Dr. Trumbly, September 2007
Transcript of Dr. Trumbly, September 2007
CPC: A Paralysis CPC: A Paralysis Quandary for a Quandary for a
DimeDimeAlan Trumbly, DOAlan Trumbly, DO
ObjectivesObjectives
Case PresentationCase Presentation Problem ListProblem List Locate the LesionLocate the Lesion Develop DifferentialDevelop Differential Relate Differential to patientRelate Differential to patient Cross fingers and choose…Cross fingers and choose…
PatientPatient CC: Progressive Upper Extremity CC: Progressive Upper Extremity
Weakness.Weakness. HPI:HPI:
70 y.o. Right handed, Hispanic Male.70 y.o. Right handed, Hispanic Male. 2 weeks ago developed fever, chills, nausea, 2 weeks ago developed fever, chills, nausea,
vomiting, abdominal cramping, diarrhea. vomiting, abdominal cramping, diarrhea. Symptoms resolved in 24-36 hours.Symptoms resolved in 24-36 hours.
2 Days PTA: Left upper extremity numbness, 2 Days PTA: Left upper extremity numbness, tingling, and weakness.tingling, and weakness.
1Day PTA: Continued to progressively 1Day PTA: Continued to progressively worsening, and by the PM both upper worsening, and by the PM both upper extremities involved.extremities involved.
Transferred to S/W on day of admission for Transferred to S/W on day of admission for further neurological workup.further neurological workup.
PatientPatient HPI: On day of admissionHPI: On day of admission
Both arms quite weak.Both arms quite weak. Dull aching neck pain, especially with Dull aching neck pain, especially with
rotation to the right.rotation to the right. NO diploplia, ptosis, dysarthria, dysphasia, NO diploplia, ptosis, dysarthria, dysphasia,
shortness of breath, lower extremity shortness of breath, lower extremity weakness, or bowel or bladder incontinence.weakness, or bowel or bladder incontinence.
NO pain in limbs or back.NO pain in limbs or back. NO prior neurological history, except NO prior neurological history, except
lumbar laminectomy for Left L4-L5 lumbar laminectomy for Left L4-L5 radiculopathy.radiculopathy.
PatientPatient
PMH:PMH: HypertensionHypertension HyperlipidemiaHyperlipidemia Diabetes: good blood sugar control, no Diabetes: good blood sugar control, no
neuropathy.neuropathy. Fam Hx: negative for neurological disease.Fam Hx: negative for neurological disease. Soc Hx: No tobacco or alcohol.Soc Hx: No tobacco or alcohol. Allergies: noneAllergies: none Medications: noneMedications: none
PatientPatient Vitals: AfebrileVitals: Afebrile PExam:PExam:
Gen: pleasant, straight-Gen: pleasant, straight-forward, Hispanic man, forward, Hispanic man, upset, but no distress, upset, but no distress, alert and oriented with no alert and oriented with no dysarthria.dysarthria.
CN: visual fields full, CN: visual fields full, pupils react 3 to 2 mm, no pupils react 3 to 2 mm, no papilledema, ocular papilledema, ocular motility full, no motility full, no nystagmus. Mild left facial nystagmus. Mild left facial weakness. Masseter and weakness. Masseter and temporalis strength and temporalis strength and bulk, as well as pterygoid bulk, as well as pterygoid strength all normal. strength all normal.
PatientPatient PExam:PExam:
Palate elevated briskly midline. Palate elevated briskly midline. Right deviation of the tongue with protrusion.Right deviation of the tongue with protrusion. Left SCM and Trapezius were weak.Left SCM and Trapezius were weak. Right SCM and Trapezius were normal.Right SCM and Trapezius were normal. Motor: no fasciculations or atrophyMotor: no fasciculations or atrophy Upper ext: Deltoids 2/5 bilaterally, Triceps 4-/5 Upper ext: Deltoids 2/5 bilaterally, Triceps 4-/5
right, and 3/5 on the left. Finger and wrist right, and 3/5 on the left. Finger and wrist extensors 0/5extensors 0/5
Reflexes: Absent in the upper extremities, , Reflexes: Absent in the upper extremities, , trace at the right knee, absent at the ankles. trace at the right knee, absent at the ankles. Plantar responses were neutral bilaterally. Plantar responses were neutral bilaterally.
