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Dr. Trumbly, September 2007

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  • Only Cerebellum does not give weakness. Usually sensory loss except again Cerebellum, plus AHC, NMJ, Muscle. Hemisphere unlikely without cognitive changes, seizures, language changes (aphasia), Dysarthria, Visual field loss, and UMN signs with upgoing toe, and hyperreflexia.
  • 3. Brainstem typically see Dysarthria, Dysconjugate gaze, B/B incontinance, and UMN signs with upgoing toe, and hyperreflexia.
  • 4. Again typically see Dysarthria, gait abnormalities, not likely
  • 5. Root typically see atrophy and fasciculations, but maybe not in subacute presentation, and you can see that from AHC to Nerve, You also typically see severe pain, “radiculopathy”, usually herniated disc, trauma/event, can be associated with weakness and sensory change, and these symptoms are in the distribution of the particular root. Other causes would be shingles, or pseudoclaudication. 6. Plexus is LMN, Not Plexus d/t affected areas, can be cervical, brachial, lumbosacral. Several nerve roots involved. Usually caused by trauma, local invasion by neoplasm, idiopathic, or diabetic amyotrophy.
  • Bilateral proximal muscle weakness Unable to rise from a seated position without help Dx: CK, signs and symptoms, EMG alteration, and a positive muscle biopsy. Sporadic Inclusion Body Myositis : months to years.
  • Bilateral proximal muscle weakness Unable to rise from a seated position without help Dx: CK, signs and symptoms, EMG alteration, and a positive muscle biopsy. Sporadic Inclusion Body Myositis : months to years.
  • small cell lung cancer warming-up phenomenon: muscle is used repetitively, it may gain in strength, correlates with an incremental increase in the amplitude of the muscle action potential with repetitive stimulation. The initial low amplitude of the muscle action potential occurs because an antibody blocks a calcium channel , slowing the entry of calcium into the presynaptic terminal to initiate vesicular release of acetylcholine.[ 44 ] Because it is only a partial blockade, however, enough calcium eventually enters with repetitive depolarizations to allow the amplitude of response to increase and strength to improve, at least temporarily.[ 45 ] In many but not all cases, the antibody against the calcium channel is generated by a cancer; thus, the Eaton-Lambert myasthenic syndrome is often a paraneoplastic disorder.[ 46 ]
  • (Usually more than one person) Prolonged, lasting months, because the toxin permanently impairs the presynaptic release of acetylcholine. Botulism produces a. Because it, and the weakness develops so rapidly, botulism can be confused with a brain stem stroke or encephalitis . It rapidly becomes generalized to include all skeletal musculature and then may be mistaken for AIDP ( Table 15-14 ). In typical cases, these conditions can be distinguished from each other by the fact that botulism starts in the ocular and cranial musculature and produces a descending paralysis , whereas AIDP starts as a weakness in the legs and produces an ascending paralysis that eventually includes the cranial musculature. This rule applies very well to botulism, which almost invariably begins in the ocular and cranial musculature, but may be violated by AIDP, which sometimes begins in the cranial musculature and descends. However, the early loss of pupillary reflexes in botulism and the predilection of AIDP for the facial and lower cranial nerves can clearly distinguish the two disorders. Weakness resulting from botulism is prolonged, lasting months, because the toxin permanently impairs the presynaptic release of acetylcholine from the terminals to which the toxin is bound. Neuromuscular transmission and strength return only after the nerve terminals sprout new endings.
  • Developed “Lumbago” ~1 days later, and developed weakness in one leg. 2 days later, bilateral paralysis, and could not stand, developed a fever of 102. 3 days later, paralysed from chest down. Involving arms, shoulders, and hands, as well as constipation and requiring catheterizations. The fever continued for 7 days. 15 th day, facial involvement, Temp 100, paralysed from waist down. Mid September, mild improvement in upper extremities, concerned about atrophy. End of October, D/Ced with improvement in back and abdomen.
  • both motor and sensory, upgoing toes, hyper-reflexia, bowel and bladder incontinence.
