PMH: No medications; Immunizations UTD No hospitalizations or surgeries Speech Delay. Birth Hx: 24 WGA, NICU x6 months

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3 HPI: 6 months of weakness and parathesias- originally in both feet x 2-3 months, then resolved. Now with parathesias and weakness in fingers x 1 week. Seen by podiatrist and given custom in-soles 1 month prior to presentation, difficulty maintaining balance PMH: No medications; Immunizations UTD No hospitalizations or surgeries Speech Delay Birth Hx: 24 WGA, NICU x6 months Fam Hx: Negative, Brother- healthy

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5 Strength 3/5 LUE, deltoid and wrist ext 4/5 LUE, slight drift with LUE Reflexes: UE 2+, LE 3+ at patella bilaterally, R ankle clonus x3 beats (No clonus to L ankle), increased tone at hamstrings bilaterally c/o feeling pins and needles, decreased vibration to fingers (7 secs), absent in toes Gait: slightly wide-based

6 Which category does her presentation suggest? 6

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10 Clinical Sign Upper Motor Neuron Lower Motor Neuron Tone Increased Decreased Reflexes Increased Decreased Weakness Present Present Babinski Present Absent Atrophy Absent* Possible Fasiculations Absent Possible *can be present due to disuse ACUTE or CHRONIC spinal cord lesions can mimic LMN disease

11 Acute Disseminated Encephalomyelitis (ADEM) Lambert Eaton Medication Side Effect Guillain Barre Syndrome (GBS) Systemic Lupus Erythematosus (SLE) Multiple Sclerosis/Clinically Isolated Syndrome Electrolyte Disorder Acute Transverse Myelitis CNS Malignancy Neuromyelitis Optica (NMO) Infectious Etiology Cerebrovascular Disease

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14 Initially flaccid, then evolves to spastic paresis with UMN signs Well-defined sensory level cervical 14

15 depends 15

16 Corticosteroids 16

17 Acute Disseminated Encephalomyelitis (ADEM) Optic Neuritis Medication Side Effect Guillain Barre Syndrome (GBS) Systemic Lupus Erythematosus (SLE) Multiple Sclerosis/Clinically Isolated Syndrome Electrolyte Disorder Acute Transverse Myelitis CNS Malignancy Neuromyelitis Optica (NMO) Infectious Etiology Cerebrovascular Disease

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19 Corticosteroids 19

20 Acute Disseminated Encephalomyelitis (ADEM) Optic Neuritis Medication Side Effect Guillain Barre Syndrome (GBS) Systemic Lupus Erythematosus (SLE) Multiple Sclerosis/Clinically Isolated Syndrome Electrolyte Disorder Acute Transverse Myelitis CNS Malignancy Neuromyelitis Optica (NMO) Infectious Etiology Cerebrovascular Disease

21 Optic neuritis- Spinal lesion- 21

22 Anti-aquaporin-4 antibody (NMO-IgG) steroids 22

23 Often misdiagnosed as multiple sclerosis 1. DOES NOT antibodies severe anti-aquaporin 4

24 Acute Disseminated Encephalomyelitis (ADEM) Optic Neuritis Medication Side Effect Guillain Barre Syndrome (GBS) Systemic Lupus Erythematosus (SLE) Multiple Sclerosis/Clinically Isolated Syndrome Electrolyte Disorder Acute Transverse Myelitis CNS Malignancy Neuromyelitis Optica (NMO) Infectious Etiology Cerebrovascular Disease

25 encephalopathy 25

26 asymmetric rule out infection 26

27 Corticosteroids

28 Acute Disseminated Encephalomyelitis (ADEM) Optic Neuritis Medication Side Effect Guillain Barre Syndrome (GBS) Systemic Lupus Erythematosus (SLE) Multiple Sclerosis/Clinically Isolated Syndrome Electrolyte Disorder Acute Transverse Myelitis CNS Malignancy Neuromyelitis Optica (NMO) Infectious Etiology Cerebrovascular Disease

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30 Clinically Isolated Syndrome-

31 nonencephalopathic, clinical CNS events with presumed inflammatory cause, separated by more than 30 days and involving more than one area 2. One nonencephalopathic episode typical of MS associated with MRI findings and a f/u MRI showing at least one new lesion consistent with criteria

32 periventricular Margins better defined

33 Acute attacks- Steroids Long term- Immunomodulatory agents

34 Acute Disseminated Encephalomyelitis (ADEM) Optic Neuritis Medication Side Effect Guillain Barre Syndrome (GBS) Systemic Lupus Erythematosus (SLE) Multiple Sclerosis/Clinically Isolated Syndrome Electrolyte Disorder Acute Transverse Myelitis CNS Malignancy Neuromyelitis Optica (NMO) Infectious Etiology Cerebrovascular Disease

35 peripheral nerves Most common cause of acute flaccid paralysis in infants and children* Campylobacter jejuni

36 diminished or absent reflexes

37 albuminocytologic dissociation

38 IVIG**

39 2. How will you work up and ultimately treat your patient? Specific Work-Up: MRI LP- MBP 23 (range 0-5), IgG 7.7 (range 0-6) Oligocloncal Bands 10 (range 0-1) Evoked potentials- visual testing indicates profound and diffuse slowing of conduction consistent with demyelination bilaterally. Aquaporin-4 Receptor antibody 0 (range <4) ACE level serum- 18 (low); CSF1.3 (normal)

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41 Final Diagnosis: Multiple Sclerosis

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