PatientPatient
MotorMotor:: ReflexesReflexes::
Finger and wrist extentsors 0/5 bilaterally.
Interosseous muscles 2/5 bilaterally.
Hand Grips 3/5 bilaterally.
Foot Dorsiflexion:4+/5 Right, and 4/5 left
Quads 5/5Right hip
flexor 4+/5 and left 5/5
Triceps 4-/5 right, 3/5 left.Deltoids 2/5 bilaterally
ABSENT at ankles
Trace at right knee
ABSENT in upper
extremities
PatientPatient
PExam:PExam: All sensory modalities reported normal.All sensory modalities reported normal. Plantar responses neutral bilaterally.Plantar responses neutral bilaterally. Gait: not testable.Gait: not testable. Cerebellar: not testable.Cerebellar: not testable.
Labs:Labs:142
4.2
103
30
13
1.0
Tot Bili: 0.8Alk Ph: 90AST: 23ALT: 32Tot Prot: 7.0Alb: 4.1
13.75.8 208
38.9
MCV: 84.4
Our MissionOur Mission
Additional studies were done upon Additional studies were done upon arrival here and a diagnosis was arrival here and a diagnosis was made???made???
Problem ListProblem List
Subacute (days) Progressive Upper Subacute (days) Progressive Upper Extremity Weakness Extremity Weakness
Absent Muscle Stretch Reflexes Absent Muscle Stretch Reflexes Rightward deviation of the tongue, and Rightward deviation of the tongue, and
Left SCM and Trapezius were weakLeft SCM and Trapezius were weak Recent Diarrhea Illness (2 weeks prior)Recent Diarrhea Illness (2 weeks prior) DiabetesDiabetes HypertensionHypertension Hx of Lumbar laminectomyHx of Lumbar laminectomy
FDRFDR
3232ndnd President President Presidency Presidency
spanned the Great spanned the Great Depression of Depression of 1930’s, and most 1930’s, and most of World War II.of World War II.
Only U.S. Only U.S. president to have president to have served more than served more than two terms.two terms.
Location, Location, Location, Location, LocationLocation
What are What are symptoms to look symptoms to look for?for?
What are the What are the locations locations possible?possible?
Signs that Distinguish Signs that Distinguish Patterns of WeaknessPatterns of Weakness
SignSign UMNUMN LMNLMN MyopathicMyopathic
AtrophyAtrophy NoneNone SevereSevere MildMild
FasciculationsFasciculations NoneNone CommonCommon NoneNone
ToneTone SpasticSpastic DecreasedDecreased Norm/DecNorm/Dec
DistributionDistribution Pyramidal/Pyramidal/regionalregional
Distal/segmentalDistal/segmental ProximalProximal
DTRDTR HyperactiveHyperactive Hypoactive/Hypoactive/absentabsent
Normal/Normal/hypoactivehypoactive
BabinskiBabinski PresentPresent AbsentAbsent AbsentAbsent
Location, Location, Location, Location, LocationLocation
HemisphereHemisphere BrainstemBrainstem CerebellumCerebellum CordCord AHCAHC RootRoot PlexusPlexus NerveNerve NMJNMJ MuscleMuscle
Location, Location, Location, Location, LocationLocation
HemisphereHemisphere BrainstemBrainstem CerebellumCerebellum CordCord AHCAHC RootRoot PlexusPlexus NerveNerve NMJNMJ MuscleMuscle
Location, Location, Location, Location, LocationLocation
HemisphereHemisphere BrainstemBrainstem CerebellumCerebellum CordCord AHCAHC RootRoot PlexusPlexus NerveNerve NMJNMJ MuscleMuscle
Location, Location, Location, Location, LocationLocation
HemisphereHemisphere BrainstemBrainstem CerebellumCerebellum CordCord AHCAHC RootRoot PlexusPlexus NerveNerve NMJNMJ MuscleMuscle
FDRFDR
Founded the National Foundation Founded the National Foundation for Infantile Paralysis in the US in for Infantile Paralysis in the US in 1938, which funded rehab programs 1938, which funded rehab programs for victims of paralytic polio and the for victims of paralytic polio and the development of the vaccine.development of the vaccine.
