charcot-Marie-Tooth
Autosomal dominant inheritance
Often presents with foot deformities or gait disturbance

in childhood or early adulthood
Progresses to distal weakness and sensory

loss

NeuropathyCharcot-Marie-ToothAutosomal dominant inheritanceOften presents with foot deformities or gait disturbance in childhood or early adulthoodProgresses to distal

Слайд 31Neuropathy
Diabetic neuropathy commonly occurs in stocking-glove distribution
Positive and negative symptoms

may occur alternately

Слайд 32Neuropathy
Toxic neuropathies may result from exposure to various agents, including

organophosphates, heavy metals, and certain drugs (phenytoin, isoniazid, nitrofurantoin)
Occupational history

and identification of clusters may help in diagnosis

NeuropathyToxic neuropathies may result from exposure to various agents, including organophosphates, heavy metals, and certain drugs (phenytoin,

Слайд 33Aside from a thorough history, serologic studies are an important

part of the work-up of polyneuropathies
GTT
ANA
ACE
B6, B12, folate
Copper
Lyme titer
HIV
RPR
SS-A, SS-B,

C-ANCA, P-ANCA
Heavy metals (if suspected)

Neuropathy

Aside from a thorough history, serologic studies are an important part of the work-up of polyneuropathiesGTTANAACEB6, B12,

Слайд 34Treatment involves addressing underlying cause
Pharmacologic treatment can involve:
NSAIDS
Antidepressants (Effexor, Cymbalta,

amitriptyline)
Anti-epileptics (Neurontin, Lyrica, carbamazepine)
Opioids in severe pain
Neuropathy

Treatment involves addressing underlying causePharmacologic treatment can involve:NSAIDSAntidepressants (Effexor, Cymbalta, amitriptyline)Anti-epileptics (Neurontin, Lyrica, carbamazepine)Opioids in severe painNeuropathy

Слайд 35Acute or subacute, progressive polyradiculoneuropathy, often preceded by an infection,

immunization or surgery
Associated with Campylobacter jejuni enteritis.
Inflammatory response that affects

the spinal root, peripheral & extra axial cranial nerves
Exact mechanism is unclear

Guillain-Barré Syndrome

Acute or subacute, progressive polyradiculoneuropathy, often preceded by an infection, immunization or surgeryAssociated with Campylobacter jejuni enteritis.Inflammatory

Слайд 36Diagnosis
Ascending weakness, primarily motor symptoms
Muscles of respiration and deglutition may

be affected
Autonomic component (tachy, hypo/HTN)
Elevated protein level in the CSF
EMG

– axonal loss, delayed F-waves
CXR, stool cultures

Guillain-Barré Syndrome

DiagnosisAscending weakness, primarily motor symptomsMuscles of respiration and deglutition may be affectedAutonomic component (tachy, hypo/HTN)Elevated protein level

Слайд 37Treatment
Close monitoring of respiratory function
Preventive measures – wound care &

DVT/PE prophylaxis
Plasma exchange & IV immunoglobulins
Prognosis
75% completely recover within

3-6 months
20% with permanent deficit

Guillain-Barré Syndrome

TreatmentClose monitoring of respiratory functionPreventive measures – wound care & DVT/PE prophylaxisPlasma exchange & IV immunoglobulinsPrognosis 75%

Слайд 38Sudden onset of facial paresis caused by an inflammatory response

involving the facial nerve
Idiopathic, although theorized to be caused by

reactivation of HSV type 1 or varicella virus
More common in patients with DM and in pregnancy

Bell’s Palsy

Sudden onset of facial paresis caused by an inflammatory response involving the facial nerveIdiopathic, although theorized to

Слайд 39Bell’s Palsy
Abrupt facial paralysis that gradually worsens
Ear pain on the

affected side
Numbness sensation
Difficulty eating
Excessive tearing
Difficulty closing ipsilateral eye
Poor fine facial

movements

Bell’s PalsyAbrupt facial paralysis that gradually worsensEar pain on the affected sideNumbness sensationDifficulty eatingExcessive tearingDifficulty closing ipsilateral

Слайд 40Routine labs or diagnostic imaging not necessary for diagnosis unless

clinical suspicion of underlying cause (herpes zoster, Lyme Disease, HIV,

tumor)
Treatment is controversial, as 60% of patients resolve completely without it
Steroids may have an impact: 2 week taper
Antivirals?
Eye drops and patching

