Downbeat nystagmus. Issue 1101 (13th December 2016)

• Record Type: Journal Article
• Authors: Sharma, Soumya; Gupta, Anu; Deshmukh, Aviraj; Singh, Satbir; Puri, Vinod
• Is Part Of: Postgraduate medical journal. Volume 93:Issue 1101(2017)
• Page Start: 437
• Page End: 437
• Publication Date: 2016-12-13
• Subjects: Medicine -- Periodicals; 610
• DOI: 10.1136/postgradmedj-2016-134457
• Languages: English
• ISSNs: 0032-5473

Abstract

ABSTRACT: A 22-year-old man presented with progressive weakness and sensory loss in the left arm and leg for 1 year, imbalance while walking for 6 months, oscillopsia, dysphagia and urinary urgency for 3 months. Examination revealed a short neck, low posterior hairline and scoliosis. Downbeat nystagmus (DBN) was appreciated in the primary position of gaze. It increased in amplitude when the patient looked down and out, the so-called Daroff's sign1 (see online supplementary video ). He also had lid nystagmus, right partial Horner's syndrome, lower cranial nerve palsies, bipyramidal involvement, ataxia (sensory and cerebellar), and left pansensory loss (up to C3). MRI of the spine revealed a 6 mm descent of the cerebellar tonsils below the foramen magnum (Arnold Chiari malformation type 1; figure 1 A (arrow)) with syringomyelia extending from the C2 to D5 vertebral level (figure 1 A (arrowhead)). Basilar invagination was noted on CT imaging of the spine, with extension of the odontoid process 10 mm above the Chamberlain line (figure 1 B (arrow)).