English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

Dear Editor, Hydromyelia was first described by Olliver d’Angers as cystic dilatation of the central spinal cord (1). Chiari described the hydromyelic cavity, which was related with or not related with the enlarged central channel, as “syringohydromyelia” (1). Conventional nerve conduction studies and needle electromyography (EMG) is very important in evaluating patients with syringomyelia but the findings may not be specific (2). We report a patient presenting with asymmetric lower extremity atrophy who was diagnosed as having a spinal cord abnormality. A 18-year-old male was admitted with asymmetric lower extremity atrophy and foot deformity. Symptoms were nonprogressive and he did not have sphincter dysfunction. There was no severe infection, systemic disease or difficult birth in his medical history. Neurologic examination showed that his bilateral dorsiflexion strength was 4/5. He had hypoesthesia and impaired vibration and position senses in his right lower extremity. Right patella and Achilles reflexes were absent. Bilateral plantar reflexes were extensor. He had bilateral foot deformities and a skin lesion in the lumbosacral area. Bilateral atrophy predominantly effecting the right side was observed. In the sensory nerve conduction studies, a right peroneal superficial nerve response could not be recorded and the right sural nerve amplitude was low. Motor nerve conduction studies showed low amplitude left peroneal nerve response. Needle EMG revealed long-term and polyphasic motor unit potentials (MUPs) with high amplitudes and showing a sparse pattern in left tensor fascia lata, extensor digitorum longus, and extensor digitorum brevis muscles. There was no pathologic spontaneous activity. Electrophysiologic findings were considered as asymmetric multiradicular involvement affecting the dorsal root ganglion at the lumbosacral level. Lumbosacral magnetic resonance imaging showed syringohydromyelia reaching an anterior-posterior diameter of 2-3 mm and a split cord abnormality in the distal spinal cord at the level of L1 vertebra and spina bifida occulta at the level of L4-L5 and L5-S1 (Figure 1A, 1B). The asymmetric sensory-motor involvement, pyramidal signs, and lumbosacral skin lesion were thought to be caused by the multiradicular effects of syringomyelia involving the dorsal root ganglion at the lumbosacral level. Syringomyelia is a liquid-filled cystic cavitation in the spinal cord parenchyma. It is localized between C2-C9 and it can expand up or down. Usually, it accompanies Chiari type 1 malformation. Other causes of syringomyelia are congenital malformations, postinfectious, post-inflammatory, post-traumatic pathologies, and spinal neoplasia. It is characterized by dissociated sensorial loss, distal weakness in the upper extremities and pyramidal signs in the lower extremities (2). Syrinx may be asymptomatic and it may be detected in spinal cord imaging, incidentally. Some patients may present with a progressive spinal cord deficit, which includes central pain syndrome showing segmental distribution (3). Patients may be sent to the EMG laboratory with a pre-diagnosis of mono/polyneuropathy or trap neuropathy, which may lead to misleading results (1).

Electrophysiologic Abnormalities in a Patient with Syringomyelia Referred for Asymmetrical Lower Limb Atrophy