Bell’s Palsy

Routine labs or diagnostic imaging not necessary for diagnosis unless clinical suspicion of underlying cause (herpes zoster,

Слайд 41Amyotrophic lateral sclerosis (ALS) "Lou Gehrig's disease"
A-myo-trophic=no muscle nourishment: leads

to atrophy
Lateral: area of spinal cord where portions of

the nerve cells that signal and control the muscles are located
Sclerosis: As this area degenerates it leads to scarring
Progressive neurodegenerative disease that affects motor neurons in the brain and the spinal cord.
The progressive degeneration of the motor neurons in ALS eventually lead to their death.
Death of motor neurons causes loss of ability of the brain to initiate and control muscle movement

Amyotrophic Lateral Sclerosis

Слайд 42ALS
Incidence higher in men
Onset from teenage years to eight decade;

average 60 in sporadic; 50 in inherited
Estimated lifetime risk for

men 1 in 350, 1 in 420 for women
Paradoxically, earlier diagnosis more likely to lead to long-term survival

ALSIncidence higher in menOnset from teenage years to eight decade; average 60 in sporadic; 50 in inheritedEstimated

Слайд 43Increasing muscle weakness, especially involving the arms and legs
Difficulty

with speech, swallowing or breathing (bulbar)
Atrophy of affected limbs
Progresses

to complete paralysis
Cognitive decline, pseudobulbar affect and parkinsonism may occur.

ALS

Increasing muscle weakness, especially involving the arms and legs Difficulty with speech, swallowing or breathing (bulbar)Atrophy of

Слайд 44ALS
Riluzole is only available treatment; it may slow progression (2-3

mos)
Supportive care, therapy are mainstays of treatment
Life expectancy 2-5 years

from diagnosis

ALSRiluzole is only available treatment; it may slow progression (2-3 mos)Supportive care, therapy are mainstays of treatmentLife

Слайд 45May occur as a primary (idiopathic) disorder
Occurs secondarily in pregnancy,

Fe deficiency anemia, peripheral neuropathy
Restlessness and sensory disturbance lead to

irresistible urge to move legs, especially during periods of relaxation
Disturbed nocturnal sleep and excessive daytime somnolence may result

Restless Legs Syndrome

May occur as a primary (idiopathic) disorderOccurs secondarily in pregnancy, Fe deficiency anemia, peripheral neuropathyRestlessness and sensory

Слайд 46RLS
Incidence twice as high in females
Can occur at any age;

most common in middle age
Typically bilateral
Can affect arms, trunk and

head, but less commonly
ETOH can exacerbate problem

RLSIncidence twice as high in femalesCan occur at any age; most common in middle ageTypically bilateralCan affect

Слайд 47Thought to have genetic component
Dopamine is thought to play a

role, as RLS is often seen in PD, which effects

basal ganglia (dopamine dependent)
Typically progresses in severity over time
Patients may experience periods of remission

RLS

Thought to have genetic componentDopamine is thought to play a role, as RLS is often seen in

Слайд 48Avoidance of caffeine, nicotine, ETOH
Treat deficiencies (Fe, Mg, folate)
Sleep hygiene
Exercise
Massage
Pharmacotherapy

with dopaminergics (Mirapex, Requip)
RLS

Avoidance of caffeine, nicotine, ETOHTreat deficiencies (Fe, Mg, folate)Sleep hygieneExerciseMassagePharmacotherapy with dopaminergics (Mirapex, Requip)RLS

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Neuro Potpourri

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Слайд 1Neuro PotpourriGerald F. Smith, MPH, MS, PA-CNeurology and Neurophysiology AssociatesPhiladelphia

UniversitySpring 2015

Слайд 2Brain trick

Слайд 3Neuro goes to the movies

Слайд 4Myasthenia GravisNeuropathiesALSRestless Legs SyndromeNeuro Potporrui

Слайд 5Chronic autoimmune neuromuscular disease Characterized by varying degrees of weakness

of skeletal musclesName literally means “grave muscle weakness”Myasthenia Gravis

Слайд 6Prevalence estimated at 14-20 cases per 100,000 population (36,000-60,000 cases)Most

likely underdiagnosedAge of onset in females typically between age 20-30;

Слайд 7Caused by defective neuromuscular transmissionAcetylcholine is the neurotransmitter involvedBoth anatomic

and physiologic changes involvedMyasthenia Gravis

Слайд 8Myasthenia GravisIn normal neuromuscular transmission, Ach released from motor nerve

terminal in quantaTravel across neuromuscular junction to receptorsBinding to receptors

