Arnold-Chiari malformation type I and chronic vocal cord paralysis: case report

Case presentation: MCOLS, 53 years old, born in Saboeiro, Ceará. 28 years ago, she gradually developed dysphonia. Eight years later, she developed symmetrical paresis in her hands and paresthesia and pain in all four limbs, asymmetrical, particularly on the left. From then on, she developed difficulty walking and recurrent attacks of neck pain and left-sided hemicranial headache, the latter appearing in stitches, with strong intensity for up to 10 minutes, triggered by coughing or throbbing, with at least five episodes per month. On examination: dysphonic speech, symmetric palate elevation and reduced amplitude. Muscle strength grade 4: deltoid, rhomboid, biceps brachii, triceps, pronator teres, extensor carpi radialis, flexor carpi radialis, interossei and abductor pollicis brevis bilaterally. Grade 1 reflexes: styloradial, bicipital and triceps bilaterally. In sensitivity assessment, there is “shawl” hypoesthesia of C4-C6. Complementary exams: 10/21/2011 Laryngoscopy: nodular formation in the middle third of both vocal folds compatible with an intrachordal cyst; presence of irregular glottal cleft with double spindle predominance. 04/03/2017 Magnetic resonance imaging of the cervical spine: posterolateral disc protrusions at levels C5-C7, determining an impression on the ventral surface of the dural sac, causing a reduction in the amplitude of the left intervertebral foramen at C6-C7; herniation of the cerebellar tonsils through the foramen magnum by approximately 0.9 cm; extensive syringomyelic cavity from C2 to the thoracic segment. 03/13/2018 Electroneuromyography of the upper limbs: acute and chronic radiculopathy of the C7 root on the left side, with signs of active and chronic denervation in the flexor carpi radialis, extensor carpi radialis and left triceps muscles; sensory and motor neuropathy of the median nerve at the level of the wrist, bilaterally, compatible with severe carpal tunnel syndrome bilaterally. Discussion: Arnold-Chiari malformations are defined as a spectrum of abnormalities of the posterior part of the brain, involving the cerebellum, brain stem, skull base and spinal cord. Type I is the most common and is characterized by the caudal displacement of the cerebellar tonsils and the medial and inferior part of the posterior lobe of the cerebellum through the spinal canal, above 3 to 5 mm from the foramen magnum. The patient presented multiradiculopathy from C5 to T1, bilateral preganglionic hypoesthesia, C4-C6 hypoesthesia, motor deficit of the upper limbs caused by type I malformation. It was associated with extensive syringohydromyelia, dysphonia related to bulbar paresis, cervicogenic paroxysmal headache and cyst on the vocal folds. After a surgical approach, the headache, dysphonia, sensory and motor deficits gradually resolved after surgical treatment of the Chiari malformation, as well as neurophysiological recovery from the assessed radiculopathy.