Introduction: Devic’s neuromyelitis optica is an autoimmune and central nervous system demyelinating disease. It mainly affects the spinal chord and optic nerves. Considered for a long time as a special form of Multiple Sclerosis (MS), it is distinguished clinically by its rarity and poor prognostic, biologically by NMO (IgG) antibody, radiologically by extended myelitis. Treatment include mega dose of methylprednisolone during relapses and immunosuppressive drug in prevention of recurrences. Observation: Devic’s disease manifests itself in outbreaks of neuritis optic or myelitis. We report the case of a patient who had no particular antecedents. She presented a spastic tetraparesis, accompanied by a decreasing left visual acuity (4/10th); the all evolving by thrust since one year. Spinal chord lesions on MRI, second stage brain damages appearance affecting the white substance and the discovery in the patient serum of anti-aquaporin 4 antibodies (anti-AQP4 Ab) allowed the diagnosis of Devic’s disease. Conclusion: Diagnosis of Devic’s neuromyelitis optica that was initially based on clinical examination and radiological assessment is currently confirmed by the discovery in the patient serum of the NMO immunoglobulin G directed against Aquaporin 4.
The neuromyelitis optica (NMO) has been described for the first time by Eugène Devic [
Miss TY, 35 years old, was admitted to a neurology department in our town. She had a spastic paraparesis associated with a decrease of the left eye visual acuity. She had no particular antecedents. The symptomatology had evolved for about a week. Three days after her hospitalization, left eye blindness was installed, associated with urinary retention. The thoracic spine Magnetic Resonance Imaging (MRI) involved highlighted an extended intra medullar signal anomaly from T9 to T12. This anomaly appeared in hypo intensity on EST1 sequences, in hyper intensity on EST2 sequences [
to T7 on EST2 [
Two months later, she introduced a new thrust. This time, there was a bilateral reduction in visual acuity and the reappearance of sphincter disorders. She was again hospitalized and under corticosteroid therapy at the same doses as above. A week later, her general condition had improved. Six months after this last episode, she introduced a third thrust marked by the worsening of the unrest. It was about quadriplegia, binocular blindness and sphincter disorders. The MRI of cervical spine, thoracic spine, skull and brain were conducted. At neck and chest, the lesions described above were increased, revealing themselves in the form of an extension of the intra spinal chord hyper intensities from C2 to T10 [
NMO is a central nervous system demyelinating disease. It preferentially affects the spinal chord and optic nerves [
Some authors state that these associations justify the non inclusion of these cases in the nosological framework of Devic’s syndrome [
This entity is not a hereditary disease, but there is a reported case of 2 homozygous sisters [
The viral and bacterial infections preceding or accompanying the NMO are a known phenomenon. The influenza-like illness that precedes the onset of neurological disease has been reported in about 25% - 30% of cases [
Clinically, Devic’s disease is characterized by acute episodes of optic neuritis and myelitis. Optic neuritis is a retrobulbar-like optic neuritis, unilateral or bilateral, often more severe than that described in MS. Cases of oculomotor abnormalities, Claude Bernard Horner’s syndrome and nystagmus have been reported by some authors [
The myelitis is transverse, acute and is clinically expressed by the association of motor, sphincter and sensitive disorders. Its onset is on average of 2 years [
Spinal chord MRI is characteristic. This imaging technic highlights an intra medullar hyper intensity on the EST2 and STIR sequences. Signal abnormalities extend to more than 3 metameres in the sagittal plan [
During the third thrust, the neck and thoracic spine MRI performed in our patient have highlighted the hyper intensity lesions on T2 and STIR sequences that stretched to 5 metameres for the cervical spine and 10 metameres for the thoracic spine. Brain MRI was normal at the beginning.
At the last thrust, it demonstrated bilateral protuberantial and sub-ependymal demyelinization lesions.
The analysis of CSF and blood makes it possible to differentiate on the one hand between the infectious and systemic inflammatory etiologies and on the other hand between NMO and MS [
The current criteria of Devic neuromyelitis are based on the combination of mandatory criteria (optic neuritis, acute myelitis and absence of signs in favor of an attack other than that of the optic nerve and marrow) to a secondary criterion (Normal MRI, extensive medullary lesion on more than 3 vertebrae, plethocytosis of the cerebrospinal fluid) or two minor secondary endpoints (bilateral optic neuropathy, severe optic neuropathy with 1/10th visual acuity and severe muscle deficiency At least one member) [
However, these criteria do not allow the diagnosis of Devic neuromyelitis subjects with neurological symptoms in regions of the central nervous system other than the optic nerve or spinal cord, and those with cerebral MRI showing lesions identical to Those of multiple sclerosis [
Thrust treatment starts classically with daily infusions of 500 mg to 1000 mg of methyl prednisolone for five to seven days. Secondarily the relay is orally taken by the corticosteroid at the dose of 1 mg/kg/d [
Devic’s disease is an extremely rare pathology. It is a rare clinical syndrome involving a unilateral or bilateral optic neuritis with a transverse myelitis. Devic’s neuromyelitis optica Clinically similar to multiple sclerosis, albeit with a more rapid and more severe, it is a distinct clinical and pathological entity. The diagnosis of NMO should be evoked facing clinical symptoms of increased aggravation of spinal and ocular neuritis. On MRI, lesions are localized first to the spinal cord and then to the brain. The diagnosis of certainty is made by the discovery of the anti-aquaporin4 antibody in the patient’s serum.
None.
Acko-Ohui, E.V., Setchéou, A., Garba, I., Acko, U.V., Konan, A. and Yapo, P. (2018) Devic’s Disease: A MRI Finding Case. Open Journal of Radiology, 8, 22-29. https://doi.org/10.4236/ojrad.2018.81003