A 52-year-old woman known to have anti-synthetase syndrome (ASS) with positive anti-alanyl-tRNA synthetase antibody (anti-PL 12) for 4 years presented with headache and progressive deterioration of cognitive functions manifested predominantly by episodes of confusion and dyslexia. Clinical, laboratory and radiological evaluation as well as response to treatment was indicative of vasculitis of the central nervous system (CNS). CNS vasculitis is one of the rare manifestations of inflammatory myositis and no case has been reported to suggest CNS vasculitis in ASS.

/pdf/cns-vasculitis-in-anti-synthetase-syndrome-3x8gcj4bur.pdf

CNS Vasculitis in Anti-Synthetase Syndrome

Central nervous system (CNS) involvement in dermatomyositis (DM) is seldom observed. However, there are very rare case reports of CNS involvement with juvenile dermatomyositis. Encephalopathy in DM may occur for a number of reasons, such as cerebral vasculitis and hypoperfusion/hypertensive encephalopathy, but mostly as a consequence of immunosuppressant treatment. We report here for the first time the case of a patient with two rare diseases, namely anti-MDA5 antibody-positive dermatomyositis and mild encephalopathy with reversible splenial lesion (MERS).Die Beteiligung des zentralen Nervensystems (ZNS) an einer Dermatomyositis (DM) wird selten beobachtet, und nur sehr selten gibt es Fallberichte über die Beteiligung des zentralen Nervensystems bei juveniler DM. Eine Enzephalopathie bei DM kann aus einer Reihe von Gründen auftreten, z. B. bei zerebraler Vaskulitis, Hypoperfusion/hypertensiver Enzephalopathie, meist aber als Folge einer immunsuppressiven Behandlung. In der vorliegenden Arbeit wird erstmals über einen Fall berichtet, in dem bei einer Patientin zwei seltene Krankheiten vorkommen, und zwar eine Anti-MDA5-Antikörper-positive DM und eine leichtgradige Enzephalopathie mit reversibler Milzläsion („mild encephalopathy with reversible splenial lesion“, MERS). 

/pdf/anti-mda5-antibody-positive-dermatomyositis-with-mild-138w42aw.pdf

Anti-MDA5 antibody-positive dermatomyositis with mild encephalopathy with reversible splenial lesion: a possible rare association?

Objective: Presentation of four cases of juvenile dermatomyositis with CNS involvement as the initial feature. Background There are very few case reports in the literature of CNS involvement with JD. There are even fewer cases reported in which the initial presentation of CNS vasculitis and demyelinating disease develops into more recognizable JD. Design/Methods: Four patient cases are presented. Results: We present case reports of four patients who presented with CNS lesions and various degrees of encephalopathy who later progressed to meet clinical criteria for the diagnosis of juvenile dermatomyositis. The first patient we report is a 13 year old male who presented with demyelinating lesions of the spinal cord and optic nerves. He went on to develop weakness of the lower extremities. The second patient, a 6 year old female, initially presented with a viral meningitis, and then went on to develop a myriad of brain lesions on MRI. A third patient, a 9 year old female presented with seizures and encephalopathy. She had both cortical and subcortical brain lesions that showed both inflammation and hemorrhage. The final patient, a 16 year old girl presented with demyelinating lesions of the brain stem and spinal cord. All four of the patients went on to develop proximal muscle weakness, primarily of the lower extremities and had muscle biopsies demonstrating perifascisular atrophy and inflammatory changes consistent with juvenile dermatomyositis. All of these patients responded well to immunosuppressive therapy and made substantial recoveries. Conclusions: These reports should encourage investigation into autoimmune disease in general and JD in particular when cases of CNS disease of unknown etiology present in pediatric patients. There are implications for both prognosis and treatment when the disease course is recognized early. Disclosure: Dr. Lee has nothing to disclose. Dr. Bishop has nothing to disclose. Dr. Vedanarayanan has nothing to disclose.

CNS Disease as the Presenting Feature in Four Cases of Juvenile Dermatomyositis (P07.218)

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CNS Disease as the Presenting Feature in Four Cases of Juvenile Dermatomyositis (P07.218)