Facial spasms revealing a Parry-Romberg syndrome: A case report.

Introduction and importance: Parry-Romberg syndrome (PRS) is a rare clinical entity characterized by progressive atrophy of the hemifacial region, occasionally associated with systemic manifestations. The presence of facial muscles spasms is exceptional. Case presentation: We report the case of a young woman who presented with progressive atrophy of the right hemiface associated with vitiligo and facial muscles spasms. The diagnosis of Parry Romberg syndrome was retained. Electromyogram showed an intermittent motor unit potential. Cerebral MRI showed atrophy of the muscle and subcutaneous fat of the right hemiface. She received corticosteroid in combination with botulinum toxin injection, which stopped the spasms. Clinical discussion: It is a rare condition with a poorly understood etiology, which is responsible for the delay in diagnosis often noted. The association of this syndrome with neurological signs is exceptional and rarely described in the literature. Injection of botulinum toxin associated with corticosteroids can stop the spasms but only surgery can reduce the facial deformities. Conclusion: Parry-Romberg syndrome is a rare disease, more frequent in women. It poses a real diagnostic problem and its treatment is poorly codified.

Neuromyelitis optica spectrum disorders (NMO-SD) in a Sub-Saharan Africa country: A preliminary study of sixteen Senegalese cases.

BACKGROUND: Neuromyelitis optica (NMO) is an autoimmune disease of the central nervous system. In Sub-Saharan Africa, publications are rare and deal with isolated cases. Our goal was to analyze the characteristics of NMO spectrum disorders in a Senegalese cohort compiled in Dakar. PATIENTS AND METHOD: This was a retrospective descriptive study conducted at the Neurology Department of Fann Teaching Hospital. We included all patients with NMO-SD according to the 2014 diagnostic criteria. RESULTS: Sixteen patients were enrolled, 4 men and 12 women with an average age of 30 years. Ten patients (62.5%) presented an acute myelopathy associated with retrobulbar optic neuritis and 5 (31.25%) had isolated spinal cord injury. Spinal MRI showed abnormal cervical (6 patients), dorsal (4 patients), bulbar-cervical (3 patients) or cervico-dorsal (2 patients) signal extended (≥3 vertebral segments) of the spinal cord. Visual evoked potentials (VEP) showed demyelinating optic nerve involvement in 8 patients. Ten patients were positive to AQP-4 IgG. Systemic corticosteroid therapy was the rule in all patients, associated with azathioprine in 10 of them. The clinical course at 3 months was predominantly favourable (10 patients). CONCLUSION: This cohort is the first one compiled in Dakar. African multicentric epidemiological studies are needed.

Metastatic Spinal Cord Compression Secondary to Liver Cancer.

Metastatic spinal cord compression (MSCC) is a medical emergency that requires rapid diagnosis and treatment to reduce pain, to preserve neurological functioning, and to prolong survival. The diagnosis of liver cancer is often neglected in the differential diagnosis of MSCC. Treatment is usually palliative and evolution is often fatal. This is a case report of a 28-year-old patient living in Mauritania hospitalized in November 2014 at the neurology department of Fann national teaching hospital in Dakar, for the management of a chronic dorsal spinal cord compression. The radiological and laboratory investigations done revealed the metastatic compression originating from a liver cancer with elevated alpha-fetoprotein and aspartate transaminase, positive hepatitis B surface antigen, and multiple metastasis in the lungs, mediastinum, ribs, iliac, and peritoneum. The hip joint X-ray showed a spontaneous fracture of the right femoral neck. The multidisciplinary treatment was palliative and the evolution was fatal within the month of hospitalization. Earlier diagnosis and treatment of MSCC may not have saved the life of the patient but may have prevented much suffering and would likely have prolonged the life of a young man.