Background. The Sjögren Syndrome (SS) can include various manifestations of central nervous system impairment. Extrapyramidal signs are known to be very rare and unusually discovered on early onset in this pathology. Observation. A 46-year-old woman with a history of progressive Parkinsonism for 6 years and a normal brain magnetic resonance imaging was partially improved with levodopa therapy. The later discovery of a sicca syndrome led to performing of further investigations, which revealed the presence of anti-SSA antibodies and a sialoadenitis of grade 4 according to Chisholm's classification on labial salivary gland biopsy. The diagnosis of primary SS was established and the adjunction of corticotherapy has remarkably improved Parkinson's signs without use of other immunosuppressive agents. Conclusion. Based on these findings, we discuss the hypothesis of either a causal link between SS and Parkinsonism or a fortuitous association of two distinct pathologies with or without a shared immunopathogenesis.
Primary retroperitoneal cavernous hemangioma is an extremely rare disease in clinical practice. It is classified as a vascular tumor. Only three cases have been reported in the literature. The diagnosis is uncommon due to the lack of specific radiological features. It becomes symptomatic as a consequence of its enormous size or complications like rupture or compression. We herein report a unique case of primary retroperitoneal cavernous hemangioma treated with conventional surgery in a 35‐year‐old male patient admitted to our department for chronic abdominal pain. Retroperitoneal cavernous hemangioma is an extremely rare vascular tumor in adulthood. Confirmation is made by histopathological examination after total surgical resection.
Neurometabolic diseases (NMD) are a frequent cause of epilepsy in children. Epilepsy is more frequently part of a complex clinical picture than a predominant symptom and may be of different types and various EEG patterns. The primary goal of this article is, departing from a large personal series, to describe the seizure type, EEG patterns and response to antiepileptic drugs in NMD and to discuss clinical value of epilepsy type in the setting of specific NMD. We found epilepsy was associated to NMD in 43.1%. Disorders of energy metabolism were the most frequent cause of epilepsy (61.3%). We observed generalized epilepsy in 75% of the patients with partial epilepsy in 25%. EEG was abnormal in only 71% of cases with variable patterns. Resistance to antiepileptic drugs was observed in 75% of cases. Valproate acid was incriminated in seizure worsening in 22.7% of the patients, all of them affected by mitochondriopathies
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