Now known as the March of Dimes.Now known as the March of Dimes.
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord tumorTumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk
Peripheral Peripheral NerveNerve Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent porphyriaAcute intermittent porphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning MuscleMuscle disease: disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning MuscleMuscle disease: disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning MuscleMuscle disease: disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning MuscleMuscle disease: disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning MuscleMuscle disease: disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Myesthenia GravisMyesthenia Gravis Young women and older menYoung women and older men Diplopia, Dysarthria, Dysphagia, DyspneaDiplopia, Dysarthria, Dysphagia, Dyspnea Deficits are fatigable.Deficits are fatigable. Pure muscular weakness without the Pure muscular weakness without the
atrophy, and normal DTR, Sensation, atrophy, and normal DTR, Sensation, Mentation, and Sphincter tone.Mentation, and Sphincter tone.
Lambert-Eaton SyndromeLambert-Eaton Syndrome: : Paraneoplastic, DTR absent but improve Paraneoplastic, DTR absent but improve with exercise, incontinence present, and with exercise, incontinence present, and antibody mediated.antibody mediated.
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
BotulismBotulism Abdominal and GI symptoms preceding Abdominal and GI symptoms preceding
syndrome that resembles myasthenia syndrome that resembles myasthenia gravis. gravis.
Improperly canned foods contaminated Improperly canned foods contaminated with the exotoxin of with the exotoxin of Clostridium botulinum. Clostridium botulinum.
Rapidly developing paralysis usually affects Rapidly developing paralysis usually affects the ocular and cranial musculature first the ocular and cranial musculature first then generalized (Descending).then generalized (Descending).
Toxin-mediated inhibition of acetylcholine Toxin-mediated inhibition of acetylcholine release from axon terminals at NMJ.release from axon terminals at NMJ.
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ NMJ disease:disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Organophosphate Organophosphate PoisoningPoisoning
Miosis, Excessive bodily secretions, Miosis, Excessive bodily secretions, and fasciculations.and fasciculations.
Decreased acetylcholinesterase Decreased acetylcholinesterase activity that causes excessive activity that causes excessive acetylcholine at the NMJ.acetylcholine at the NMJ.
Symptoms vary 5 to 12 hours after Symptoms vary 5 to 12 hours after exposure.exposure.
Tx with atropine.Tx with atropine.
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
FDRFDR August, 10 1921, August, 10 1921,
FDR was 39 y.o. FDR was 39 y.o. fell into Bay of fell into Bay of Fundy, near Fundy, near Campobello, New Campobello, New Brunswick.Brunswick.
1933, elected 1933, elected President of the President of the US with US with symmetrical symmetrical lower extremity lower extremity weakness.weakness.
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Spinal Cord DiseaseSpinal Cord Disease Signs and symptoms occur at affected area and Signs and symptoms occur at affected area and
below.below. Associated with UMN lesions.Associated with UMN lesions. Caused by compressive or noncompressive Caused by compressive or noncompressive
lesion.lesion. Central Cord Syndrome:Central Cord Syndrome:
Most often caused by Most often caused by syringomyeliasyringomyelia and and intramedullary cord tumors. intramedullary cord tumors.
Pathological process starts centrally and proceeds Pathological process starts centrally and proceeds centrifugally, producing motor and sensory signs.centrifugally, producing motor and sensory signs.
Suspended sensory lossSuspended sensory loss: decussating spinothalamic : decussating spinothalamic tract fibers are affected, loss of pain and temperature tract fibers are affected, loss of pain and temperature is bilateral, “cape distribution”>>> “sacral sparring”.is bilateral, “cape distribution”>>> “sacral sparring”.
