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CPC: A Paralysis Quandary for a Dime Alan Trumbly, DO.

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Presentation on theme: "CPC: A Paralysis Quandary for a Dime Alan Trumbly, DO."— Presentation transcript:

1 CPC: A Paralysis Quandary for a Dime Alan Trumbly, DO

2

3 Objectives Case Presentation Case Presentation Problem List Problem List Locate the Lesion Locate the Lesion Develop Differential Develop Differential Relate Differential to patient Relate Differential to patient Cross fingers and choose… Cross fingers and choose…

4 Patient CC: Progressive Upper Extremity Weakness. CC: Progressive Upper Extremity Weakness. HPI: HPI: 70 y.o. Right handed, Hispanic Male. 70 y.o. Right handed, Hispanic Male. 2 weeks ago developed fever, chills, nausea, vomiting, abdominal cramping, diarrhea. Symptoms resolved in 24-36 hours. 2 weeks ago developed fever, chills, nausea, vomiting, abdominal cramping, diarrhea. Symptoms resolved in 24-36 hours. 2 Days PTA: Left upper extremity numbness, tingling, and weakness. 2 Days PTA: Left upper extremity numbness, tingling, and weakness. 1Day PTA: Continued to progressively worsening, and by the PM both upper extremities involved. 1Day PTA: Continued to progressively worsening, and by the PM both upper extremities involved. Transferred to S/W on day of admission for further neurological workup. Transferred to S/W on day of admission for further neurological workup.

5 Patient HPI: On day of admission HPI: On day of admission Both arms quite weak. Both arms quite weak. Dull aching neck pain, especially with rotation to the right. Dull aching neck pain, especially with rotation to the right. NO diploplia, ptosis, dysarthria, dysphasia, shortness of breath, lower extremity weakness, or bowel or bladder incontinence. NO diploplia, ptosis, dysarthria, dysphasia, shortness of breath, lower extremity weakness, or bowel or bladder incontinence. NO pain in limbs or back. NO pain in limbs or back. NO prior neurological history, except lumbar laminectomy for Left L4-L5 radiculopathy. NO prior neurological history, except lumbar laminectomy for Left L4-L5 radiculopathy.

6 Patient PMH: PMH: Hypertension Hypertension Hyperlipidemia Hyperlipidemia Diabetes: good blood sugar control, no neuropathy. Diabetes: good blood sugar control, no 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: none Allergies: none Medications: none Medications: none

7 Patient Vitals: Afebrile Vitals: Afebrile PExam: PExam: Gen: pleasant, straight- forward, Hispanic man, upset, but no distress, alert and oriented with no dysarthria. Gen: pleasant, straight- forward, Hispanic man, upset, but no distress, alert and oriented with no dysarthria. CN: visual fields full, pupils react 3 to 2 mm, no papilledema, ocular motility full, no nystagmus. Mild left facial weakness. Masseter and temporalis strength and bulk, as well as pterygoid strength all normal. CN: visual fields full, pupils react 3 to 2 mm, no papilledema, ocular motility full, no nystagmus. Mild left facial weakness. Masseter and temporalis strength and bulk, as well as pterygoid strength all normal.

8 Patient 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 atrophy Motor: no fasciculations or atrophy Upper ext: Deltoids 2/5 bilaterally, Triceps 4-/5 right, and 3/5 on the left. Finger and wrist extensors 0/5 Upper ext: Deltoids 2/5 bilaterally, Triceps 4-/5 right, and 3/5 on the left. Finger and wrist extensors 0/5 Reflexes: Absent in the upper extremities,, trace at the right knee, absent at the ankles. Plantar responses were neutral bilaterally. Reflexes: Absent in the upper extremities,, trace at the right knee, absent at the ankles. Plantar responses were neutral bilaterally.

9 Patient Motor: Motor: Reflexes: Reflexes: 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 5/5 Right 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

10 Patient 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: 0.8 Alk Ph: 90 AST: 23 ALT: 32 Tot Prot: 7.0 Alb: 4.1 13.7 5.8208 38.9 MCV: 84.4

11 Our Mission Additional studies were done upon arrival here and a diagnosis was made??? Additional studies were done upon arrival here and a diagnosis was made???

