2/26/2014

Morning Report

Nolan Sandygren, PGY-2

Case

10-month-old girl presenting with lower extremity weakness after a fall yesterday

Fell from bed to carpeted floor, approximately 3 ft Mother was present in room, but facing the other way No LOC, immediately started crying after the fall Initially appeared to be fine, but later became fussy, wanted to held Gradually seemed to get weaker in LEs over next few hours

Became unable to support weight with legs

Previously able to stand with support and walk with assistance

HPI

Otherwise behaving normally

Reportedly weaker cry than usual No difficulty breathing or eating One episode of emesis following evening feeding No fever, cough, congestion, or diarrhea Reported GI illness 5 weeks prior Received influenza vaccine 4 weeks ago

Outside Hospital

Taken to OSH ED

Plain films of T-spine, L-spine, pelvis, and bilateral lower extremities normal Discharged home, advised to watch carefully Parents concerned that not moving her legs at all

Brought to PCMC ED later that night

History

PMHx:

Born full term, no complications Reflux

Development: Normal PSHx: None Meds: None Allergies: NKDA

Immunizations:

Up to date, with flu vaccine 4 weeks ago

History

Family Hx:

Mother with developmental delay, functions at 6 y.o. level 7 y.o. half brother with ADHD

Social Hx:

Lives with foster parents and their 2 y.o. child for past 6 months Possible domestic violence in the home that she came from, but foster parents dont think that she was directly physically abused

ROS otherwise negative

Physical Exam

Vitals:

Weight 7.4 kg, Temp 36.8, HR 120, Resp 38, BP 97/54, SaO2 95% on RA

Exam:

General: awake, alert, interactive, no acute distress HEENT: Atraum, normoceph, EOMI, PERRL, no conjunct, Nl TMs, nares clear, MMM, no oral lesions Neck: supple without LAD, no tenderness or meningismus

CV: RRR, nl S1,S2, no MGR, cap refill < 2s

Lungs: CTAB, no WRR, nl WOB Skin: No petechiae, purpura, jaundice, or cyanosis Abdomen: S/ND/NTTP, nl BS, no HSM Back: No bruising, tenderness, deformity, or bony step-off Extremities: No bruising, edema, or deformities of upper or lower extremities, normal ROM passively Neuro: Makes eye contact and tracks, CN 2-12 grossly in tact. Moves both arms equally, reaches for objects, normal tone. Doesnt spontaneously move either leg at all, but withdraws both lower extremities to nailbed pressure. Decreased sensation i n LEs. No tone against gravity, doesnt support weight when held in standing position. 1+ patellar reflexes, 2+ achilles, bicep s, and triceps reflexes. Decreased rectal tone.

10 m.o. girl presenting with lower extremity weakness x 1 day after a fall.

Differential Diagnosis

Neuro:

Infectious:

Guillain-Barr syndrome (acute inflammatory demyelinating polyradiculoneuropathy) Acute disseminated encephalomyelitis (ADEM) Multiple Sclerosis Transverse myelitis Epilepsy

Acute bacterial/viral encephalitis or meningitis Viral myelitis Lyme disease West Nile virus Syphilis HIV

Migraine

Inflammatory/Vasculitis:

Musculoskeletal:

Systemic lupus erythematosus Antiphospholipid antibody syndrome Behcet disease

Spinal cord injury or compression Trauma

Malignancy:

Vascular:

Lymphoma High grade glioma

Spinal cord infarction

Metabolic:

Vitamin deficiency (B12, folate)

ED Course

C-spine XR, with overread of outside films -> no fx Planned to admit to trauma, with MRI of T-spine and Lspine pending

MRI T and L spine wo contrast

MRI initially read as normal, with no evidence of traumatic injury

Pt transferred to pediatric service with neuro consult. GBS was working diagnosis given MRI findings, and time course and distribution of symptoms
MRI read changed later that evening

MRI T and L spine wo contrast

MRI Thoracic Spine wo Contrast:

Long segment T2 hyperintensity and mild cord enlargement in midthoracic spinal cord extending approximately 6 vertebral levels, T4-10. No additional findings are noted to indicate a traumatic thoracic spine injury.