  • SPECTRUM OF MOTOR NEURON DISEASE — Amyotrophic lateral sclerosis (ALS) is one of multiple degenerative motor neuron diseases (MND) that are clinically defined, based on the involvement of upper and/or lower motor neurons [ 1,2,8 ]. ALS is the most common form and includes upper motor neuron (UMN) and lower motor neuron (LMN) pathology. no ethnic or racial predisposition to ALS. Prior to the age of 65 or 70, the incidence of ALS is higher in men than in women, but thereafter the gender incidence is equal. ALS has an age distribution that peaks in the seventh to eight decade. is the most common presentation of ALS (80 percent). Upper extremity onset is most often heralded by hand weakness but may begin in the shoulder girdle muscles. Symptoms initially spread within the segment of onset and then to other regions in a relatively predictable pattern [ 1,13 ]. In patients with unilateral arm onset the most common (about 60 to 70 percent of patients) pattern of spread is to the contralateral arm, then to the ipsilateral leg, then to the contralateral remaining leg, and then to the bulbar muscles. clinical hallmark of amyotrophic lateral sclerosis (ALS) is the combination of upper motor neuron (UMN) and lower motor neuron (LMN) signs and symptoms. UMN findings of weakness, hyperreflexia, and spasticity result from degeneration of frontal motor neurons. The LMN findings of weakness, atrophy or amyotrophy, and fasciculations are a direct consequence of degeneration of LMNs in the brainstem and spinal cord
  • In 1952, shortly before the introduction of the polio vaccine, more than 20,000 cases were reported in the US [ 1 ]. resulted in a dramatic decline of new polio cases; with widespread vaccination fewer than 10 new cases of paralytic polio are reported per year in the United States [ 2,3 ].
  • Medications, Maladies, Malnutrition, Menstration Peripheral neuropathy is a common feature of AIP. Muscle weakness often begins proximally in the legs but may involve the arms or the distal extremities. Motor neuropathy may also involve the cranial nerves, or lead to bulbar paralysis, respiratory impairment, and death. Sensory, patchy neuropathy may also occur.
  • Maximum weakness is usually reached within 2 weeks; demyelination by EMG. Most pts are hospitalized; one-third require ventilatory assistance. 85% make a complete or near-complete recovery with supportive care. Intravenous immune globulin (IVIg) (2 g/kg given over 5 days) or plasmapheresis (40–50 mL/kg daily for 4–5 days) significantly shortens the course. Glucocorticoids are ineffective. AIDP is the most common subtype in developed countries, while axonal forms are more common in northern China.
  • , which in turn cross-reacts with peripheral nerve components because of the sharing of cross-reactive epitopes (molecular mimicry) [ 2 ]. The end result is an acute polyneuropathy. This immune response can be directed towards the myelin or the axon of peripheral nerve.
  • GBS variant with ophthalmoplegia, ataxia, areflexia, and, often, facial weakness but at most minor limb weakness. The onset is subacute and recovery is usually good. Antibodies to the ganglioside GQ1B are found in most cases. These antibodies are capable of blocking neuromuscular transmission in the mouse nerve–diaphragm preparation, and may be active at ocular motor nerve terminals in humans. Mechanisms of ataxia and areflexia are not clear. No good data exist on the usefulness of specific treatments, but the relatively benign prognosis must be kept in mind.
  • of more than one limb, ranging from minimal weakness of the legs to total paralysis of all four limbs, the trunk, bulbar and facial muscles, and external ophthalmoplegia While universal areflexia is typical, distal areflexia with hyporeflexia at the knees and biceps will suffice if other features are consistent. (decrement or loss of sensation below a spinal cord root level as determined by neurologic examination)
  • Hypotension can usually be treated with fluids, but low-dose phenylephrine can be used if fluids are not effective. Episodes of severe hypertension (MAP>125) can be treated with labetalol , esmolol , or nitroprusside [ 5,6 ] For treating ileus, erythromycin or neostigmine may be effective [ 1 ]. Promotility agents are contraindicated in patients with dysautonomia.
  • AAN recommends plasma exchange or IVIG for GBS treatment as follows [ 19 ]: Plasma exchange is recommended for nonambulatory adult patients with GBS who start treatment within four weeks of the onset of neuropathic symptoms. Plasma exchange is also recommended for ambulatory patients who start treatment within two weeks of the onset of neuropathic symptoms IVIG is recommended for nonambulatory adult patients with GBS who start treatment within two or possibly four weeks of the onset of neuropathic symptoms
  • Transcript

    • 1. CPC: A Paralysis Quandary for a Dime Alan Trumbly, DO
    • 2.  
    • 3. Objectives
      • Case Presentation
      • Problem List
      • Locate the Lesion
      • Develop Differential
      • Relate Differential to patient
      • Cross fingers and choose…
    • 4. Patient
      • CC: Progressive Upper Extremity Weakness.
      • HPI:
        • 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 Days PTA: Left upper extremity numbness, tingling, and weakness.