12 Problem List Subacute (days) Progressive Upper Extremity Weakness Subacute (days) Progressive Upper Extremity Weakness Absent Muscle Stretch Reflexes Absent Muscle Stretch Reflexes Rightward deviation of the tongue, and Left SCM and Trapezius were weak Rightward deviation of the tongue, and Left SCM and Trapezius were weak Recent Diarrhea Illness (2 weeks prior) Recent Diarrhea Illness (2 weeks prior) Diabetes Diabetes Hypertension Hypertension Hx of Lumbar laminectomy Hx of Lumbar laminectomy

13 FDR 32 nd President 32 nd President Presidency spanned the Great Depression of 1930’s, and most of World War II. Presidency spanned the Great Depression of 1930’s, and most of World War II. Only U.S. president to have served more than two terms. Only U.S. president to have served more than two terms.

14 Location, Location, Location What are symptoms to look for? What are symptoms to look for? What are the locations possible? What are the locations possible?

15 Signs that Distinguish Patterns of Weakness SignUMNLMNMyopathic AtrophyNoneSevereMild FasciculationsNoneCommonNone ToneSpasticDecreasedNorm/Dec DistributionPyramidal/regionalDistal/segmentalProximal DTRHyperactiveHypoactive/absentNormal/hypoactive BabinskiPresentAbsentAbsent

16 Location, Location, Location Hemisphere Hemisphere Brainstem Brainstem Cerebellum Cerebellum Cord Cord AHC AHC Root Root Plexus Plexus Nerve Nerve NMJ NMJ Muscle Muscle

17 Location, Location, Location Hemisphere Hemisphere Brainstem Brainstem Cerebellum Cerebellum Cord Cord AHC AHC Root Root Plexus Plexus Nerve Nerve NMJ NMJ Muscle Muscle

18 Location, Location, Location Hemisphere Hemisphere Brainstem Brainstem Cerebellum Cerebellum Cord Cord AHC AHC Root Root Plexus Plexus Nerve Nerve NMJ NMJ Muscle Muscle

19 Location, Location, Location Hemisphere Hemisphere Brainstem Brainstem Cerebellum Cerebellum Cord Cord AHC AHC Root Root Plexus Plexus Nerve Nerve NMJ NMJ Muscle Muscle

20 FDR Founded the National Foundation for Infantile Paralysis in the US in 1938, which funded rehab programs for victims of paralytic polio and the development of the vaccine. Founded the National Foundation for Infantile Paralysis in the US in 1938, which funded rehab programs for victims of paralytic polio and the development of the vaccine. Now known as the March of Dimes. Now known as the March of Dimes.

21 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

22 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

23 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

24 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

25 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

26 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

27 Myesthenia Gravis Young women and older men Young women and older men Diplopia, Dysarthria, Dysphagia, Dyspnea Diplopia, Dysarthria, Dysphagia, Dyspnea Deficits are fatigable. Deficits are fatigable. Pure muscular weakness without the atrophy, and normal DTR, Sensation, Mentation, and Sphincter tone. Pure muscular weakness without the atrophy, and normal DTR, Sensation, Mentation, and Sphincter tone. Lambert-Eaton Syndrome: Paraneoplastic, DTR absent but improve with exercise, incontinence present, and antibody mediated. Lambert-Eaton Syndrome: Paraneoplastic, DTR absent but improve with exercise, incontinence present, and antibody mediated.

28 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

29 Botulism Abdominal and GI symptoms preceding syndrome that resembles myasthenia gravis. Abdominal and GI symptoms preceding syndrome that resembles myasthenia gravis. Improperly canned foods contaminated with the exotoxin of Clostridium botulinum. Improperly canned foods contaminated with the exotoxin of Clostridium botulinum. Rapidly developing paralysis usually affects the ocular and cranial musculature first then generalized (Descending). Rapidly developing paralysis usually affects the ocular and cranial musculature first then generalized (Descending). Toxin-mediated inhibition of acetylcholine release from axon terminals at NMJ. Toxin-mediated inhibition of acetylcholine release from axon terminals at NMJ.

30 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

31 Organophosphate Poisoning Miosis, Excessive bodily secretions, and fasciculations. Miosis, Excessive bodily secretions, and fasciculations. Decreased acetylcholinesterase activity that causes excessive acetylcholine at the NMJ. Decreased acetylcholinesterase activity that causes excessive acetylcholine at the NMJ. Symptoms vary 5 to 12 hours after exposure. Symptoms vary 5 to 12 hours after exposure. Tx with atropine. Tx with atropine.