MRI Lumbar Spine wo Contrast:

Normal imaging appearance of conus. No traumatic vertebral or posterior element fractures noted. No intervertebral disc herniation Marked urinary bladder distention.

Transverse Myelitis

Acute Transverse Myelitis

Neuro-inflammatory spinal cord disorder Presentation:

Rapid onset of signs and symptoms

Motor, sensory, and/or autonomic dysfunction

Annual incidence: one to eight new cases per million

Classification

Acute partial TM

mild or asymmetric, extends 1-2 vertebral seg

Acute complete TM

near complete symmetric, extends 1-2 vert seg

Longitudinally extensive transverse myelitis (LETM)

complete or incomplete, extending 3+ vert seg

Etiology

Idiopathic TM

Typically occurs as post infectious complication, likely autoimmune process

Secondary (disease-associated) TM

Systemic inflammatory autoimmune condition

MS, neuromyelitis optica, and acute disseminated encephalomyelitis (ADEM)

Diagnostic criteria

Sensory, motor, or autonomic dysfunction attributable to spinal cord Bilateral signs and/or symptoms Clearly defined sensory level No evidence of compressive cord lesion

Inflammation defined by CSF pleocytosis or elevated IgG index or gadolinium enhancement

Progression to nadir between 4 hours and 21 days

Symptoms

Motor

Rapidly progressing paraparesis that can involve UEs

Initial flaccidity followed by spasticity

Sensory

Pain Dysesthesia (burning, wetness, itching, electric shock) Paresthesia (pins and needles)

Autonomic

Increased urinary urgency Bowel and bladder incontinence

Urinary retention, constipation

Sexual dysfunction

Treatment

High-dose IV glucocorticoid treatment x 3-5 days

Methylprednisolone 15 mg/kg/day IV div q6h x 3 days Prednisolone 7.5 mg PO QD x 5 days, followed by taper

Consider plasma exchange for patients with acute TM complicated by motor impairment (5x QOD)

Recovery

Most have at least a partial recovery within 1-3 months

Persistent symptoms in 40% Rapid onset is associated with poorer outcomes Recurrence

25-33% of pts with idiopathic TM up to 70% of pts with disease associated

Pts with acute complete TM have 5-10% risk of MS

Labs & Micro

CSF:

Virology

Protein: 12 Glucose: 55 Cell count: WBC <1, RBC <1, CSF diff: Lymphocytes 30%, Monocytes 70% Culture: no growth

EBV PCR negative CMV PCR negative Varicella PCR negative

Pending at discharge:

ESR: 6, CRP <0.5 B12: 474 ANA IgG not detected

Aquaporin 4 ab, anti-NMO, oligoclonal banding, hypercoagulable workup (homocysteine, lupus anticoagulant reflexive panel, anticardiolipin ab, etc)

Case development

Symptoms evolved during hospitalization and she became hyporeflexic with decreasing sensation, then more spastic.

Significant bladder distention requiring intermittent catheterization q4-6h during hospital stay.
Started to regain strength after initiation of steroids, with continued improvement in weight bearing as outpatient.

References

Beh SC, Greenberg BM, Frohman T, Frohman EM. Transverse myelitis. Neurol Clin 2013; 31:79.
Krishnan C, Kaplin AI, Pardo CA, et al. Demyelinating disorders: update on transverse myelitis. Curr Neurol Neurosci Rep 2006; 6:236. Pidcock FS, Krishnan C, Crawford TO, et al. Acute transverse myelitis in childhood: center-based analysis of 47 cases. Neurology 2007; 68:1474. Wolf VL, Lupo PJ, Lotze TE. Pediatric acute transverse myelitis overview and differential diagnosis. J Child Neurol 2012; 27:1426.