        • 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.
    • 5. Patient
      • HPI: On day of admission
        • Both arms quite weak.
        • 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 pain in limbs or back.
        • NO prior neurological history, except lumbar laminectomy for Left L4-L5 radiculopathy.
    • 6. Patient
      • PMH:
        • Hypertension
        • Hyperlipidemia
        • Diabetes: good blood sugar control, no neuropathy.
      • Fam Hx: negative for neurological disease.
      • Soc Hx: No tobacco or alcohol.
      • Allergies: none
      • Medications: none
    • 7. Patient
      • Vitals: Afebrile
      • PExam:
        • 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.
    • 8. Patient
      • PExam:
        • Palate elevated briskly midline.
        • Right deviation of the tongue with protrusion.
        • Left SCM and Trapezius were weak.
        • Right SCM and Trapezius were normal.
        • 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
        • Reflexes: Absent in the upper extremities, , trace at the right knee, absent at the ankles. Plantar responses were neutral bilaterally.
    • 9. Patient
      • Motor :
      • 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:
        • All sensory modalities reported normal.
        • Plantar responses neutral bilaterally.
        • Gait: not testable.
        • Cerebellar: not testable.
      • 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 5.8 208 38.9 MCV: 84.4
    • 11. Our Mission
      • Additional studies were done upon arrival here and a diagnosis was made???
    • 12. Problem List
      • Subacute (days) Progressive Upper Extremity Weakness
      • Absent Muscle Stretch Reflexes
      • Rightward deviation of the tongue, and Left SCM and Trapezius were weak
      • Recent Diarrhea Illness (2 weeks prior)
      • Diabetes
      • Hypertension
      • Hx of Lumbar laminectomy
    • 13. FDR
      • 32 nd President
      • 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.
    • 14. Location, Location, Location
      • What are symptoms to look for?
      • What are the locations possible?
    • 15. Signs that Distinguish Patterns of Weakness Absent Absent Present Babinski Normal/hypoactive Hypoactive/absent Hyperactive DTR Proximal Distal/segmental Pyramidal/regional Distribution Norm/Dec Decreased Spastic Tone None Common None Fasciculations Mild Severe None Atrophy Myopathic LMN UMN Sign
    • 16. Location, Location, Location
      • Hemisphere
      • Brainstem
      • Cerebellum
      • Cord
      • AHC
      • Root
      • Plexus
      • Nerve
      • NMJ
      • Muscle
    • 17. Location, Location, Location
      • Hemisphere
      • Brainstem
      • Cerebellum
      • Cord
      • AHC
      • Root
      • Plexus
      • Nerve
      • NMJ
      • Muscle
    • 18. Location, Location, Location
      • Hemisphere
      • Brainstem
      • Cerebellum
      • Cord
      • AHC
      • Root
      • Plexus
      • Nerve
      • NMJ
      • Muscle
    • 19. Location, Location, Location
      • Hemisphere
      • Brainstem
      • Cerebellum
      • Cord
      • AHC
      • Root
      • Plexus
      • Nerve
      • NMJ
      • 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.
      • Now known as the March of Dimes.
    • 21. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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
      • Diplopia, Dysarthria, Dysphagia, Dyspnea
      • Deficits are fatigable.
      • 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.
    • 28. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • Improperly canned foods contaminated with the exotoxin of Clostridium botulinum.
      • 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.
    • 30. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • Decreased acetylcholinesterase activity that causes excessive acetylcholine at the NMJ.
      • Symptoms vary 5 to 12 hours after exposure.
      • Tx with atropine.
    • 32. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • 1933, elected President of the US with symmetrical lower extremity weakness.
    • 34. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • Associated with UMN lesions.
      • Caused by compressive or noncompressive lesion.
      • Central Cord Syndrome:
        • Most often caused by syringomyelia and intramedullary cord tumors.
        • 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”.
    • 36. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • Amyotrophic Lateral Sclerosis :
        • 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).
        • Asymmetric limb weakness.
    • 38. Post-Polio Syndrome
      • 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.
      • Small group would develop fever, HA, meningeal irritation, soreness, and asymmetric paralysis.
      • Introduction of the inactivated polio vaccine in 1954.
    • 39. West Nile Virus
      • RNA flavivirus.
      • 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.
      • 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:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • 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.
      • Marked disturbances in autonomic function can occur 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.
    • 42. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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).
      • 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.
      • Minor sensory symptoms but constitutional signs are usually absent.
      • 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.
    • 44. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • Emerald Green.