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHCAHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
diskdisk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Anterior Horn Cell Anterior Horn Cell DiseaseDisease
Cell bodies of peripheral motor nerves. Cell bodies of peripheral motor nerves.
Amyotrophic Lateral SclerosisAmyotrophic Lateral Sclerosis: : Most common, affects AHC and Most common, affects AHC and
corticospinal tracts.corticospinal tracts. Distal bilateral weakness, with atrophy Distal bilateral weakness, with atrophy
and fasciculation's (LMN signs), combined and fasciculation's (LMN signs), combined with bulbar signs, hyper-reflexia, upgoing with bulbar signs, hyper-reflexia, upgoing toes (UMN signs).toes (UMN signs).
Asymmetric limb weakness.Asymmetric limb weakness.
Post-Polio SyndromePost-Polio Syndrome
Major cause of morbidity and death Major cause of morbidity and death throughout the world during the first half throughout the world during the first half of the 20th century. of the 20th century.
Young children characterized by a mild, Young children characterized by a mild, brief febrile illness.brief febrile illness.
Small group would develop fever, HA, Small group would develop fever, HA, meningeal irritation, soreness, and meningeal irritation, soreness, and asymmetric paralysis. asymmetric paralysis.
Introduction of the inactivated polio Introduction of the inactivated polio vaccine in 1954.vaccine in 1954.
West Nile VirusWest Nile Virus RNA flavivirus.RNA flavivirus. Majority asymptomatic, 20% develop febrile Majority asymptomatic, 20% develop febrile
disease, and only 1% will develop disease, and only 1% will develop neuroinvasive disease (aseptic meningitis, neuroinvasive disease (aseptic meningitis, encephalitis, or flaccid paralysis).encephalitis, or flaccid paralysis).
Abrupt onset of fever, headache, myalgia, Abrupt onset of fever, headache, myalgia, weakness, and often, abdominal pain, nausea, weakness, and often, abdominal pain, nausea, vomiting, or diarrhea. vomiting, or diarrhea.
Flaccid paralysis caused by WNV infection is Flaccid paralysis caused by WNV infection is similar clinically and pathologically to similar clinically and pathologically to poliomyelitis caused by poliovirus, with poliomyelitis caused by poliovirus, with damage of anterior horn cells.damage of anterior horn cells.
Acute WeaknessAcute Weakness Spinal Spinal CordCord Disease: Disease:
Transverse MyelitisTransverse Myelitis AHC AHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Transverse MyelitisTransverse Myelitis Inflammatory diseases of the Spinal Cord: Inflammatory diseases of the Spinal Cord:
viral, bacterial, fungal, parasitic, noninfectious.viral, bacterial, fungal, parasitic, noninfectious. Antecedent event that precede symptoms by days to Antecedent event that precede symptoms by days to
1-2 weeks.1-2 weeks. Demyelinating and inflammatory process leading to Demyelinating and inflammatory process leading to
an incomplete cord lesion initially produce a flaccid an incomplete cord lesion initially produce a flaccid areflexic paralysis known as spinal shock, and acute areflexic paralysis known as spinal shock, and acute UMN paralysis. UMN paralysis.
Marked disturbances in autonomic function can Marked disturbances in autonomic function can occur below the level of the lesion. occur below the level of the lesion.
All sensory modalities are lost below the level of the All sensory modalities are lost below the level of the lesion.lesion.
Radicular pain is common at the level of the lesion. Radicular pain is common at the level of the lesion.