Слайд 9Myasthenia GravisIn MG, several factors contribute to defective neuromuscular transmissionAntibodies

block or destroy Ach receptors on the muscle end-plate

Слайд 10Myasthenia GravisIn MG, the post-synpatic membrane morphology is changed, having

lost it normal folded shapeAlso, the number of Ach receptors

Слайд 11Myasthenia GravisThymus gland plays a role in MG10% of MG

patients have thymus tumor (mostly benign)Lymphoid hyperplasia allows interaction of

Слайд 12Myasthenia GravisAs a result of these changes to the post-synaptic

membrane, the effect of Ach is reducedResultant effect is greatly

Слайд 13Myasthenia GravisUnusual distribution of symptoms often presents puzzling clinical pictureClassic

symptoms include: drooping of eyelids, diplopia, dysphagia, and dysarthriaThese symptoms

Слайд 14Myasthenia GravisMuscle weakness, particularly in the extremities, is a common

symptomThe muscle weakness becomes more severe as the day progressesRest

Слайд 15Myasthenia GravisMuscles which control breathing, swallowing (bulbar) may be affectedMyasthenic

crisis occurs when these muscles weaken to the point where

Слайд 16Myasthenia GravisSeveral classes of ABX: aminoglycosides, quinolones, macrolidesAntihypertensives: B-blockers and

CCBLithiumMagnesium (including Maalox, Mylanta)

Слайд 17Myasthenia GravisTensilon test: IV administration of this drug, which blocks

degradation of Ach, provides for improvement in muscle weaknessTherapeutic trial

Слайд 18Myasthenia GravisAntibody testing is utilized to detect Anti-Ach receptor antibodiesNearly

75% of patients with generalized MG have serum antibodies to

Слайд 19Myasthenia GravisEMG testing, using repetitive nerve stimulation, shows decremental change

in amplitude of muscle responseNot specific to MGMay be normal

Слайд 20Myasthenia GravisLimited number of clinical trials conducted to evaluate treatment

of MGTreatment should be tailored to severity of symptomsResponse to

Слайд 21Myasthenia GravisAnticholinesterase drugs impair the degradation of Ach (Mestinon)Allow Ach

to accumulate at the neuromuscular junctionResponse is variableGI side effects

Слайд 22Myasthenia GravisPrednisone often provides rapid improvement in symptomsMost effective in

patients with recent onset of symptomsApprox. 1/3 of patients will

Слайд 23Myasthenia GravisImmunosuppresive drugs may be considered when other therapies have

produced sub-optimal responsesAzathioprine, Cyclosporine, and Cyclophosphamide have been used with

Слайд 24Myasthenia GravisThymectomy may offer benefit to certain patient populations, in

particular younger patients earlier in the course of their diseaseThymoma

Слайд 25Peripheral neuropathies can be subdivided broadly into mononeuropathies and polyneuropathiesMononeuropathies

result from injury to nerve along its course or by

Слайд 26NeuropathyRadial nerve palsyCompression of radial nerve in spiral groove of

humerus“Saturday night palsy” or “Honeymoon palsy”

Слайд 27NeuropathyCarpal tunnel syndromeCompression of medial nerve between transverse carpal ligament

and deeper structures of wristPain, burning or tingling in distribution

Слайд 28NeuropathyAtrophy of thenar eminence can occur laterRisk factors include repetitive

motions of hands, pregnancy, and secondarily in conditions such as

Слайд 29Polyneuropathies lead to symmetric sensory, motor or mixed deficitMost prominent

distallyMay be broadly classified as hereditary, metabolic or toxicPain may

Слайд 30NeuropathyCharcot-Marie-ToothAutosomal dominant inheritanceOften presents with foot deformities or gait disturbance

in childhood or early adulthoodProgresses to distal weakness and sensory

Слайд 31NeuropathyDiabetic neuropathy commonly occurs in stocking-glove distributionPositive and negative symptoms

may occur alternately

Слайд 32NeuropathyToxic neuropathies may result from exposure to various agents, including

organophosphates, heavy metals, and certain drugs (phenytoin, isoniazid, nitrofurantoin)Occupational history

Слайд 33Aside from a thorough history, serologic studies are an important

part of the work-up of polyneuropathiesGTTANAACEB6, B12, folateCopperLyme titerHIVRPRSS-A, SS-B,

