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Occult neoplasms in facial paralysis.

A review of 14 cases of occult neoplasms associated with facial paralysis indicated that when the onset was gradual over a period of weeks or months, when all of the scientific and technological investigations, including CT scanning, polytomes, neurosensory and otologic tests, sialography, lacrimation, taste, internal acoustic meatus, evaluation of the deep lobe of the parotid, and clinical examination of the parotid and neck were negative, the tumor was almost always found in association with the main trunk of the extratemporal portion of the parotid gland. Of these neoplasms, 80% were malignant tumors of the parotid gland and 15% were neurilemmomas of the main trunk of the facial nerve. The above situation is an indication for human assessment by surgical investigation of the facial nerve, with the first priority being given to the main trunk of the extratemporal portion.

Adult↗

The use of sialodochoplasty in the treatment of benign inflammatory obstructive submandibular gland disease.

One of the most common causes of submandibular gland enlargement is benign inflammatory disease. The usual cause is ductal outflow obstruction due to either a calculus or stenosis allowing stasis and retrograde movement of the saliva into the acinar structures leading to an inflammatory response and gland enlargement. Faced with a submandibular mass, a work-up that will rule out neoplastic causes of submandibular enlargement is instituted. This includes clinical assessment, probing of the gland, radiocontrast sialography, and CT scanning. The treatment should reflect the obstructive nature of the disease. Plastic reconstruction of the duct allows the removal of calculi, shortening of the duct, and enlargement of the outflow opening preventing recurrence and allowing healing of the gland. The procedure is performed intraorally as an outpatient, does not disrupt oral functioning, or subject the patient to the risks of gland removal or loss of that organ's function. Our experience with 27 patients over a 7-year period is presented with a detailed description of the technique and an analysis of the results. The procedure was successful in 22 of the 27 patients.

Evaluation Studies as Topic↗

An asymptomatic radiolucent lesion in the mandible.

The lingual cortical mandibular defect is easily diagnosed radiographically. The key features include: a well-defined radiolucent area, location below the mandibular canal, and placement anterior to the angle of the mandible. Routine surgical exploration is not indicated, but sialography or radiographic follow-up may be useful.

Aged↗

[A case of Hashimoto's thyroiditis associated with renal tubular acidosis, Sjögren syndrome and empty sella syndrome].

This report describes a 48-year old female patient with Hashimoto's thyroiditis, distal-type renal tubular acidosis (d-RTA), Sjögren syndrome (SjS), and empty sella syndrome (ESS). She has been receiving replacement of thyroxine for Hashimoto's thyroiditis since 1967. She felt muscle weakness and numbness in the extremities and was found to have low serum potassium (2.9 mEq/l) in 1987. Since then she has been administrated potassium chloride orally. She was admitted to our hospital because of recurrence of muscle weakness and numbness of the extremities in November 1990. Laboratory examination revealed that her serum levels of antimicrosomal antibody and anti-thyroglobulin antibody were highly positive (MCHA: x 2(10) x 100, and TGHA: x 100). Furthermore, she was revealed to have 1) d-RTA by oral tolerance tests with the administration of NH4Cl and NaHCO3, 2) SjS by Schirmer test and sialography, and 3) ESS by computed tomography and magnetic resonance imaging examinations of the pituitary. Association of Hashimoto's thyroiditis, d-RTA, SjS and ESS in this case may possibly be caused by common autoimmune mechanism.

Acidosis, Renal Tubular↗

A case of Sjögren's syndrome with valvular diseases.

The case of a 61 year old woman with Sjögren's syndrome with aortic and mitral stenosis is reported. She suffered from rheumatic fever at a young age. Physical and echocardiographic examinations showed findings of mitral and aortic valve stenosis. In addition, she had experienced xerostomia, a gritty sensation in the eyes and Raynaud's phenomenon. Blood examination showed hypergammaglobulinemia, positive rheumatoid factor, antinuclear and anti-Ro (SS-A) antibodies. The diagnosis of Sjögren's syndrome was confirmed by sialography and biopsy of the labial salivary gland. The combination of valvular disease and Sjögren's syndrome is rare and the etiological correlation is discussed.

Antibodies, Antinuclear↗

Pulmonary MALT lymphoma with amyloid production in a patient with primary Sjögren's syndrome.