Acute WeaknessAcute Weakness Spinal Cord Disease:Spinal Cord Disease:
Transverse MyelitisTransverse Myelitis AHC AHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Tick ParalysisTick Paralysis Rocky Mountain wood tick (Rocky Mountain wood tick (Dermacentor andersoniDermacentor andersoni) )
and the American dog tick (and the American dog tick (Dermacentor varaibilis).Dermacentor varaibilis). Symptoms within 2-7 days.Symptoms within 2-7 days. Bilateral Lower Extremity weakness that progresses Bilateral Lower Extremity weakness that progresses
to paralysis, ascends upward to trunk, arms, and to paralysis, ascends upward to trunk, arms, and head within hours and may lead to respiratory head within hours and may lead to respiratory failure and death.failure and death.
Minor sensory symptoms but constitutional signs Minor sensory symptoms but constitutional signs are usually absent.are usually absent.
DTR’s are usually hypoactive or absent and DTR’s are usually hypoactive or absent and opthalmoplegia and bulbar palsy can occur. opthalmoplegia and bulbar palsy can occur.
Human cases are rare and usually occur in children Human cases are rare and usually occur in children under the age of 10.under the age of 10.
Acute WeaknessAcute Weakness Spinal Cord Disease:Spinal Cord Disease:
Transverse MyelitisTransverse Myelitis AHC AHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Arsenic PoisoningArsenic Poisoning
Symptoms: violent GI symptoms, sense Symptoms: violent GI symptoms, sense of dryness and tightness in the throat, of dryness and tightness in the throat, thirst, hoarseness, and difficulty of thirst, hoarseness, and difficulty of speech.speech.
Emerald Green. Emerald Green. Arsenicosis - chronic arsenic poisoning Arsenicosis - chronic arsenic poisoning
from drinking water, New Hampshire.from drinking water, New Hampshire. Check hair follicles.Check hair follicles. Tx: Chelating agents.Tx: Chelating agents.
Acute WeaknessAcute Weakness Spinal Cord Disease:Spinal Cord Disease:
Transverse MyelitisTransverse Myelitis AHC AHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Acute Intermittent Acute Intermittent PorphyriaPorphyria
Rare metabolic disorder in the Rare metabolic disorder in the production of heme.production of heme.
Deficiency of the enzyme Deficiency of the enzyme porphobilinogen deamniase leads to the porphobilinogen deamniase leads to the metabolite porphyrin accumulating in metabolite porphyrin accumulating in the cytoplasm. the cytoplasm.
Symptoms of AIP may include abdominal Symptoms of AIP may include abdominal pain, constipation, and muscle weakness.pain, constipation, and muscle weakness.
Look for trigger. Look for trigger.
Acute WeaknessAcute Weakness Spinal Cord Disease:Spinal Cord Disease:
Transverse MyelitisTransverse Myelitis AHC AHC DiseaseDisease Epidural and Extradural Epidural and Extradural
Tumor or Spinal Cord Tumor or Spinal Cord tumortumor
Epidural Hematoma Epidural Hematoma Herniated intervertebral Herniated intervertebral
disk disk Peripheral Peripheral NerveNerve
Disease:Disease: Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome Acute intermittent Acute intermittent
porphyriaporphyria Arsenic poisoningArsenic poisoning Tick paralysisTick paralysis
NMJ disease:NMJ disease: Myasthenia gravisMyasthenia gravis BotulismBotulism Organophosphate Organophosphate
poisoningpoisoning Muscle disease:Muscle disease:
PolymyositisPolymyositis Rhabdomyolysis-Rhabdomyolysis-
myoglobinuriamyoglobinuria Acute alcoholic Acute alcoholic
myopathymyopathy Electrolyte imbalancesElectrolyte imbalances Endocrine diseaseEndocrine disease
Guillain-Barré SyndromeGuillain-Barré Syndrome 1859, Landry’s ascending paralysis.1859, Landry’s ascending paralysis. 1916, Guillain and Barré described the CSF 1916, Guillain and Barré described the CSF
findings.findings. Acquired demyelinating disorders of the Acquired demyelinating disorders of the
peripheral nervous systemperipheral nervous system with an acute onset.with an acute onset. Heterogeneous syndrome with several Heterogeneous syndrome with several
variants:variants: Acute inflammatory demyelinating Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP) polyradiculoneuropathy (AIDP) Miller-Fisher SyndromeMiller-Fisher Syndrome Acute axon loss ("axonal") polyradiculopathy Acute axon loss ("axonal") polyradiculopathy
Acute motor axonal neuropathy Acute motor axonal neuropathy Acute motor-sensory axonal neuropathyAcute motor-sensory axonal neuropathy
Other variantsOther variants
GBS: PathogenesisGBS: Pathogenesis Antecedent infection evokes an immune Antecedent infection evokes an immune
response, this in turn cross-reacts with response, this in turn cross-reacts with peripheral nerve components. peripheral nerve components. (Molecular Mimicry)(Molecular Mimicry)
Results in acute polyneuropathy as the Results in acute polyneuropathy as the response in directed toward myelin or response in directed toward myelin or the axon of PN.the axon of PN.