Слайд 34Treatment involves addressing underlying causePharmacologic treatment can involve:NSAIDSAntidepressants (Effexor, Cymbalta,

amitriptyline)Anti-epileptics (Neurontin, Lyrica, carbamazepine)Opioids in severe painNeuropathy

Слайд 35Acute or subacute, progressive polyradiculoneuropathy, often preceded by an infection,

immunization or surgeryAssociated with Campylobacter jejuni enteritis.Inflammatory response that affects

Слайд 36DiagnosisAscending weakness, primarily motor symptomsMuscles of respiration and deglutition may

be affectedAutonomic component (tachy, hypo/HTN)Elevated protein level in the CSFEMG

Слайд 37TreatmentClose monitoring of respiratory functionPreventive measures – wound care &

DVT/PE prophylaxisPlasma exchange & IV immunoglobulinsPrognosis 75% completely recover within

Слайд 38Sudden onset of facial paresis caused by an inflammatory response

involving the facial nerveIdiopathic, although theorized to be caused by

Слайд 39Bell’s PalsyAbrupt facial paralysis that gradually worsensEar pain on the

affected sideNumbness sensationDifficulty eatingExcessive tearingDifficulty closing ipsilateral eyePoor fine facial

Слайд 40Routine labs or diagnostic imaging not necessary for diagnosis unless

Слайд 1Neuro Potpourri
Gerald F. Smith, MPH, MS, PA-C
Neurology and Neurophysiology Associates
Philadelphia

University
Spring 2015

Слайд 4Myasthenia Gravis

Neuropathies

ALS

Restless Legs Syndrome
Neuro Potporrui

Слайд 5Chronic autoimmune neuromuscular disease

Characterized by varying degrees of weakness

of skeletal muscles

Name literally means “grave muscle weakness”

Myasthenia Gravis

Слайд 6Prevalence estimated at 14-20 cases per 100,000 population (36,000-60,000 cases)
Most

likely underdiagnosed
Age of onset in females typically between age 20-30;

Слайд 7Caused by defective neuromuscular transmission
Acetylcholine is the neurotransmitter involved
Both anatomic

and physiologic changes involved

Myasthenia Gravis

Слайд 8Myasthenia Gravis
In normal neuromuscular transmission, Ach released from motor nerve

terminal in quanta
Travel across neuromuscular junction to receptors
Binding to receptors

Слайд 9Myasthenia Gravis
In MG, several factors contribute to defective neuromuscular transmission

Antibodies

Слайд 10Myasthenia Gravis
In MG, the post-synpatic membrane morphology is changed, having

lost it normal folded shape

Also, the number of Ach receptors

Слайд 11Myasthenia Gravis
Thymus gland plays a role in MG
10% of MG

patients have thymus tumor (mostly benign)
Lymphoid hyperplasia allows interaction of

Слайд 12Myasthenia Gravis
As a result of these changes to the post-synaptic

membrane, the effect of Ach is reduced

Resultant effect is greatly

Слайд 13Myasthenia Gravis
Unusual distribution of symptoms often presents puzzling clinical picture
Classic

symptoms include: drooping of eyelids, diplopia, dysphagia, and dysarthria
These symptoms

Слайд 14Myasthenia Gravis
Muscle weakness, particularly in the extremities, is a common

symptom
The muscle weakness becomes more severe as the day progresses
Rest

Слайд 15Myasthenia Gravis
Muscles which control breathing, swallowing (bulbar) may be affected
Myasthenic

Слайд 16Myasthenia Gravis
Several classes of ABX: aminoglycosides, quinolones, macrolides
Antihypertensives: B-blockers and

CCB
Lithium
Magnesium (including Maalox, Mylanta)

Слайд 17Myasthenia Gravis
Tensilon test: IV administration of this drug, which blocks

degradation of Ach, provides for improvement in muscle weakness
Therapeutic trial

Слайд 18Myasthenia Gravis
Antibody testing is utilized to detect Anti-Ach receptor antibodies
Nearly

