Lu Lu Waterhouse, MD PGY-3 July 2, 2012

12yo previously healthy female who awoke with weakness with numbness in all extremities

OSH: got Ativan, Zofran, and Pepcid. No imaging or labs done. PMHx: Healthy. Got Menactra, Dtap, and HPV immunizations 10 days ago. MEDS: Ibuprofen, ALLERGIES: NKDA FHx: Anxiety in mother and maternal GM SHx: Lives with family. No h/o abuse, psych issues. Family recently cut ties with paternal GM with whom patient was close.

Mild upper back pain prior to going to sleep. Otherwise normal activity, PO, etc that day. Loss of sensation along middle of abdomen.

VS: temp 37.2, HR 106, RR 20, BP 96/54, O2 sat 94% on RA; Weight 37.2kg GEN: WDWN in NAD, lying on stretcher. HEENT, CV, RESP, ABD SKIN: normal NEURO:

MS: Alert, oriented, talking appropriately CN: II-XII intact, no visual changes Motor: Can minimally move UEs with diminishing strength from shoulders and unable to move fingers; no movement of Les, decreased tone Sensation: normal in all extremities. Subjective decrease in light touch and pinprick in umbilical dermatome. Reflexes: trace in biceps and brachioradialis, 1+ with distraction of L patella, could not elicit R patella or Achilles tendon bilat. Babinski negative bilat Gait/Coordination: Unable to perform.

CBC: WBC 7 (11B/60N/23L), Hb 13, Hct 38, Plt 246 CMP: Na 137, K 4.4, Cl 101, bicarb 24, BUN 13, Cr 0.6, gluc 115, Ca 9.3, prot 7.5, alb 4.3, bili 0.7, alk phos 215, ALT 23, AST 30 Tox screen (urine & serum): negative U/A: trace prot, neg nitrites and LE, 1 WBC, 1 RBC, neg bacteria CSF: opening pressure 22, WBC <1, RBC 151, gluc 55, prot 23, diff: 3B/45N/41L/11M, gram stain negative CT scan head: negative

Neurology has been consulted. Differential Diagnosis??

Cerebral Cortex

Spinal Cord

Intracranial hemorrhage (SAH)* Head trauma (SDH, EDH)* Stroke (thrombotic or embolic)* Brain tumor* Postictal Todd paralysis Hemisyndrome migraine Acute disseminated encephalomyelitis (ADEM) Multiple Sclerosis (1st attack) Spinal cord trauma (EDH)* Spinal cord tumor* Discitis Epidural abscess* Transverse myelitis Vascular Abnormality

Anterior Horn Cell Peripheral Nerve

Poliomyelitis* Guillain-Barre Syndrome* Paralytic fish/shellfish poisoning* Heavy metal poisoning* Acute intermittent porphyria* Botulism* Mysthenia gravis* Organophosphate poisoning* Tick paralysis* Rhabdomyolysis* Viral myositis Pyomyositis Periodic paralysis Trichinellosis

Neuromuscular junction

Muscle

Upper Motor Neuron

Lower Motor Neuron

Other: Electrolyte disturbances, drug-related, Conversion disorder

Segmental spinal cord injury caused by acute inflammation (no evidence of compressive lesion) 1-5 cases per million annually Symptoms: motor and sensory deficits attributable to involvement of one or both sides of the spinal cord
Paresthesias, weakness, sphincter dysfunction (bowel and bladder)
Almost all have leg weakness; arm weakness if inflammation is in cervical spine Tight banding or girdle-like sensation around trunk Back and radicular pain is common

Onset can be hours (hyper-acute) to several weeks (subacute)

Mostly idiopathic, presumably autoimmune

May be manifestation of an acquired demyelinating disease (eg, ADEM, MS, Devic disease)

Almost of patients have a preceding infection/febrile illness One study had of patients with preceding vaccinations Associated with connective tissue diseases (eg, SLE, Sjogrens, Scleroderma, Antiphospholipid antibody syndrome, Ankylosing spondylitis, Rheumatoid arthritis)

TM with partial and asymmetric cord involvement is more likely to develop into MS Presence of oligoclonal bands in CSF makes TM more likely to evolve into MS

MRI of the spine

CSF studies: abnormal in about of patients

Elevated protein (usually 100-120mg/100mL) Moderate lymphocytosis (usually <100/mm3) Send for oligoclonal bands in CSF and serum

Usually there is swelling of affected segments (thoracic most common, but can be cervical) Typically shows gadolinium-enhancing signal abnormality

Treatment

Most often treated with parenteral corticosteroid therapy (ASAP), but there is limited evidence that this alters outcomes May receive other immunosuppressive and immunomodulatory therapies if TM associated with systemic autoimmune disease

More likely to have respiratory involvement if inflammation in cervical spine -> usually admitted to PICU Most patients with idiopathic TM have at least partial recovery, usually within 1-3 months 40% have some degree of persistent disability