Rabbits sensitized with C. jejuni Rabbits sensitized with C. jejuni lipooligosaccharide or GM1, would lipooligosaccharide or GM1, would develop anti-GM1 IgG antibodies and develop anti-GM1 IgG antibodies and paralysis.paralysis.
GBS: Clinical FeaturesGBS: Clinical Features Usually begins distal legs, but 10% in arms Usually begins distal legs, but 10% in arms
or face. 50% will develop facial weakness or face. 50% will develop facial weakness and/or oropharygeal weakness. and/or oropharygeal weakness.
15% develop oculomotor weakness.15% develop oculomotor weakness. 80% develop parasthesias, usually mild.80% develop parasthesias, usually mild. Often severe low back pain.Often severe low back pain. 30% develop severe respiratory muscle 30% develop severe respiratory muscle
weakness requiring ventilatory support.weakness requiring ventilatory support. Dysautonomia in 70%: HR, BP, urinary Dysautonomia in 70%: HR, BP, urinary
retention, ileus, loss of sweating, and retention, ileus, loss of sweating, and occasionally Sudden Death.occasionally Sudden Death.
GBS: Antecedent EventsGBS: Antecedent Events
70% of cases or 2/3’s, 1-3 weeks prior.70% of cases or 2/3’s, 1-3 weeks prior. Campylobacter jejuni:Campylobacter jejuni: Most common, worse Most common, worse
prognosisprognosis HIV: any stage.HIV: any stage. Other infections: CMV, EBV, Hepatitis, Other infections: CMV, EBV, Hepatitis,
Mycoplasma pneumoniae, Mycoplasma pneumoniae, Influenza, Herpes. Influenza, Herpes. Vaccination:Vaccination: Influenza, Meningococcal (MCV4; Influenza, Meningococcal (MCV4;
report to VAERS).report to VAERS). Small percentage: Surgery, Trauma, BM Small percentage: Surgery, Trauma, BM
Transplant, TNF-alpha antagonist, and Transplant, TNF-alpha antagonist, and systemic illnesses.systemic illnesses.
AIDPAIDP
Most Common in US and Europe. Most Common in US and Europe. 85-90% of cases.85-90% of cases.
Peripheral nerve myelin is the target Peripheral nerve myelin is the target of immune attack.of immune attack.
Typical clinical features: Typical clinical features: progressive, fairly symmetric muscle progressive, fairly symmetric muscle weakness accompanied by absent or weakness accompanied by absent or depressed DTR. depressed DTR.
Miller-Fisher syndrome Miller-Fisher syndrome
5% of cases in US and 25% in Japan. 5% of cases in US and 25% in Japan. Ophthalmoplegia, ataxia, and Ophthalmoplegia, ataxia, and
areflexia. And ~1/3 will have some areflexia. And ~1/3 will have some extremity weakness.extremity weakness.
Associated with antibodies to Associated with antibodies to ganglioside GQ1b in 85-90%. ganglioside GQ1b in 85-90%.