32 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

33 FDR August, 10 1921, FDR was 39 y.o. fell into Bay of Fundy, near Campobello, New Brunswick. August, 10 1921, FDR was 39 y.o. fell into Bay of Fundy, near Campobello, New Brunswick. 1933, elected President of the US with symmetrical lower extremity weakness. 1933, elected President of the US with symmetrical lower extremity weakness.

34 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

35 Spinal Cord Disease Signs and symptoms occur at affected area and below. Signs and symptoms occur at affected area and below. Associated with UMN lesions. Associated with UMN lesions. Caused by compressive or noncompressive lesion. Caused by compressive or noncompressive lesion. Central Cord Syndrome: Central Cord Syndrome: Most often caused by syringomyelia and intramedullary cord tumors. Most often caused by syringomyelia and intramedullary cord tumors. Pathological process starts centrally and proceeds centrifugally, producing motor and sensory signs. Pathological process starts centrally and proceeds centrifugally, producing motor and sensory signs. Suspended sensory loss: decussating spinothalamic tract fibers are affected, loss of pain and temperature is bilateral, “cape distribution”>>> “sacral sparring”. Suspended sensory loss: decussating spinothalamic tract fibers are affected, loss of pain and temperature is bilateral, “cape distribution”>>> “sacral sparring”.

36 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

37 Anterior Horn Cell Disease Cell bodies of peripheral motor nerves. Cell bodies of peripheral motor nerves. Amyotrophic Lateral Sclerosis: Amyotrophic Lateral Sclerosis: Most common, affects AHC and corticospinal tracts. Most common, affects AHC and corticospinal tracts. Distal bilateral weakness, with atrophy and fasciculation's (LMN signs), combined with bulbar signs, hyper-reflexia, upgoing toes (UMN signs). Distal bilateral weakness, with atrophy and fasciculation's (LMN signs), combined with bulbar signs, hyper-reflexia, upgoing toes (UMN signs). Asymmetric limb weakness. Asymmetric limb weakness.

38 Post-Polio Syndrome Major cause of morbidity and death throughout the world during the first half of the 20th century. Major cause of morbidity and death throughout the world during the first half of the 20th century. Young children characterized by a mild, brief febrile illness. Young children characterized by a mild, brief febrile illness. Small group would develop fever, HA, meningeal irritation, soreness, and asymmetric paralysis. Small group would develop fever, HA, meningeal irritation, soreness, and asymmetric paralysis. Introduction of the inactivated polio vaccine in 1954. Introduction of the inactivated polio vaccine in 1954.

39 West Nile Virus RNA flavivirus. RNA flavivirus. Majority asymptomatic, 20% develop febrile disease, and only 1% will develop neuroinvasive disease (aseptic meningitis, encephalitis, or flaccid paralysis). Majority asymptomatic, 20% develop febrile disease, and only 1% will develop neuroinvasive disease (aseptic meningitis, encephalitis, or flaccid paralysis). Abrupt onset of fever, headache, myalgia, weakness, and often, abdominal pain, nausea, vomiting, or diarrhea. Abrupt onset of fever, headache, myalgia, weakness, and often, abdominal pain, nausea, vomiting, or diarrhea. Flaccid paralysis caused by WNV infection is similar clinically and pathologically to poliomyelitis caused by poliovirus, with damage of anterior horn cells. Flaccid paralysis caused by WNV infection is similar clinically and pathologically to poliomyelitis caused by poliovirus, with damage of anterior horn cells.

40 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

41 Transverse Myelitis Inflammatory diseases of the Spinal Cord: viral, bacterial, fungal, parasitic, noninfectious. Inflammatory diseases of the Spinal Cord: viral, bacterial, fungal, parasitic, noninfectious. Antecedent event that precede symptoms by days to 1-2 weeks. Antecedent event that precede symptoms by days to 1-2 weeks. Demyelinating and inflammatory process leading to an incomplete cord lesion initially produce a flaccid areflexic paralysis known as spinal shock, and acute UMN paralysis. Demyelinating and inflammatory process leading to an incomplete cord lesion initially produce a flaccid areflexic paralysis known as spinal shock, and acute UMN paralysis. Marked disturbances in autonomic function can occur below the level of the lesion. Marked disturbances in autonomic function can occur below the level of the lesion. All sensory modalities are lost below the level of the lesion. All sensory modalities are lost below the level of the lesion. Radicular pain is common at the level of the lesion. Radicular pain is common at the level of the lesion.

