Cortico-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): about two case reports characterized by a gap between the diagnosis of autoimmune thyroiditis and neurological disorders

We report two cases of steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) often called "Hashimoto's encephalopathy" in which the neurological manifestations develop years before or after the Hashimoto's diagnosis. Because of this specific presentation, the etiological diagnosis of this type of encephalopathy can be a difficult task. In our patients there was a gap of 10 to 20 years between the proof of autoimmune thyroiditis and the neurological symptoms. Case reports of this type of presentation are rare in the literature. A dramatic responsiveness to steroids with total recovery, after several relapses, was confirmed 3 years after the end of treatment. We suggest that antithyroid antibodies should be checked in all patients with unexplained acute or subacute encephalopathy even in elderly subjects in whom the most important differential diagnosis with Creutzfeldt-Jacob disease remains rapidly progressive Alzheimer's disease. A brief review of the literature is proposed.     

Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience.

BACKGROUND & AIMS: The Japan Pancreas Society criteria for diagnosis of autoimmune pancreatitis (AIP) mandate presence of characteristic imaging (diffuse pancreatic enlargement with diffusely irregular, narrow pancreatic duct). AIP has unique histologic features associated with infiltration of tissues of affected organs with abundant IgG4-positive cells. We propose expanded diagnostic criteria for AIP with a cohort of histologically confirmed AIP. METHODS: We reviewed the pancreatic imaging findings on computed tomography scans, serum IgG4 levels, other organ involvement, and response to steroids in 29 consecutive patients who met histologic criteria for AIP. RESULTS: Computed tomography scans (n = 22) showed diffuse pancreatic enlargement in 6 (27%) patients; the rest had focal enlargement, distinct mass, normal pancreas, or focal acute pancreatitis. Serum IgG4 level was elevated in 15 of 21 (71%) patients, and other organ involvement (eg, intrahepatic biliary strictures) was noted in 11 of 29 (38%) patients. All 17 patients treated with steroids exhibited resolution/marked improvement of pancreatic/extrapancreatic manifestation. On the basis of this experience we propose that diagnosis of AIP can be made in patients with > or =1 of these criteria: (1) diagnostic histology, (2) characteristic imaging on computed tomography and pancreatography with elevated serum IgG4 level, or (3) response to steroid therapy of pancreatic/extrapancreatic manifestations of AIP. Twenty additional patients met expanded diagnostic criteria for AIP, and their demographic and clinical profile was similar to that of the 29 patients meeting histologic criteria. CONCLUSIONS: AIP defined by histological criteria shows a wide spectrum of radiologic features, with characteristic imaging seen only in a minority. Incorporation of additional features into current diagnostic criteria can identify the full spectrum of clinical presentations of AIP.     

Mixed connective tissue disease associated with autoimmune hepatitis and thyroiditis.

The case is reported of a 27 year old woman who had mixed connective tissue disease (MCTD) associated with chronic active hepatitis and thyroiditis. Although hepatomegaly is sometimes observed in MCTD, only four cases of MCTD and chronic active hepatitis have been described. It is thought that this is the first report of an association between MCTD, chronic active hepatitis and thyroiditis.     

Turner's syndrome associated with Hashimoto's thyroiditis and sarcoidosis: a case report.

A 32-yr-old Japanese female who was suffering from Turner's syndrome associated with Hashimoto's thyroiditis and sarcoidosis is reported. It is known that Hashimoto's thyroiditis is not infrequently complicated with Turner's syndrome on the basis of an abnormal X chromosome. In this case, sarcoidosis occurred 4 months after Hashimoto's thyroiditis. This extremely interesting coincidence of Hashimoto's thyroiditis and sarcoidosis in Turner's syndrome has not been reported to date to our knowledge.     

A case of rheumatoid arthritis associated with silent thyroiditis.

A 41-year-old female with rheumatoid arthritis had nontender enlarged thyroid gland. Thyroid function tests revealed increased concentrations of serum free T3 (FT3, 10.8 pmol/L) and free T4 (FT4, 31.1 pmol/L) with suppressed concentration of thyrotropin (TSH, lower than 0.1 mU/L) and low 24-hour thyroidal radioactive iodine uptake (1.6%). Serum thyrotropin receptor antibody (TRAb) was negative (0%) and she had positive anti-thyroglobulin and anti-microsomal antibodies. A diagnosis of silent thyroiditis was made based on laboratory findings. Serum concentrations of FT3 and FT4 normalized one month later without treatment. The causal relationship between the two diseases is discussed.     

Encephalopathy associated with Hashimoto's thyroiditis: an additional case

A 35-year-old female with a history of Hashimoto's thyroiditis (HT) presented with episodic dysphasia and a burst of axial jerks with abdominal muscle contraction and asymmetric blepharospasm as manifestations of encephalopathy associated with HT. Brain magnetic resonance imaging (MRI) showed scattered supratentorial white matter hyperintense T2 foci. Brain single photon emission computerized tomography (SPECT) demonstrated a cortical hypoperfusion in the upper frontal and parietal areas. Corticosteroid therapy induced a rapid improvement of the clinical picture.     

Pulmonary hypertension associated with autoimmune hemolytic anemia: a case report

Endovascular treatment of thoracic aortic pathology has become a valuable alternative therapeutic option to open surgery, however complications both during implantation and the postoperative period may occur. In this case report we present an intimal dehiscence during endovascular procedure. Diagnosis was made by transoesophageal echocardiography.     

Autoimmune hepatitis associated with thyroiditis and hypophysitis. A case report

Autoimmune hepatitis primarily affects women and 40% of cases are associated with extrahepatic autoimmune dysfunction. Thyroiditis, ulcerative colitis and rheumatoid arthritis are the most commonly implicated entities. We present a 46-year-old woman with type-II autoimmune hepatitis and Graves disease who presented deterioration in level of consciousness, her symptoms mimicking severe liver failure. Hormone studies and nuclear magnetic resonance imaging revealed hypophysitis, which led to hypothyroidism and metabolic encephalopathy. The syndrome was resolved with hormone replacement therapy.     

Painless thyroiditis associated with severe inflammatory reactions in amyloid goiter: a case report

We report the case of a 64-year-old woman with rheumatoid arthritis (RA) associated with high grade fever, malaise, and painless swelling of thyroid gland. Laboratory findings showed severe systemic inflammatory reactions, including increases in various cytokines such as IL-6. Gallium-67 citrate imaging revealed intense uptake in the painlessly enlarged thyroid gland. Histologically, biopsied specimens of thyroid showed diffuse amyloid infiltrations, which included amyloid A (AA) protein. Biopsies of rectum and stomach revealed similar amyloid depositions, indicating that the amyloid had a secondary origin, potentially due to RA. All clinical symptoms were relieved by intravenous pulsatile administration of methylprednisolone followed by oral prednisone, resulting in prolonged hypothyroid status. To our knowledge, this is the first case report in Japan describing painless thyroiditis with severe inflammatory reactions in amyloid goiter.