AMAN/AMSANAMAN/AMSAN First recognized in 1986. First recognized in 1986. More frequent in China, Japan, and Mexico, More frequent in China, Japan, and Mexico,
but still 5-10% of GBS in US.but still 5-10% of GBS in US. More severe course than demyelinating GBS; More severe course than demyelinating GBS;
antibodies to GM1 in some cases.antibodies to GM1 in some cases. 60-70% preceded by 60-70% preceded by Campylobacter jejuniCampylobacter jejuni
infection. infection. Seasonal incidence, being more frequent in the Seasonal incidence, being more frequent in the
summer. summer. AMSAN: More severe form of AMAN, AMSAN: More severe form of AMAN,
pathology is predominantly axonal lesions of pathology is predominantly axonal lesions of both motor and sensory nerve fibers.both motor and sensory nerve fibers.
GBS: Lab featuresGBS: Lab features
Albuminocytologic Dissociation: normal Albuminocytologic Dissociation: normal WBC with an elevated CSF Protein level. WBC with an elevated CSF Protein level. 80-90% of patients with GBS at one 80-90% of patients with GBS at one week after sx onset.week after sx onset.
EMG and NCS: acute polyneuropathy EMG and NCS: acute polyneuropathy with predominate demylinating features with predominate demylinating features in AIDP, and axonal in AMAN and in AIDP, and axonal in AMAN and AMSAN.AMSAN.
Glycoprotein Antibodies: Anti GQ1b in Glycoprotein Antibodies: Anti GQ1b in 85-90% of MFS.85-90% of MFS.
GBS: Diagnostic CriteriaGBS: Diagnostic Criteria
RequiredRequired features: Progressive weakness and Areflexia. features: Progressive weakness and Areflexia. SupportiveSupportive features include: features include:
Progression of symptoms over days to four weeks Progression of symptoms over days to four weeks Relative symmetry Relative symmetry Mild sensory symptoms or signs Mild sensory symptoms or signs Cranial nerve involvement, especially bilateral facial nerve weakness Cranial nerve involvement, especially bilateral facial nerve weakness Recovery starting two to four weeks after progression halts Recovery starting two to four weeks after progression halts Autonomic dysfunction Autonomic dysfunction No fever at the onset No fever at the onset Elevated protein in CSF with a cell count <10 mm3 Elevated protein in CSF with a cell count <10 mm3 Electrodiagnostic abnormalities consistent with GBS Electrodiagnostic abnormalities consistent with GBS
GBS GBS doubtfuldoubtful:: Sensory levelSensory level Marked, persistent asymmetry of weakness Marked, persistent asymmetry of weakness Severe and persistent bowel and bladder dysfunction Severe and persistent bowel and bladder dysfunction More than 50 white cells in the CSF More than 50 white cells in the CSF
Criteria for diagnosis of Guillain-Barre syndrome. Ann Neurol 1978; 3:565.
GBS: TreatmentGBS: Treatment Supportive Care:Supportive Care:
Impending Respiratory Arrest: FVC <20 mL/kg, Impending Respiratory Arrest: FVC <20 mL/kg, Maximum inspiratory pressure <30 cmH2O, Maximum Maximum inspiratory pressure <30 cmH2O, Maximum expiratory pressure <40 cmH2O.expiratory pressure <40 cmH2O.
Prospective study of 722 GBS patients, 313 req Prospective study of 722 GBS patients, 313 req mechanical ventilation.mechanical ventilation.
Predictors of intubation: Predictors of intubation: Time of onset to admission less than seven days Time of onset to admission less than seven days Inability to cough Inability to cough Inability to stand Inability to stand Inability to lift the elbows Inability to lift the elbows Inability to lift the head Inability to lift the head Liver enzyme increases Liver enzyme increases
Sharshar T; et.al. Crit Care Med 2003 Jan;31(1):278-83.
GBS: TreatmentGBS: Treatment
Autonomic dysfunction: monitor BP, fluid Autonomic dysfunction: monitor BP, fluid status, and cardiac rhythm. Monitor B/B status, and cardiac rhythm. Monitor B/B function.function.
Pain Control: 40-50% pts have Pain Control: 40-50% pts have neuropathic pain.neuropathic pain.