42 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

43 Tick Paralysis Rocky Mountain wood tick (Dermacentor andersoni) and the American dog tick (Dermacentor varaibilis). Rocky Mountain wood tick (Dermacentor andersoni) and the American dog tick (Dermacentor varaibilis). Symptoms within 2-7 days. Symptoms within 2-7 days. Bilateral Lower Extremity weakness that progresses to paralysis, ascends upward to trunk, arms, and head within hours and may lead to respiratory failure and death. Bilateral Lower Extremity weakness that progresses to paralysis, ascends upward to trunk, arms, and head within hours and may lead to respiratory failure and death. Minor sensory symptoms but constitutional signs are usually absent. Minor sensory symptoms but constitutional signs are usually absent. DTR’s are usually hypoactive or absent and opthalmoplegia and bulbar palsy can occur. DTR’s are usually hypoactive or absent and opthalmoplegia and bulbar palsy can occur. Human cases are rare and usually occur in children under the age of 10. Human cases are rare and usually occur in children under the age of 10.

44 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

45 Arsenic Poisoning Symptoms: violent GI symptoms, sense of dryness and tightness in the throat, thirst, hoarseness, and difficulty of speech. Symptoms: violent GI symptoms, sense of dryness and tightness in the throat, thirst, hoarseness, and difficulty of speech. Emerald Green. Emerald Green. Arsenicosis - chronic arsenic poisoning from drinking water, New Hampshire. Arsenicosis - chronic arsenic poisoning from drinking water, New Hampshire. Check hair follicles. Check hair follicles. Tx: Chelating agents. Tx: Chelating agents.

46 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

47 Acute Intermittent Porphyria Rare metabolic disorder in the production of heme. Rare metabolic disorder in the production of heme. Deficiency of the enzyme porphobilinogen deamniase leads to the metabolite porphyrin accumulating in the cytoplasm. Deficiency of the enzyme porphobilinogen deamniase leads to the metabolite porphyrin accumulating in the cytoplasm. Symptoms of AIP may include abdominal pain, constipation, and muscle weakness. Symptoms of AIP may include abdominal pain, constipation, and muscle weakness. Look for trigger. Look for trigger.

48 Acute Weakness Spinal Cord Disease: Spinal Cord Disease: Transverse Myelitis Transverse Myelitis AHC Disease AHC Disease Epidural and Extradural Tumor or Spinal Cord tumor Epidural and Extradural Tumor or Spinal Cord tumor Epidural Hematoma Epidural Hematoma Herniated intervertebral disk Herniated intervertebral disk Peripheral Nerve Disease: Peripheral Nerve Disease: Guillain-Barre’ Syndrome Guillain-Barre’ Syndrome Acute intermittent porphyria Acute intermittent porphyria Arsenic poisoning Arsenic poisoning Tick paralysis Tick paralysis NMJ disease: Myasthenia gravis Botulism Organophosphate poisoning Muscle disease: Polymyositis Rhabdomyolysis- myoglobinuria Acute alcoholic myopathy Electrolyte imbalances Endocrine disease

49 Guillain-Barré Syndrome 1859, Landry’s ascending paralysis. 1859, Landry’s ascending paralysis. 1916, Guillain and Barré described the CSF findings. 1916, Guillain and Barré described the CSF findings. Acquired demyelinating disorders of the peripheral nervous system with an acute onset. Acquired demyelinating disorders of the peripheral nervous system with an acute onset. Heterogeneous syndrome with several variants: Heterogeneous syndrome with several variants: Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) Miller-Fisher Syndrome Miller-Fisher Syndrome Acute axon loss ("axonal") polyradiculopathy Acute axon loss ("axonal") polyradiculopathy Acute motor axonal neuropathy Acute motor axonal neuropathy Acute motor-sensory axonal neuropathy Acute motor-sensory axonal neuropathy Other variants Other variants

50 GBS: Pathogenesis Antecedent infection evokes an immune response, this in turn cross-reacts with peripheral nerve components. (Molecular Mimicry) Antecedent infection evokes an immune response, this in turn cross-reacts with peripheral nerve components. (Molecular Mimicry) Results in acute polyneuropathy as the response in directed toward myelin or the axon of PN. Results in acute polyneuropathy as the response in directed toward myelin or the axon of PN. Rabbits sensitized with C. jejuni lipooligosaccharide or GM1, would develop anti-GM1 IgG antibodies and paralysis. Rabbits sensitized with C. jejuni lipooligosaccharide or GM1, would develop anti-GM1 IgG antibodies and paralysis.