      • Arsenicosis - chronic arsenic poisoning from drinking water, New Hampshire.
      • Check hair follicles.
      • Tx: Chelating agents.
    • 46. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • 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.
      • Look for trigger.
    • 48. Acute Weakness
      • Spinal Cord Disease:
        • Transverse Myelitis
        • AHC Disease
        • Epidural and Extradural Tumor or Spinal Cord tumor
        • Epidural Hematoma
        • Herniated intervertebral disk
      • Peripheral Nerve Disease:
        • Guillain-Barre’ Syndrome
        • Acute intermittent porphyria
        • Arsenic poisoning
        • 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.
      • 1916, Guillain and Barré described the CSF findings.
      • Acquired demyelinating disorders of the peripheral nervous system with an acute onset.
      • Heterogeneous syndrome with several variants:
        • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)  
        • Miller-Fisher Syndrome
        • Acute axon loss ("axonal") polyradiculopathy    
          • Acute motor axonal neuropathy    
          • Acute motor-sensory axonal neuropathy
        • Other variants
    • 50. GBS: Pathogenesis
      • 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.
      • 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.
      • 15% develop oculomotor weakness.
      • 80% develop parasthesias, usually mild.
      • Often severe low back pain.
      • 30% develop severe respiratory muscle weakness requiring ventilatory support.
      • 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.
      • Campylobacter jejuni: Most common, worse prognosis
      • HIV: any stage.
      • Other infections: CMV, EBV, Hepatitis, Mycoplasma pneumoniae, Influenza, Herpes.
      • Vaccination: Influenza, Meningococcal (MCV4; report to VAERS).
      • Small percentage: Surgery, Trauma, BM Transplant, TNF-alpha antagonist, and systemic illnesses.
    • 53. AIDP
      • Most Common in US and Europe. 85-90% of cases.
      • Peripheral nerve myelin is the target of immune attack.
      • 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.
      • Ophthalmoplegia, ataxia, and areflexia. And ~1/3 will have some extremity weakness.
      • Associated with antibodies to ganglioside GQ1b in 85-90%.
    • 55. AMAN/AMSAN
      • First recognized in 1986.
      • 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.
      • 60-70% preceded by Campylobacter jejuni infection.
      • 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.
    • 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.
      • 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.
    • 57. GBS: Diagnostic Criteria
      • Required features: Progressive weakness and Areflexia.
      • Supportive features include:
        • Progression of symptoms over days to four weeks
        • Relative symmetry
        • Mild sensory symptoms or signs
        • Cranial nerve involvement, especially bilateral facial nerve weakness
        • Recovery starting two to four weeks after progression halts
        • Autonomic dysfunction
        • No fever at the onset
        • Elevated protein in CSF with a cell count <10 mm3
        • Electrodiagnostic abnormalities consistent with GBS
      • GBS doubtful :
        • Sensory level
        • Marked, persistent asymmetry of weakness
        • Severe and persistent bowel and bladder dysfunction
        • 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:
        • 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.
      • Predictors of intubation:
        • Time of onset to admission less than seven days
        • Inability to cough
        • Inability to stand
        • Inability to lift the elbows
        • Inability to lift the head
        • 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.
      • Pain Control: 40-50% pts have neuropathic pain.
      • Plasma Exchange: remove circulating antibodies, complement, and other agents.
      • IVIG : unknown, possibly anti-idiotypic antibodies interfering with T and B cells.
    • 60. GBS: Treatment
      • AAN Observations:
        • Treatment with plasma exchange or IVIG hastens recovery from GBS.
        • The beneficial effects of plasma exchange and IVIG are equivalent.
        • Combining the two treatments 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.
        • 1) Protracted symmetric ascending paralysis over 10-13 days
        • 2) Facial paralysis
        • 3) Bladder and bowel dysfunction
        • 4) Numbness and Dysaesthesia
        • 5) Absence of meningismus
        • 6) Descending pattern of recovery
        • 7) Fever
        • 8) Permanent paralysis
      • Disease incidence in age group x symptom probability.
      • Six of eight favored GBS.
      J Med Biogr. 11: 232–240 (2003)
    • 62. FINAL ANSWER
      • Perform LP, anti GQ1b, EMG and NCS.
      • GBS, possibly AMAN variant.
    • 63. References
      • www.uptodate.com
      • Harrisons
      • Goetz: Textbook of Clinical Neurology, 2nd ed. Copyright © 2003 Saunders, An Imprint of Elsevier .
      • 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.
      • 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)