Thursday, February 26, 2009

Nonviolent elaborate behaviors in REM sleep behavior disorder

Neurology 2009; 72:551-557
Authors hypothesized that nonviolent behaviors may be underestimated. Behaviors are nonstereotyped and complex. They include talking, laughing, shouting, swearing, yelling, crying, gesturing, grabbing, arm flailing, slapping punching, kicking, jerking, strangulating, thrashing, sitting up, leaping from bed, crawling and running.

Unpleasant, action filled aspects include being pursued or attacked, authors add chewing, feeding drinking, urinating defecating or performing a sexual act (masturbating), coitus like thrusting, thumbs up, flying and others. Authors suggest a release of learned, engraved programs.

Saturday, February 21, 2009

lupus associated vasculopathy with MMN and livedo reticularis

NEJM Case records 2009 Case 5 2009 A 47 year old woman with a rash and numbness and pain in the legs. 360:711-720

Also see post on livedo reticularis and neurologic disease:

Case: 47 year old woman developed slowly progressive numbness then pain in LE's, swelling, mottled discoloration, swelling and blanching nodules. Rheum screen was normal. Biopsy of TA and nodule showed lymphistiocytic infiltrate but not vasculitis. family history positive for Sjogren's ( mother, with AI hepatitis) and "vasculitis" (sister). She atrophie blanche and cytopenias as well.

Teaching Points
1. MMN--with the other findings point to SLE, Sjogren's, APL syndrome, cutaneous PAN, or livedoid vasculopathy. Second tier ddx includes cryoglobulinemia, sarcoid, DM, amyloid, neoplasms and infections.

2. 50 % SLE patients have rash before meeting criteria for SLE. LRET and nodules is enough to count. Small vessel vasculitis characterized by palpable purpura is commonest, but medium size vasculitis with livedo reticularis also occurs. Patients with SLE and medium sized vasculitis have more MMN, visceral vasculitis, and ischemic cutaneous lesions, but less malar rash and discoid lesions than patients without medium sized vasculitis.

3. ANA negative SLE is rare since introduction of testing with HEp-2 cell substrate (10 %) and this patient initially had a FALSE negative ANA due to wrong method for test being performed by the commercial lab. Occassional patients who are ANA negative are anti Ro *SSA) positive or have secondary APL syndrome. Repeat testing showed positive 1:1280 ANA.

4. APL syndrome may be associated with thrombocytopenia and leuokopenia and be primary or secondary. Patient was initially negative, then positive.

5. Cutaneous PAN is challenging since there are no antibodies that are distinctive. Typically one sees elevation of acute phase reactants and anemia which is not the presentation here.

6. The patient's biopsy showed livedoid vasculopathy, she was treated with ASA and plaquenil with warfarin in reserve and she did well.