Plasma Exchange: remove circulating Plasma Exchange: remove circulating antibodies, complement, and other antibodies, complement, and other agents.agents.
IVIG : unknown, possibly anti-idiotypic IVIG : unknown, possibly anti-idiotypic antibodies interfering with T and B cells.antibodies interfering with T and B cells.
GBS: TreatmentGBS: Treatment
AAN Observations: AAN Observations: Treatment with plasma exchange or Treatment with plasma exchange or
IVIG hastens recovery from GBS. IVIG hastens recovery from GBS. The beneficial effects of plasma The beneficial effects of plasma
exchange and IVIG are equivalent.exchange and IVIG are equivalent. Combining the two treatments is not Combining the two treatments is not
beneficial.beneficial. Steroid treatment alone is not Steroid treatment alone is not
beneficial.beneficial.
Neurology 2003 Sep 23;61(6):736-40. Neurology 2003 Sep 23;61(6):736-40.
““What was the cause of What was the cause of FDR’s paralytic illness?” FDR’s paralytic illness?”
A.S. Goldman, et. al. Journal of Medical Biography A.S. Goldman, et. al. Journal of Medical Biography 2003; 11:232-240.2003; 11:232-240. 1) Protracted symmetric ascending paralysis over 10-13 1) Protracted symmetric ascending paralysis over 10-13
days days 2) Facial paralysis2) Facial paralysis 3) Bladder and bowel dysfunction3) Bladder and bowel dysfunction 4) Numbness and Dysaesthesia4) Numbness and Dysaesthesia 5) Absence of meningismus5) Absence of meningismus 6) Descending pattern of recovery6) Descending pattern of recovery 7) Fever7) Fever 8) Permanent paralysis8) Permanent paralysis
Disease incidence in age group x symptom Disease incidence in age group x symptom probability. probability.
Six of eight favored GBS.Six of eight favored GBS.J Med Biogr. 11: 232–240 (2003)J Med Biogr. 11: 232–240 (2003)
FINAL ANSWERFINAL ANSWER Perform LP, Perform LP,
anti GQ1b, anti GQ1b, EMG and NCS.EMG and NCS.
GBS, possibly GBS, possibly AMAN variant.AMAN variant.
ReferencesReferences www.uptodate.comwww.uptodate.com HarrisonsHarrisons Goetz: Textbook of Clinical Neurology, 2nd ed. Copyright © Goetz: Textbook of Clinical Neurology, 2nd ed. Copyright ©
2003 Saunders, An Imprint of Elsevier2003 Saunders, An Imprint of Elsevier.. Criteria for diagnosis of Guillain-Barre syndrome. Ann Neurol Criteria for diagnosis of Guillain-Barre syndrome. Ann Neurol
1978; 3:565. 1978; 3:565. Early predictors of mechanical ventilation in Guillain-Barre Early predictors of mechanical ventilation in Guillain-Barre
syndrome. Sharshar T; Chevret S; Bourdain F; Raphael. Crit syndrome. Sharshar T; Chevret S; Bourdain F; Raphael. Crit Care Med 2003 Jan;31(1):278-83. Care Med 2003 Jan;31(1):278-83.
Practice parameter: immunotherapy for Guillain-Barre Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Hughes RA; Wijdicks the American Academy of Neurology. Hughes RA; Wijdicks EF; Barohn R; Benson E; Cornblath DR; Hahn AF; Meythaler EF; Barohn R; Benson E; Cornblath DR; Hahn AF; Meythaler JM; Miller RG; Sladky JT; Stevens JC. Neurology 2003 Sep JM; Miller RG; Sladky JT; Stevens JC. Neurology 2003 Sep 23;61(6):736-40. 23;61(6):736-40.
Goldman, AS Goldman, AS et alet al, , What was the cause of Franklin Delano What was the cause of Franklin Delano Roosevelt's paralytic illness?Roosevelt's paralytic illness?. J Med Biogr. 11: 232–240 . J Med Biogr. 11: 232–240 (2003) (2003)