Unusual Presentation of Polyautoimmunity and Renal Tubular Acidosis in an Adolescent With Hashimoto's Thyroiditis and Central Pontine Myelinolysis.

Background: Hashimoto's thyroiditis is frequently associated with other autoimmune diseases and may include renal involvement. Case description: A 17-year-old female with previously diagnosed Hashimoto's thyroiditis and vitiligo was admitted to a pediatric intensive care unit with hypokalemic paralysis and acidosis, after having suffered from recurrent muscular weakness for approximately one year. A few days later she developed central pontine myelinolysis. After initial stabilization she was also diagnosed with distal renal tubular acidosis (dRTA) and tubular proteinuria which can occur in Sjögren's syndrome. Extended screening for autoimmune diseases additionally revealed celiac disease. Treatment with Prednisone and substitution of potassium quickly lead to the resolution of proteinuria and dRTA, but unilateral paralysis of the sixth nerve as a result of central pontine myelinolysis was irreversible. Conclusions: This is the rare case of polyautoimmunity including autoimmune thyroiditis, Sjögren's syndrome, vitiligo and celiac disease in an adolescent with few disease-specific symptoms. The diagnoses were made via a complicating nephritis causing dRTA and proteinuria. Delay in diagnosis lead to permanent neurological damage. This case highlights the need for pediatricians to be aware of rare accompanying diseases and their complications in "common" pediatric autoimmune diseases like Hashimoto's thyroiditis and celiac disease.

[Efficacy of intravenous immunoglobulins in central pontine myelinolysis]. / Eficacia de las inmunoglobulinas intravenosas en la mielinolisis central pontina.

INTRODUCTION: Central pontine myelinolysis (CPM) is a neurological condition with unknown pathogenesis. It is most often observed in patients suffering hydroelectrolyte disturbances. There is no specific treatment and it can have an unfortunate outcome. We present a case of CPM that responded well to immunoglobulins. CASE REPORT: The case of a 31 year old female patient with bulimia, impulsive type personality, potomania and chronic alcoholism is presented. After admission to the intensive care unit due to severe hyponatremia (104 mEq/l) with correction in 7 days, she had a rapidly progressive picture of dysarthria and gait instability, tetraparesis and pyramidalism. Due to suspected CPM, emergency magnetic resonance imaging was requested, observing a hypertense lesion in central, bilateral and symmetric T2 of the pons. Treatment was initiated with dexamethasone with clinical progression so that i.v. immunoglobulin treatment was prescribed, with a spectacular improvement after the third day. CONCLUSIONS: Treatment with immunoglobulins may be a therapeutic option to consider in patients with CPM and clinical progression after the usual treatment. Furthermore, this response opens questions on a possible dysimmune mechanism in this disease.

The subcellular localization of the carbohydrate epitope 3-fucosyl-N-acetyllactosamine is different in normal and reactive astrocytes.

The carbohydrate epitope 3-fucosyl-N-acetyllactosamine (CD15) is involved in cell-to-cell recognition processes in various tissues. In the present study the subcellular localization of CD15 was immunocytochemically studied in normal and pathological central nervous system fiber tracts of humans and rats. In normal human white matter of the brain, CD15 immunoreactivity was found on the cell surface of astrocytes and within the cytoplasm of oligodendrocytes. In freshly demyelinated lesions of two human diseases (central pontine myelinolysis and multiple sclerosis) strong cytoplasmic CD15 staining was observed in reactive astrocytes. In normal rats CD15 immunostaining was restricted to the surface of astrocytes. In crush-induced lesions of rat optic nerves, however, astrocytes showed a cytoplasmic localization of CD15, 4 and 6 days after injury. In conclusion, abnormal localization of CD15 in reactive astrocytes may be related to altered functional states of these cells during disease processes.