Слайд 19Myasthenia Gravis
EMG testing, using repetitive nerve stimulation, shows decremental change

in amplitude of muscle response
Not specific to MG
May be normal

Слайд 20Myasthenia Gravis
Limited number of clinical trials conducted to evaluate treatment

of MG
Treatment should be tailored to severity of symptoms
Response to

Слайд 21Myasthenia Gravis
Anticholinesterase drugs impair the degradation of Ach (Mestinon)
Allow Ach

to accumulate at the neuromuscular junction
Response is variable
GI side effects

Слайд 22Myasthenia Gravis
Prednisone often provides rapid improvement in symptoms
Most effective in

patients with recent onset of symptoms
Approx. 1/3 of patients will

Слайд 23Myasthenia Gravis
Immunosuppresive drugs may be considered when other therapies have

produced sub-optimal responses

Azathioprine, Cyclosporine, and Cyclophosphamide have been used with

Слайд 24Myasthenia Gravis
Thymectomy may offer benefit to certain patient populations, in

particular younger patients earlier in the course of their disease
Thymoma

Слайд 25Peripheral neuropathies can be subdivided broadly into mononeuropathies and polyneuropathies
Mononeuropathies

Слайд 26Neuropathy
Radial nerve palsy
Compression of radial nerve in spiral groove of

humerus
“Saturday night palsy” or “Honeymoon palsy”

Слайд 27Neuropathy
Carpal tunnel syndrome
Compression of medial nerve between transverse carpal ligament

and deeper structures of wrist
Pain, burning or tingling in distribution

Слайд 28Neuropathy

Atrophy of thenar eminence can occur later
Risk factors include repetitive

Слайд 29Polyneuropathies lead to symmetric sensory, motor or mixed deficit
Most prominent

distally
May be broadly classified as hereditary, metabolic or toxic
Pain may

Слайд 30Neuropathy
Charcot-Marie-Tooth
Autosomal dominant inheritance
Often presents with foot deformities or gait disturbance

in childhood or early adulthood
Progresses to distal weakness and sensory

Слайд 31Neuropathy
Diabetic neuropathy commonly occurs in stocking-glove distribution
Positive and negative symptoms

Слайд 32Neuropathy
Toxic neuropathies may result from exposure to various agents, including

organophosphates, heavy metals, and certain drugs (phenytoin, isoniazid, nitrofurantoin)
Occupational history

part of the work-up of polyneuropathies
GTT
ANA
ACE
B6, B12, folate
Copper
Lyme titer
HIV
RPR
SS-A, SS-B,

Слайд 34Treatment involves addressing underlying cause
Pharmacologic treatment can involve:
NSAIDS
Antidepressants (Effexor, Cymbalta,

amitriptyline)
Anti-epileptics (Neurontin, Lyrica, carbamazepine)
Opioids in severe pain
Neuropathy

immunization or surgery
Associated with Campylobacter jejuni enteritis.
Inflammatory response that affects

Слайд 36Diagnosis
Ascending weakness, primarily motor symptoms
Muscles of respiration and deglutition may

be affected
Autonomic component (tachy, hypo/HTN)
Elevated protein level in the CSF
EMG

Слайд 37Treatment
Close monitoring of respiratory function
Preventive measures – wound care &

DVT/PE prophylaxis
Plasma exchange & IV immunoglobulins
Prognosis
75% completely recover within

involving the facial nerve
Idiopathic, although theorized to be caused by

Слайд 39Bell’s Palsy
Abrupt facial paralysis that gradually worsens
Ear pain on the

affected side
Numbness sensation
Difficulty eating
Excessive tearing
Difficulty closing ipsilateral eye
Poor fine facial

Слайд 41Amyotrophic lateral sclerosis (ALS) "Lou Gehrig's disease"
A-myo-trophic=no muscle nourishment: leads

to atrophy
Lateral: area of spinal cord where portions of

Слайд 42ALS
Incidence higher in men
Onset from teenage years to eight decade;

average 60 in sporadic; 50 in inherited
Estimated lifetime risk for

Слайд 43Increasing muscle weakness, especially involving the arms and legs
Difficulty

with speech, swallowing or breathing (bulbar)
Atrophy of affected limbs
Progresses

Слайд 44ALS
Riluzole is only available treatment; it may slow progression (2-3

mos)
Supportive care, therapy are mainstays of treatment
Life expectancy 2-5 years

Слайд 45May occur as a primary (idiopathic) disorder
Occurs secondarily in pregnancy,

Fe deficiency anemia, peripheral neuropathy
Restlessness and sensory disturbance lead to

Слайд 46RLS
Incidence twice as high in females
Can occur at any age;

most common in middle age
Typically bilateral
Can affect arms, trunk and

Слайд 47Thought to have genetic component
Dopamine is thought to play a

Слайд 48Avoidance of caffeine, nicotine, ETOH
Treat deficiencies (Fe, Mg, folate)
Sleep hygiene
Exercise
Massage
Pharmacotherapy

with dopaminergics (Mirapex, Requip)
RLS