51 GBS: Clinical Features Usually begins distal legs, but 10% in arms or face. 50% will develop facial weakness and/or oropharygeal weakness. Usually begins distal legs, but 10% in arms or face. 50% will develop facial 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 weakness requiring ventilatory support. 30% develop severe respiratory muscle weakness requiring ventilatory support. Dysautonomia in 70%: HR, BP, urinary retention, ileus, loss of sweating, and occasionally Sudden Death. Dysautonomia in 70%: HR, BP, urinary retention, ileus, loss of sweating, and occasionally Sudden Death.

52 GBS: 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: Most common, worse prognosis Campylobacter jejuni: Most common, worse prognosis HIV: any stage. HIV: any stage. Other infections: CMV, EBV, Hepatitis, Mycoplasma pneumoniae, Influenza, Herpes. Other infections: CMV, EBV, Hepatitis, Mycoplasma pneumoniae, Influenza, Herpes. Vaccination: Influenza, Meningococcal (MCV4; report to VAERS). Vaccination: Influenza, Meningococcal (MCV4; report to VAERS). Small percentage: Surgery, Trauma, BM Transplant, TNF-alpha antagonist, and systemic illnesses. Small percentage: Surgery, Trauma, BM Transplant, TNF-alpha antagonist, and systemic illnesses.

53 AIDP Most Common in US and Europe. 85-90% of cases. Most Common in US and Europe. 85-90% of cases. Peripheral nerve myelin is the target of immune attack. Peripheral nerve myelin is the target of immune attack. Typical clinical features: progressive, fairly symmetric muscle weakness accompanied by absent or depressed DTR. Typical clinical features: progressive, fairly symmetric muscle weakness accompanied by absent or depressed DTR.

54 Miller-Fisher syndrome 5% of cases in US and 25% in Japan. 5% of cases in US and 25% in Japan. Ophthalmoplegia, ataxia, and areflexia. And ~1/3 will have some extremity weakness. Ophthalmoplegia, ataxia, and areflexia. And ~1/3 will have some extremity weakness. Associated with antibodies to ganglioside GQ1b in 85-90%. Associated with antibodies to ganglioside GQ1b in 85-90%.

55 AMAN/AMSAN First recognized in 1986. First recognized in 1986. More frequent in China, Japan, and Mexico, but still 5- 10% of GBS in US. More frequent in China, Japan, and Mexico, but still 5- 10% of GBS in US. More severe course than demyelinating GBS; antibodies to GM1 in some cases. More severe course than demyelinating GBS; antibodies to GM1 in some cases. 60-70% preceded by Campylobacter jejuni infection. 60-70% preceded by Campylobacter jejuni infection. Seasonal incidence, being more frequent in the summer. Seasonal incidence, being more frequent in the summer. AMSAN: More severe form of AMAN, pathology is predominantly axonal lesions of both motor and sensory nerve fibers. AMSAN: More severe form of AMAN, pathology is predominantly axonal lesions of both motor and sensory nerve fibers.

56 GBS: Lab features Albuminocytologic Dissociation: normal WBC with an elevated CSF Protein level. 80-90% of patients with GBS at one week after sx onset. Albuminocytologic Dissociation: normal WBC with an elevated CSF Protein level. 80-90% of patients with GBS at one week after sx onset. EMG and NCS: acute polyneuropathy with predominate demylinating features in AIDP, and axonal in AMAN and AMSAN. EMG and NCS: acute polyneuropathy with predominate demylinating features in AIDP, and axonal in AMAN and AMSAN. Glycoprotein Antibodies: Anti GQ1b in 85- 90% of MFS. Glycoprotein Antibodies: Anti GQ1b in 85- 90% of MFS.

57 GBS: Diagnostic Criteria Required features: Progressive weakness and Areflexia. Required features: Progressive weakness and Areflexia. Supportive features include: Supportive 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 doubtful: GBS doubtful: Sensory level Sensory 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.

58 GBS: Treatment Supportive Care: Supportive Care: Impending Respiratory Arrest: FVC <20 mL/kg, Maximum inspiratory pressure <30 cmH2O, Maximum expiratory pressure <40 cmH2O. Impending Respiratory Arrest: FVC <20 mL/kg, Maximum inspiratory pressure <30 cmH2O, Maximum expiratory pressure <40 cmH2O. Prospective study of 722 GBS patients, 313 req mechanical ventilation. Prospective study of 722 GBS patients, 313 req 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.