A 53-year-old woman was admitted to our hospital complaining of cough, low grade fever, chest pain and sicca symptoms. A chest radiograph showed an abnormal shadow and chest computed tomography revealed a tumor in left S6. She was diagnosed as Sjögren's syndrome by sialography and histological findings of labial biopsy. The surgically resected tumor specimen showed proliferation of lymphoid cells with lymphoepithelial lesions, which were positive for CD20 and kappa light chain. Kappa light chain-positive amyloid was found within the tumor. The tumor showed rearranged kappa light chain genes. The diagnosis was pulmonary mucosa associated lymphoid tissue lymphoma with amyloid production.

Amyloid↗

A case of sicca syndrome due to primary amyloidosis.

We report the rare occurrence of sicca syndrome associated with primary amyloidosis. A 63-year-old man with apparent keratoconjunctivitis sicca and xerostomia died of the cardiac failure. Neither sialography nor labial gland biopsy revealed findings compatible with Sjögren's syndrome. Macroglobulinemia and a positive Bence-Jones protein were noted in urine testing. Amyloid deposition was demonstrated by Congo red staining on biopsied rectal tissues, and by typical birefringence under polarized light microscopy. At autopsy, the amyloid deposition was identified histochemically in the submandibular gland and the minor salivary glands of oral cavity.

Amyloidosis↗

Association of mixed connective tissue disease, Sjögren's syndrome and autoimmune hepatitis: report of a case.

A 51-year-old female was admitted to the hospital due to liver dysfunction. She had suffered from arthralgia, Raynaud's phenomenon, dry mouth and dry eyes. Laboratory data showed positive LE cells and anti-RNP. The diagnosis of mixed connective tissue disease (MCTD) was made according to the criteria of the international symposium on MCTD and Antinuclear Antibodies (August 1986, Tokyo). Liver biopsy showed chronic active hepatitis. The results of sialography, lip biopsy and rose-bengal test were compatible with Sjögren's syndrome (SjS). This is the report of a rare case with MCTD associated with SjS and autoimmune hepatitis.

Antibodies, Antinuclear↗

A case of maternal subclinical Sjögren's syndrome associated with congenital heart block.

A case of subclinical Sjögren's syndrome was diagnosed when the patient delivered a baby with SSA antibody-positive congenital heart block (CHB). The mother had been asymptomatic throughout her life. The laboratory findings including sialography, lip biopsy, Schirmer's test and a Rose-Bengal test showed Sjögren's syndrome. Such an asymptomatic case is termed subclinical Sjögren's syndrome. Thus, it was important to investigate an asymptomatic mother who delivers a SSA antibody-positive CHB baby.

Acidosis, Renal Tubular↗

Primary amyloidosis with dry eyes and dry mouth--a case report.

We report a rare case of dry eyes and dry mouth caused by primary amyloidosis. A 66-year-old woman with keratoconjunctivitis sicca and xerostomia died of acute respiratory failure. Shirmer's test, gum test, and sialography indicated Sjögren's syndrome. Lip biopsy revealed amyloid deposition around the salivary ducts. Bence-Jones protein was noted in the urine. At autopsy, amyloid deposition was identified histochemically in many organs, mainly on the vessel walls. Primary amyloidosis should be considered as a differential diagnosis of Sjögren's syndrome.

Aged↗

[A case of Sjögren's syndrome with rheumatoid arthritis manifesting transverse myelitis with antineuronal antibody].

We report a sixty-year-old woman with transverse myelitis who had suffered from rheumatoid arthritis since age of 52. She was admitted to our department because of muscle weakness and painful tonic spasm in the bilateral lower extremities, sensory disturbance below the mamillary level and bladder disturbance. She had sicca symptoms. As a result of sialography, Sjögren's syndrome was diagnosed. Antineuronal antibody was found in the sera of the patient. She had no symptom of systemic vasculitis. Lupus anticoagulant and anticaldiolipin antibody were negative. The pathogenesis of transverse myelitis in rheumatic disease is still uncertain. Vasculitis and the immunological reaction of antineuronal antibody have been suggested as possible causes. This report suggests the influence of direct immunological reaction on the central nervous system.

