Japanese encephalitis (JE) shows characteristic brain lesions, including bilateral thalamus, midbrain, internal capsule, basal ganglia, and occasionally involves an anterior horn cell. We encountered a case of a 44-year-old man who initially presented with encephalitis, which was finally diagnosed as Japanese encephalomyelitis with syringomyelia. The patient showed severe motor weakness followed by delayed recovery of functional motor activities. Cervical magnetic resonance imaging showed syrinx formation at the C5 level suggesting myelitis, and abnormal electromyographic findings were noted. Clinicians should consider the possibility that the spinal cord may be involved; an example would be syringomyelia due to myelitis in a case of JE presenting with severe and prolonged motor weakness.
Citations
Guillain-Barre syndrome (GBS) and syringomyelia are diseases of different entities. GBS is an acute post-infectious autoimmune disease which is mediated by autoantibodies against the myelin of peripheral nerves. Syringomyelia is a chronic disease characterized by a cavity extending longitudinally inside the spinal cord. A 67-year-old man is being hospitalized due to severe numbness and ascending weakness in all limbs. On neurological examination, the motor power of all limbs are decreased and show absence of deep tendon reflexes (DTRs). The patient is being diagnosed with GBS on the basis of the acute clinical course, nerve conduction studies of segmental demyelinating polyneuropathy, and a finding of albuminocytologic dissociation in the cerebrospinal fluid. The patient is presented with a new set of symptoms thereafter, which composes of sensory changes in the upper extremities, the urinary dysfunction including frequency and residual urine, spastic bilateral lower extremities, and increased reflexes of the knee and the biceps at follow-up examinations. The spinal magnetic resonance imaging in the sagittal section revealed a syrinx cavity between the fifth cervical and the first thoracic vertebral segment in the cord. The somatosensory evoked potential show sensory pathway defects between both the brachial plexus and the brain stem. Thus, this patient is being diagnosed with both GBS and syringomyelia. We report a case of symptomatic syringomyelia coexisting with GBS. Since the GBS is presented with a progressive muscle weakness and reduced DTRs, the muscle weakness and stiffness in the extremities suggests a concurrent syringomyelia might be easily overlooked.
Citations
The association between pediatric Chiari malformation and the development of syringomyelia has been well documented. Scoliosis in the patient with syringomyelia is thought to be secondary to anterior horn cell damage, which innervate the muscles of trunk, by an asymmetrically expanded syrinx. In pediatric patients, the neurologic signs and symptoms due to Chiari malformation and syringomyelia show much lower frequency but the incidence of scoliosis is very high. Thus, the MRI study for the diagnosis of the underlying syringomyelia and Chiari malfornation is essential in pediatric scoliosis patients, which may otherwise be misdiagnosed for idiopathic scoliosis.
We present a case of Chiari type I malformation associated with syringomyelia and scoliosis.
Syringomyelia can occur as a complication of tuberculous meningitis despite of appropriate chemotherapy and almost it is founded with motor and sensory disturbances.
We have experienced a case of an extensive syringomyelia & syringobulbia in tuberculous
meningitis who complained only mild numbness & diplopia without specific motor disturbance.
So we think that we should not overlook even mild symptoms and have to evaluate the syringomyelia timely by radiographic study.
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