Abstract

Chiari type I malformation with cervicothoracic syringomyelia although very common in clinical practice usually in children can progress slowly and mimic muscular dystrophies in adulthood. We present a rare adult case of Chiari type I malformation with cervicothoracic syringomyelia subterfuge as Flail arm syndrome. A 44-year-old man was diagnosed with congenital type I Chiari malformation with cervicothoracic syringomyelia about 21 years ago without surgery. His health status deteriorated over the years until 21 days prior to presentation when he had severe pain in the right knee. In his upper limbs, he had bilateral corresponding severe weakness of 0/5 proximal strength and 0/5 strength in his distal muscles. Magnetic resonance imaging (MRI) revealed an enlargement of the spinal cord from C1-C4 level with a mass that appeared hypo-dense on T1 and hyperdense on T2. Syringomyelia is a potentially serious neurologic condition that can mimic other neuromuscular disorders. Early detection and diagnosis with MRI is crucial to avoid irreversible neurological complications. We suggest that whether asymptomatic or symptomatic, decompressive surgery should be carried out to allow for free flow of cerebrospinal fluid thereby improving the quality of life for the patient.

Highlights

  • A 44-year-old man presented with worsening chronic motor symptoms for 21 years. He is a known case of congenital type I Chiari malformation with cervicothoracic syringomyelia diagnosed about 21 years ago without surgery

  • Most of the authors have come to the conclusion that malformation of the mesodermal occipital somite is accountable for the tiny nature of the posterior fossa leading to the anomalous CSF flow at the level of the foramen magnum.[1,11,12]

  • Most disorders originate from the cervical spinal cord and can enlarge superiorly into the brainstem leading to the formation of syringobulbia or inferiorly into the thoracic and sometimes the lumbar regions.[3,4]

Read more

Summary

Case Report

A 44-year-old man presented with worsening chronic motor symptoms for 21 years He is a known case of congenital type I Chiari malformation with cervicothoracic syringomyelia diagnosed about 21 years ago without surgery. Electromyography (EMG) of ically made up of excess cerebrospinal fluid his lower extremities spared (Figure 1A) In his upper extremities revealed of diffuse (CSF), cumulates inside the spinal cord the upper limbs, he had bilateral correspon- chronic neurogenic changes in almost all either within the parenchyma as a focal ding severe weakness of 0/5 proximal the nerves but normal in all the nerves in the dilation of the central canal.[1,2,3,4,5] Chiari mal- strength and 0/5 strength in his distal mus- lower extremities.

Non Discussion
Mechanisms underlying the formation
Proceedings of the staff meetings Mayo
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call