Kosin Med J.  2018 Dec;33(2):257-262. 10.7180/kmj.2018.33.2.257.

Acute combined central and peripheral nervous system demyelination: a case report

• Roh YE
• Kim YM

• KMID: 2430744
• DOI: http://doi.org/10.7180/kmj.2018.33.2.257

Abstract

Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.

Keyword

Demyelinating disease; Encephalomyelitis; Guillain-Barre syndrome

MeSH Terms

Ankle
Cerebrospinal Fluid
Cough
Demyelinating Diseases*
Diagnosis
Encephalomyelitis
Encephalomyelitis, Acute Disseminated
Extremities
Female
Fever
Guillain-Barre Syndrome
Humans
Knee
Korea
Leukocytosis
Lymphocytes
Magnetic Resonance Imaging
Nervous System Diseases
Neural Conduction
Neuroimaging
Peripheral Nervous System*
Pons
Reflex, Stretch
Spinal Cord

Figure

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  Fig. 1 Abdomen X-ray showed paralytic ileus.

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  Fig 2, 3 Magnetic resonance imaging studies of brain and thoracolumbar spine. Initial T2 weighted images showed diffuse, subtle, high signal lesion in brain stem (Figure 2) and conus medullaris of spinal cord (arrowhead), and distended bladder due to neurogenic bladder (Figure 3).

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