Painful tonic spasms and brainstem involvement in a patient with neuromyelitis optica spectrum disorder

Abstract

Neuromyelitis optica (NMO) is an inflammatory-demyelinating disease of the central nervous system classically characterized by optic neuritis and severe myelitis. New diagnostic criteria defined neuromyelitis optica spectrum disorder as limited forms of NMO or diverse neurologic presentations in the presence of specific antiaquaporin-4 antibodies. We report the case of a 57-year-old woman admitted in our department for recurrent attacks of optic neuritis, tetraparesis with severe painful tonic spasms of the left limbs and brainstem involvement. Painful tonic spasms have been described as movement disorders associated with multiple sclerosis, but a growing number of reports describe them in cases of NMO.

Neurorehabilitation in basilar artery occlusion in a young patient with thrombophilia and antiphospholipid antibody syndrome – a case report

“…Differential diagnosis with other autoimmune disorders such as multiple sclerosis or Balo-like lesions and neuromyelitis optica (NMO) was discussed. In the case of Balo-like lesions, the lesions are characteristic, with rings of demyelination, surrounded by partially demyelinated regions, reflecting concentricity within the lesion (6), and even though brainstem involvement, with or without transverse myelitis, is rarely seen in the classic type of NMO, this diagnosis was also ruled out by MRI which showed an ischemic pontine lesion, knowing that the most characteristic brainstem lesion in NMO involves the area postrema (7).…”

Prevalence and Pattern of Craniofacial Pain and Headache in Danish Patients with Neuromyelitis Optica Spectrum Disorder

“…Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease of the central nervous system (CNS) causing severe inflammation and demyelination of the optic nerve (optic neuritis [ON]), the spinal cord (transverse myelitis [TM]) and the brainstem. 1,2 There is a dearth of epidemiological data on the prevalence of NMOSD; conclusive data on the occurrence of NMOSD are lacking and worldwide there are few epidemiological studies on the disorder. [3][4][5] It has been estimated that the prevalence of NMOSD ranges from 1.0-4.4 out of 100,000 in the Western world (Europe and North America).…”

“…Patients were interviewed by telephone due to the distance between the investigator and the patients. Furthermore, the telephone interviews were the most convenient way of interviewing patients with vision and/ 1st telephone call Excluded (2 patients) • Did not answer telephone call (2) Screening ( The minimum score was 0 and the maximum score was 90.…”

Commentary on painful tonic spasms and brainstem involvement in a patient with neuromyelitis optica spectrum disorder

“…Dr Roman-Filip et al [1], has provided an interesting description of the presence of Painful tonic spasms and brainstem involvement in a patient with neuromyelitis optica spectrum disorders (NMOsd). The painful paroxysmal tonic spasms (PPTS) are defined as a localized muscle spasms (in one or more limbs and/or trunk), recurrent, stereotyped accompanied by severe pain and dystonic posturing that usually last for 20-45 seconds [2].…”

“…Initially was described in multiple sclerosis (MS) by Matthews in 1958 [2], has recently been published more frequently in neuromyelitis optica (NMO) [2][3][4][5][6]. The incidence varies from 3-98% [1][2][3][4][5] demyelinating myelopathy patients such as MS, NMO spectrum disorders (NMOsd) or idiopathic. Recently, a study found that the presence of PTSS after a first attack of acute myelitis had a specificity of 100% and sensitivity of 67% for NMOsd [7].…”