mycophenolic-acid and Vertigo

Topics: Adult; Albumins; Aphasia; Audiometry, Pure-Tone; Blood-Brain Barrier; Confusion; Corpus Callosum; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Enzyme Inhibitors; Female; Fluorescein Angiography; Glucocorticoids; Hearing Loss; Humans; Immunologic Factors; Magnetic Resonance Imaging; Mycophenolic Acid; Retinal Artery Occlusion; Serum Albumin; Susac Syndrome; Vertigo

Susac syndrome is a rare disorder caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear. These occlusions lead to a characteristic clinical triad of central nervous system dysfunction, visual disturbances and vestibule-cochlear deficits. The diagnosis is based on clinical manifestations and complementary studies, which demonstrate the involvement of three systems. There are different treatments that include various immunosuppressive drugs combinations such as corticosteroids, intravenous immunoglobulin, mycophenolate mofetil, among others. We present the case of a 26-year-old woman with left hearing loss, tinnitus and episodes of recurrent vertigo, four weeks after bilateral blurred vision, cerebellar ataxia and encephalopathy. Magnetic resonance imaging of the brain showed multiple rounded hyperintense lesions in t2 and fluid-attenuated inversion recovery (FLAIR), hypointense in t1, at the middle level of the corpus callosum, internal capsule, cerebellum and right middle cerebellar peduncle. The audiometry evidenced bilateral perceptual hearing loss, predominantly in the left ear. Angiography by optical coherence tomography showed obstruction in the deep layer retina arteries. The Susac syndrome was diagnosed and treatment started with methylprednisolone pulses therapy, intravenously 1000 mg/day for 5 days, followed by maintenance with mycophenolate, which completely reversed the encephalopathy, with persistence of mild ataxia and hearing loss. It is important to know the clinical triad characteristic and the complementary studies necessary to arrive at the diagnosis, since immunosuppressive treatment can often be delayed. Our case had an excellent response to corticosteroids.. El síndrome de Susac es una enfermedad rara causada por oclusión autoinmune de la microvasculatura del cerebro, de la retina y del oído interno, lo que provoca la triada clínica característica de disfunción del sistema nervioso central, trastornos visuales y déficit vestíbulo-coclear. El diagnóstico se basa en las manifestaciones clínicas y en estudios complementarios que demuestren el compromiso de los tres sistemas. Existen diferentes tratamientos que incluyen combinaciones de varios fármacos inmunosupresores como corticoides, inmunoglobulina intravenosa, micofenolato mofetilo, entre otros. Presentamos el caso de una mujer de 26 años que manifestó hipoacusia izquierda, zumbidos y episodios de vértigo recurrente. Cuatro semanas después agregó visión borrosa bilateral, ataxia cerebelosa y encefalopatía. La resonancia magnética de cerebro mostró múltiples lesiones redondeadas hiperintensas en t2 y FLAIR (fluid-attenuated inversion recovery), hipointensas en t1 a nivel medial del cuerpo calloso, cápsula interna, cerebelo y pedúnculo cerebeloso medio derecho. La audiometría evidenció hipoacusia perceptual bilateral a predominio del oído izquierdo y en la angiografía por tomografía de coherencia óptica se observó obstrucción de arterias de la capa profunda de la retina. Se diagnosticó síndrome de Susac y se inició tratamiento con pulsos de metilprednisolona por 5 días y mantenimiento con micofenolato, revirtiendo totalmente la encefalopatía, con persistencia de leve ataxia e hipoacusia. Es importante conocer la triada clínica característica y los estudios complementarios necesarios para arribar al diagnóstico, ya que muchas veces se puede demorar el tratamiento inmunosupresor. Nuestro caso tuvo una excelente respuesta a los corticoides.

Topics: Anti-Inflammatory Agents; Brain Diseases; Computed Tomography Angiography; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Methylprednisolone; Mycophenolic Acid; Neuroprotective Agents; Prednisolone; Pregnancy; Susac Syndrome; Vertigo

Topics: Adult; Autoimmune Diseases; Cyclosporine; Enzyme Inhibitors; Female; Hearing Loss, Sensorineural; Humans; Immunosuppressive Agents; Keratitis; Mycophenolic Acid; Prednisone; Treatment Outcome; Vertigo

The patient was a 55 year-old-woman with chronic renal failure due to idiopathic mesngial deposition of Ig A. She received a second allograft of a kidney from a cadaver. Results of a preoperative serologic Ig G tests for EBV and CMV were positive. She was given triple-drug immunosuppressive therapy, consisting of cyclosporine,azathioprine, and steroids. Seven years later, azathioprine was changed to mycophenolate mofetil. One year later, she was admitted to the hospital with a three to four week history of vertigo (which did not improve after sulpiride was administrated) and an influenza-like syndrome. A CT scan of the brain appeared normal, so paroxysmal positional vertigo was the diagnosis. Two weeks after admission to the hospital, the patient reported visual hallucinations and impairment of consciousness. Results of laboratory tests were leukocyte increase (polymorphonuclear leukocytes), anemia, hyponatremia and renal failure. Chest radiography, brain CT, and electroencephalography revealed no pathologic signs. The CSF examination revealed 300 cells/ml (79% PMNL), glucose 63 mg/dl, protein 45 mg/dl. Six hours later the treatment was initiated with ampicillin, ceftriaxone and ganciclovir iv, she experienced seizures that affected the left side of her body, but without interictal recovery. The patient required intubation and mechanical ventilation in the intensive care unit. An MRI of the brain images, revealed high signal-intensity regions indicating lesions on the bulb, protuberance, mesencephalon, left thalamus and parenchyma adjacent to the corpus callosum (fig. 1). Six days later, the patient partially recovered consciousness, and she had not neurologic sequelae. Intubation was terminated. As soon as PCR revealed EBV DNA in CSF samples, the treatment with ceftriaxone and ampicillin was discontinued. Treatment with ganciclovir was maintained for 8 weeks (4 weeks with iv and another 4 weeks with oral treatment). On day 35, the examination of a specimen of CSF revealed: glucose 46, protein 78, 15 cells/ml (100% lymphocytes). The patient went home on day 55 after admission to our hospital. She regained her normal neurologic function. Three weeks later MRI, showed reduction of the size of the lesions and the lesions on the brain stem had disappeared.

Topics: Adrenal Cortex Hormones; Antiviral Agents; Cyclosporine; DNA, Viral; Encephalitis, Viral; Epstein-Barr Virus Infections; Female; Ganciclovir; Hallucinations; Herpesvirus 4, Human; Humans; Immunocompromised Host; Immunosuppressive Agents; Kidney Transplantation; Middle Aged; Mycophenolic Acid; Postoperative Complications; Status Epilepticus; Vertigo