acute transverse myelitis 02.26.2014

8/12/2019 Acute Transverse Myelitis 02.26.2014

1/23

2/26/2014

Morning Report Nolan Sandygren, PGY-2


2/23

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


3/23

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


4/23

Outside Hospital

Taken to OSH ED

Plain films of T-spine, L-spine, pelvis, and bilaterallower extremities normal

Discharged home, advised to watch carefully

Parents concerned that not moving her legs at all

Brought to PCMC ED later that night


5/23

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


6/23

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, butfoster parents dont think that she was directly physically abused

ROS otherwise negative


7/23

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 in

LEs. No tone against gravity, doesnt support weight when held in standing position. 1+ patellar reflexes, 2+ achilles, biceps, and

triceps reflexes. Decreased rectal tone.


8/23

10 m.o. girl presenting with lower extremity weakness x

1 day after a fall.


9/23

Differential Diagnosis

Neuro:

Guillain-Barr syndrome (acute inflammatory demyelinating

polyradiculoneuropathy)

Acute disseminated encephalomyelitis (ADEM)

Multiple Sclerosis

Transverse myelitis Epilepsy

Migraine

Musculoskeletal:

Spinal cord injury or compression

Trauma

Malignancy:

Lymphoma

High grade glioma

Metabolic:

Vitamin deficiency (B12, folate)

Infectious:

Acute bacterial/viral encephalitis or meningitis

Viral myelitis

Lyme disease

West Nile virus

Syphilis

HIV

Inflammatory/Vasculitis:

Systemic lupus erythematosus

Antiphospholipid antibody syndrome

Behcet disease

Vascular:

Spinal cord infarction


10/23

ED Course

C-spine XR, with overread of outside films -> no fx

Planned to admit to trauma, with MRI of T-spine and L-

spine pending


11/23

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


12/23

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 vertebrallevels, 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 orposterior element fractures noted. No intervertebral disc herniation

Marked urinary bladder distention.


13/23

Transverse Myelitis


14/23

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


15/23

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


16/23

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)


17/23

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 orelevated IgGindex orgadolinium enhancement

Progression to nadir between 4 hours and 21 days


18/23

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


19/23

Treatment

High-dose IV glucocorticoid treatment x 3-5 days

Methylprednisolone 15 mg/kg/day IV div q6h x 3days

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)


20/23

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


21/23

Labs & Micro

CSF:

Protein: 12

Glucose: 55

Cell count: WBC


22/23

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.


23/23

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.

acute transverse myelitis 02.26.2014

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