Arthritis, Rheumatoid↗

[A case of Sjögren's syndrome associated with inflammatory pseudotumor of the liver].

A 71-year-old man had noticed a dry sensation in the mouth with swelling of bilateral parotid glands in 1988. He was given a diagnosis of Sjögren's syndrome (SS) on the basis of characteristic findings of sialography and a minor salivary gland biopsy. He was admitted to our department in Febuary 1995 because of general fatigue of 2 month's duration. Laboratory data showed both positive anti-SSA and anti-SSB antibodies, liver dysfunction, hypoalbuminemia, and thrombocytopenia. Abdominal CT and MRI demonstrated a 2-cm intrahepatic mass (S 6) with enhanced areas at the periphery. The liver biopsy yielded fragments from the intrahepatic mass and hepatic parenchyma. The former was composed of plasma cells, lymphocytes, and histiocytes, compatible with the diagnosis of inflammatory pseudotumor of the liver. The pathological diagnosis of the latter specimen was primary biliary cirrhosis, although antimitochondrial antibody was negative. The intrahepatic mass gradually decreased in size without treatment. Inflammatory pseudotumor is considered to be a benign inflammatory condition simulating a neoplasma and the possibility of an autoimmune reaction is suggested on the basis of etiology. This is the first report of an inflammatory pseudotumor associated with Sjögren's syndrome developing in the liver. The inflammatory pseudotumor should be considered as a possible diagnosis in cases where the tumor is embedded in the liver.

Aged↗

[Peripheral neuropathy and dysautonomia in a patient with primary Sjögren's syndrome].

A 46 year-old female presented with dry eyes and a dry mouth which she had been experiencing for about 15 years. She also began to notice dizziness (orthostatic hypotension) during the last 5 years. The symptoms gradually increased whereupon she began to have polyarthralgia, facial flashing, hyper and hyposweating in some areas on the face and trunk. Her sialography showed a damaged parotid gland. Minor salivary gland biopsy revealed chronic sialoadenitis. The Sirmer test was low, and the Rosebengal test indicated keratoconjunctivitis sicca. Her serological tests showed hypocomplementemia and were positive for antinuclear antibody and SS-A antibody. The diagnosis of primary Sjögren's syndrome (SjS) was made based on these findings. Prednisolone, at a dose of 15 mg per day, was given orally. As a result of this therapy, arthralgia disappeared immediately, although it had no effect on the neuropathy found in this patient. This is a rare case of SjS associated with peripheral neuropathy and severe dysautonomia.

Autonomic Nervous System Diseases↗

[Report of a patient of primary Sjögren syndrome, IgA nephropathy and chronic idiopathic thrombocytopenic purpura].

We describe the case of a 61-year-old woman diagnosed with primary Sjögren's syndrome (SS) after an 8-year history of IgA nephropathy and a 3-year history of recurrent purpuric rashes. Her two daughters had previously been diagnosed with other autoimmune diseases. One daughter had Graves' disease and the other had Hashimoto's disease and systemic lupus erythematosus. The diagnosis of SS was made based on dryness of mucous membranes, Shirmer test, and parotid sialography. Thrombocytopenia, high platelet-aggregated IgG (PA-IgG) level, and normal megakaryocytes count in bone marrow suggested that her recurrent purpuric rashes were due to idiopathic thrombocytopenic purpura (ITP). Patients with SS may develop other autoimmune diseases. This case aids understanding of the immune pathogenesis and genetic background of SS.

Chronic Disease↗

[Clinical significance of cevimeline hydrochloride in the treatment of dry mouth in patients with Sjögren's syndrome].

To evaluate the efficacy and safety of cevimeline hydrochloride for the treatment of dry mouth in patients with Sjögren's syndrome (SS), eight SS patients received 30 mg of cevimeline twice or three times daily for 24 weeks. Six out of the eight patients had improvement in dry mouth. Five patients had more than 20% increase in saliva secretion. In the assessment of salivary gland scintigraphy, three patients showed improvement. There was a significant negative correlation between the improvement of saliva secretion and the severity of tissue damage assessed by MR sialography (r= - 0.754, p<0.05). One patient stopped cevimeline at 4 weeks because of headache and nausea. There was no significant change in laboratory data. Cevimeline is safe and effective medicine for dry mouth in patients with SS, in particular, with less severe salivary gland destruction.

