ABSTRACT
INTRODUCTION: Differing cranial nerve involvement has been reported in the context of Gougerot-Sjögren's syndrome. Involvement of the V, III and VII nerves has been reported, the most characteristic being nerve V, notably its lower branch. Rare, well documented, cases of facial palsy have also been described. OBSERVATION: Recurrent facial palsy in a 40 year-old woman revealed a primary Sjögren's syndrome and vitamin B12 deficiency. DISCUSSION: The onset of facial palsy has been linked with Gougerot-Sjögren's syndrome. The contribution of vitamin B12 deficiency is discussed.
Subject(s)
Facial Paralysis/etiology , Sjogren's Syndrome/complications , Adult , Anti-Inflammatory Agents/therapeutic use , Antirheumatic Agents/therapeutic use , Electromyography , Facial Paralysis/rehabilitation , Female , Humans , Hydroxychloroquine/therapeutic use , Immunosuppressive Agents/therapeutic use , Magnetic Resonance Imaging , Physical Therapy Modalities , Recurrence , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/drug therapy , Thyroiditis, Autoimmune/complications , Treatment Outcome , Vitamin B 12/therapeutic use , Vitamin B 12 Deficiency/complications , Vitamin B 12 Deficiency/diagnosis , Vitamin B 12 Deficiency/drug therapyABSTRACT
The history of diabetes mellitus is punctuated from the Egyptian Antiquity by sometimes amazingly acute clinical observations and inversely several missed opportunities on the way of the discovery of the mechanisms of the disease and of the development of its therapies.Alsace, and more generally the Rhine countries, have played a major role in this adventure, of which a crucial step has been the experimental demonstration of the role of the pancrease in the pathogenis of the disease by Oskar Minkowski and Joseph von Mering in Strasbourg in 1889.
Subject(s)
Biographies as Topic , Diabetes Mellitus/history , France , Germany , History, 15th Century , History, 16th Century , History, 17th Century , History, 18th Century , History, 19th Century , History, 20th Century , SwitzerlandABSTRACT
BACKGROUND/AIMS: Adult Still's disease is one of the febrile disorders of unknown etiology, characterized by high fever, transient cutaneous rash and leukocytosis. Liver dysfunction in adult Still's disease has been described in some case reports. The objective of this study was to analyze the pattern and the frequency of liver abnormalities in a monocenter series of adult Still's disease patients. METHODOLOGY: Data of 17 patients with adult Still's disease (fulfilling Yamaguchi's diagnostic criteria) were retrospectively reviewed. These patients were followed in an Internal Medicine Department over a period of 7 years. RESULTS: The median age was 27 years with a sex ratio M/F of 1.4. Fever was present in 100% of the cases and hepatomegaly occurred in 47% of the cases. Abnormalities in liver biochemistry, apparent in 76% of the subjects were characterized from moderate (elevation of transaminases between 2 and 5 N) (65%) to severe cytolysis (level of transaminases > 5 N) (12%), cholestasis (elevation of gamma GT and/or PAL) (65%), and increase in the level of LDH (35%). All of these symptoms disappeared either spontaneously or under treatment (83%), within a median period of 18 days. CONCLUSIONS: This study confirms the high frequency of liver dysfunction in adult Still's disease patients. Although it is moderate and asymptomatic in most cases, severe cytolytic hepatitis has been described. This study especially puts forward the need for exploring the possibility of adult Still's disease in the presence of a fever and hepatic cytolysis.
Subject(s)
Liver Diseases/etiology , Still's Disease, Adult-Onset/complications , Adult , Female , Humans , Liver Diseases/diagnosis , Liver Diseases/therapy , Male , Retrospective Studies , Severity of Illness Index , Still's Disease, Adult-Onset/diagnosis , Still's Disease, Adult-Onset/therapyABSTRACT
BACKGROUND: The present study reports a monocentric experience of 90 drug-induced agranulocytosis cases and discusses their management, in particular the role of hematopoietic growth factors. METHODS: Data from 90 patients with drug-induced agranulocytosis who met the criteria of the IAAAS group and of Bénichou and Solal-Celigny [Nouv Rev Fr Hematol 1993; 33: 257.] were retrospectively reviewed. All cases were extracted from a cohort study of the Hopitaux Universitaires de Strasbourg, France. Data were specifically analyzed with regard to the use of hematopoietic growth factors (in 42 patients). RESULTS: Mean patient age was 63 (range 17-95) years and the sex ratio (M/F) was 0.39. An underlying disease was present in 37% of the patients. Antibiotics (25%), antithyroid drugs (23%), and antiaggregative platelet agents (16%) were the most frequent causative drugs. Main clinical features included isolated fever (41%), septicemia or septic shock (31%), and pneumonia (10%). Mean neutrophil count was 0.13 (range 0-0.46)x10(9)/l. Outcome was favorable in 98% of patients. The mean durations of hematological recovery (neutrophil count over 1.5x10(9)/l), antibiotic therapy, and hospitalization was 8.5 (range 2-21) days, 9.2 (range 2-21) days, and 10.5 (range 3-23) days, respectively. All patients were treated with broad-spectrum antibiotics and 42 patients with hematopoietic growth factors. In these 42 patients, the mean durations for hematological recovery, antibiotic therapy, and hospitalization were significantly reduced at: 6.3 (range 2-16) days, 7.1 (range 2-16) days, and 9.1 (range 3-23) days, respectively (all P<0.05). CONCLUSIONS: The present study shows that new causative drugs are emerging (antibiotics, antithyroid, and antiaggregative platelet agents), that drug-induced agranulocytosis remains typically a serious accident with severe sepsis, and that modern management with broad spectrum antibiotics and hematopoietic growth factors may reduce the mortality.