59 GBS: Treatment Autonomic dysfunction: monitor BP, fluid status, and cardiac rhythm. Monitor B/B function. Autonomic dysfunction: monitor BP, fluid status, and cardiac rhythm. Monitor B/B function. Pain Control: 40-50% pts have neuropathic pain. Pain Control: 40-50% pts have neuropathic pain. Plasma Exchange: remove circulating antibodies, complement, and other agents. Plasma Exchange: remove circulating antibodies, complement, and other agents. IVIG : unknown, possibly anti-idiotypic antibodies interfering with T and B cells. IVIG : unknown, possibly anti-idiotypic antibodies interfering with T and B cells.

60 GBS: Treatment AAN Observations: AAN Observations: Treatment with plasma exchange or IVIG hastens recovery from GBS. Treatment with plasma exchange or IVIG hastens recovery from GBS. The beneficial effects of plasma exchange and IVIG are equivalent. The beneficial effects of plasma exchange and IVIG are equivalent. Combining the two treatments is not beneficial. Combining the two treatments is not beneficial. Steroid treatment alone is not beneficial. Steroid treatment alone is not beneficial. Neurology 2003 Sep 23;61(6):736-40.

61 “What was the cause of FDR’s paralytic illness?” A.S. Goldman, et. al. Journal of Medical Biography 2003; 11:232-240. A.S. Goldman, et. al. Journal of Medical Biography 2003; 11:232-240. 1) Protracted symmetric ascending paralysis over 10-13 days 1) Protracted symmetric ascending paralysis over 10-13 days 2) Facial paralysis 2) Facial paralysis 3) Bladder and bowel dysfunction 3) Bladder and bowel dysfunction 4) Numbness and Dysaesthesia 4) Numbness and Dysaesthesia 5) Absence of meningismus 5) Absence of meningismus 6) Descending pattern of recovery 6) Descending pattern of recovery 7) Fever 7) Fever 8) Permanent paralysis 8) Permanent paralysis Disease incidence in age group x symptom probability. Disease incidence in age group x symptom probability. Six of eight favored GBS. Six of eight favored GBS. J Med Biogr. 11: 232–240 (2003)

62 FINAL ANSWER Perform LP, anti GQ1b, EMG and NCS. Perform LP, anti GQ1b, EMG and NCS. GBS, possibly AMAN variant. GBS, possibly AMAN variant.

63 References www.uptodate.com www.uptodate.com www.uptodate.com Harrisons Harrisons Goetz: Textbook of Clinical Neurology, 2nd ed. Copyright © 2003 Saunders, An Imprint of Elsevier. Goetz: Textbook of Clinical Neurology, 2nd ed. Copyright © 2003 Saunders, An Imprint of Elsevier.2003 Saunders, An Imprint of Elsevier2003 Saunders, An Imprint of Elsevier Criteria for diagnosis of Guillain-Barre syndrome. Ann Neurol 1978; 3:565. Criteria for diagnosis of Guillain-Barre syndrome. Ann Neurol 1978; 3:565. Early predictors of mechanical ventilation in Guillain-Barre syndrome. Sharshar T; Chevret S; Bourdain F; Raphael. Crit Care Med 2003 Jan;31(1):278-83. Early predictors of mechanical ventilation in Guillain-Barre syndrome. Sharshar T; Chevret S; Bourdain F; Raphael. Crit Care Med 2003 Jan;31(1):278-83. Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Hughes RA; Wijdicks EF; Barohn R; Benson E; Cornblath DR; Hahn AF; Meythaler JM; Miller RG; Sladky JT; Stevens JC. Neurology 2003 Sep 23;61(6):736-40. Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Hughes RA; Wijdicks EF; Barohn R; Benson E; Cornblath DR; Hahn AF; Meythaler JM; Miller RG; Sladky JT; Stevens JC. Neurology 2003 Sep 23;61(6):736-40. Goldman, AS et al, What was the cause of Franklin Delano Roosevelt's paralytic illness?. J Med Biogr. 11: 232–240 (2003) Goldman, AS et al, What was the cause of Franklin Delano Roosevelt's paralytic illness?. J Med Biogr. 11: 232–240 (2003)What was the cause of Franklin Delano Roosevelt's paralytic illness?What was the cause of Franklin Delano Roosevelt's paralytic illness?


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