Female↗

[Analysis of clinical questionnaire of Tokyo Metropolitan Government for Sjogren's syndrome].

Sjögren's syndrome is not one of the forty-five diseases included in the incurable disease treatment research project funded by the government. However, it is approved as a designated incurable disease for government funded treatment in Tokyo. The criteria of approval are based on diagnostic criteria revised in 1999. Complications and other factors are taken into consideration in the recognition standard established independently in Tokyo. In this study we examined the recognition standard of approval and their significance by analyzing the clinical questionnaires of patients with Sjögren's syndrome in Tokyo through 2003. The major objective of the recognition standard (2003) appears to have been the approval of subjects with severe Sjögren's syndrome with such conditions as advanced glandular involvement xerosis affecting their daily lives and visceral complications. However, examinations using biopsy, salivary gland scintigraphy, and contrast sialography can only be performed at an institution with appropriate facilities. For an approval process that is impartial and accomplished with ease, we believe that combinations of simple multiple tests should be considered as auxiliary items which could be substituted for the aforementioned examinations. In addition, future investigation is necessary to examine the high cost incurred in requiring findings within six months for an approval application, performing of annual invasive tests, and the validity of the process for determining the approved or unapproved status using the present clinical questionnaire profiles.

Humans↗

Sonography of the salivary glands.

In a blinded retrospective study of the efficacy of sonography of the salivary glands, we obtained sonograms on 849 patients. The patients were referred over the last 5 years from the ear, nose, and throat department of our university hospital. Eight-eight percent of the patients had symptoms related to the salivary glands. One hundred fifty-six of the patients (18%) were subsequently proved to be normal. The remaining 693 patients had diseases confirmed by histologic examination (332 patients), sialography (274 patients), or clinical follow-up (87 patients). Of these, 289 had salivary gland tumors (231 benign, 58 malignant), 185 had calculi, 154 had inflammation, 48 had autoimmune disease, 14 had cysts, and three had trauma. Two hundred eighty-seven (95%) of the 302 space-occupying lesions could be completely delineated by sonography, and the benign or malignant nature of the lesions was correctly assessed in 272 (90%). Of the 185 patients with calculi, the calculi were visualized and localized (intraductal vs intraglandular) on the sonograms in 174 (94%) patients. Abscesses were shown on the sonograms in 13 patients, and acute inflammation was shown on the sonograms in 84 patients. These results indicate that sonography is a useful procedure for diagnosis of diseases of the salivary glands.

Autoimmune Diseases↗

Texture analysis of sonographic features of the parotid gland in Sjögren's syndrome.

OBJECTIVE: The purpose of this study was to develop a method for quantitative analysis of the sonographic features of parotid glands as a noninvasive tool for the diagnosis of Sjögren's syndrome. SUBJECTS AND METHODS: Sonographic texture analyses were performed on the parotid glands of 44 patients with Sjögren's syndrome, 83 healthy volunteers, and 17 patients with chronic parotitis, using a fast Fourier transform program. RESULTS: Texture analysis of sonographic studies of the parotid gland using a Fourier transform showed that the sum of the normalized radial power spectrum in the low-spatial-frequency region (S value) of the parotid gland was significantly higher (p < .0001) in the patients with definite Sjögren's syndrome [6.70 +/- 2.13 (x10(5))] than in the 72 age-matched normal volunteers [3.25 +/- 1.08 (x10(5))]. However, patients with probable Sjögren's syndrome showed S values [3.92 +/- 1.88 (x10(5))] similar to those of the controls. On the other hand, SDs of the echo levels in the parotid gland showed significantly greater (p < .0001) values in patients with definite (4.63 1.07) and probable (4.53 1.47) Sjögren's syndrome than in the normal controls (3.30 0.76). Discriminant analysis showed that a combination of these two distinctive values increased diagnostic accuracy to 96.9%. Furthermore, S values and SDs correlated well with the qualitative grading of sonographic features and with the gradings of sialography. CONCLUSION: The system we describe for texture analysis of sonographic images is useful in the diagnosis of Sjögren's syndrome.

Adult↗