Patient and disease characteristics Reference No. of cases Sex/age AOSD characteristics Neurological findings Conclusion Reginato et al. 1987 [2] 3 (of authors’ 20/F Fevers, arthritis, sore throat, rash (1) Acute disorientation, hallucinations First two patients had improvement in    own patients)    during high fever (preceding liver failure)    neurological symptoms with resolution of 28/F Fevers, arthritis, sore throat, rash (2) Asterixis, mental status confusion,    liver failure while on high-dose steroids    increased intracranial pressure during Third patient had residual Bell's palsy    liver failure    after steroid therapy 25/M Fevers, arthritis, sore throat, rash (3) Lower motor neurone facial nerve Overall, only one patient had a true    palsy during acute flare    manifestation of a neurological    disorder with an acute flare of Still's    disease. The other two were complicated    by liver failure 6 (in review) Not available Unknown Transient pyramidal tract signs (French), Authors' review on neurological    third nerve palsy (French), Bell's palsy    manifestations of these six patients. Three    [18], status epilepticus [27], brain stem    non-English references, 1 reference    haemorrhage (German), symmetric    unavailable. Bell's palsy patient had    sensory neuropathy (English)    palsy since childhood; status epilepticus    patient with erosion of sella tursica    died with status epilepticus and    cardiovascular collapse (see below).    Overall not clear who had true    neurological manifestations Garrote et al. 1993 [14] 1 16/M Sore throat, myalgia, arthralgia, Transient diplopia, oscillopsia, horizontal Neurological symptoms resolved    headache and vomiting, rash,    nystagmus on left gaze, paraesthesias,    prior to non-steroidal therapy. The    fever and hepatosplenomegaly    dysaesthesias/on left side of face, unstable    authors hypothesize that the    present on admission    gait, weakness upper right extremity and    neurological manifestations may    clonus right ankle    be due to vascular involvement CT with hypodensity in left pons; MRI    of the CNS and may be focal and    with T2 hyperintensity on left middle    transient; however, it is not clear    cerebellar and cerebral peduncles    that it is associated with AOSD as    it resolved without therapy Denault et al. 1990 [15] 1 20/M Headache, fever, cough, Neck stiffness, confusion, decreased Evidence of meningoencephalitis:    myalgias and weight loss with    consciousness and incontinence. CSF    resolved without therapy raising    development of arthritis later in    pleocytosis noted. CT normal. Mentation    the issue of whether it was related    the course    slowly improved without therapy. Two    to Still's disease or a presentation    months later patient developed    of an initial viral illness    sensorimotor peripheral neuropathy The sensorimotor peripheral    documented by NCV and sural nerve    neuropathy resolved with steroid    biopsy    therapy Ohta et al. 1990 [4] 11 Age/Sex not Unknown in specific cases. All 5 with peripheral neuropathy; 4 with No discussion in paper of timing of    specified    patients had definite Still's fever,    aseptic meningoencephalitis; 1 with    diagnosis, i.e. acute flare of Still's    joint symptoms, rash,    delirium, convulsion, rigidity (1 person    or not, association with other    lymphadenopathy    had both peripheral and CNS involvement)    complications, etc. Pouchot et al. 1991 [1] 5 Age/Sex not Unknown in specific cases; 2 patients with disorientation and No details of timing of diagnosis,    specified    Patients had diagnosis of AOSD    confusion who developed hepatic failure;    resolution of symptoms or timing    based on Medsger and Christy    1 patient with peripheral facial nerve palsy;    with therapy; however, patients    criteria    2 patients with transient deafness (not due    with disorientation and confusion    to ASA); 1 patient with vertical diplopia    also had AOSD related hepatic    with ptosis due to 3rd nerve palsy    failure Ohta et al. 1987 [3] Review of 228 Unknown in specific cases 3 patients with meningoencephalopathy French; Ohta's own 2 patients    cases of AOSD 1 patient with brainstem haemorrhage See [16]    from the literature; 1 patient with status epilepticus See [27]    11 patients with 1 patient with pyramidal syndrome French paper    neurological 2 patients with CNS abnormalities See Baker et al. [17]    symptoms 2 patients with cranial nerve paresis French paper; see [18] 1 patient with orbital pseudotumour See [25] Wouters et al. 1985 [16] 1 33/M High fever, evanescent rash, Patient developed diplopia and ptosis of Paper not specific on activity of    polyarthritis    left eyelid after having a total hip    disease but presumably active with    replacement; CT with small haemorrhage    polyarthritis. Prednisone therapy    in brainstem. Patient presumably with    relieved polyarthritis. No mention of    active flare given significant polyarthritis    neurological complications/resolution Baker et al. 1979 [17] 2 Age/sex not Spiking fever, evanescent rash, Abstract mentions CNS manifestations in If 2 patients described with liver    specified    polyarthritis, leucocytosis    two patients. Unclear if two patients with    failure and mentation difficulties    acute liver failure who developed delirium    are the 2 with CNS manifestations,    and encephalopathy with increased ICP    this would not qualify as a direct    were those two patients    result of AOSD Kaplinsky et al. 1980 [18] 1 34/F Polyarthralgia, fever, rash Facial nerve paresis ‘that has been present No relationship between AOSD and    since childhood’    neurological complications Marie et al. 1999 [12] 1 17/M Headache, left 7th cranial nerve 7th cranial nerve palsy Treatment with high dose aspirin    palsy, sore throat, high fever, Meningeal symptoms with CSF showing    resulted in complete resolution of    arthralgias, myalgia,    lymphocytosis    symptoms. There appears to be a    lymphadenopathy and meningeal CT scan normal    true relationship between the    syndrome    AOSD and a neurological condition Markusse et al. 1988 [20] 1 26/M 9 year history of fevers, Gradual development of hearing loss and All studies pointed to cochlear    evanescent rash, arthralgias,    tinnitus on admission with fevers, arthritis,    involvement, not related to    lymphadenopathy, leucocytosis    rash, and weight loss. CT normal, CSF low    indomethacin, all occurring during    and liver enzyme abnormalities    cell count; indomethacin treatment    acute AOSD flare. This case    stopped—hearing loss remained. Improved    supports an association between    with prednisone; all other symptoms    neurological conditions and AOSD    improved Scully et al. 1989 [21] 1 59/F Patient initially had a facial Facial palsy preceded symptoms of AOSD Unclear if AOSD and neurological    palsy with resolution. 1 week    and resolved within 1 week    symptom were related. NEJM    later patient developed sore    discussant describes three other    throat, weakness and fevers    patients with lymphocytic    meningoencephalitis, transient    bilateral third cranial nerve palsy,    and unilateral peripheral facial    nerve palsy of 3 months duration.    Timing of diagnosis, resolution of    symptoms, etc. is not further    described Cush et al. 1985 [22] 1 21/M Fever, chills, sweats, sore throat, Patient developed ptosis of the right eye All symptoms resolved with    myalgia and anorexia,    with diplopia and orbital pain on lateral    anti-inflammatory therapy. Patient    leucocytosis    gaze. CT normal. Patient developed ptosis    was believed to have developed    in the left eye along with fever and    an acute inflammatory orbital    confluent rash    pseudo-tumour Kurabayashi et al. 1 75/F High fevers, salmon-pink Left sided hemiparesis and disturbance of Given patient's age it is difficult to    1996 [23]    eruption, lymphadenopathy,    consciousness    know if haemorrhage was    splenomegaly, high ferritin, CT with cerebral haemorrhage in region    secondary to age or to AOSD    negative ANA    of right thalamus to putamen Mok et al. 1998 [24] 0 Sixteen Chinese patients No neurological abnormalities    identified as having AOSD by    were seen in a Chinese patient    Medsger and Christy criteria    panel Cush et al. 1987 [25] 2 Age/sex not Total of 21 patients identified 2 patients described having peripheral No further comment on onset or    specified    with AOSD—fever, rash,    neuropathy and ulnar neuropathy    resolution with therapy    arthralgia, etc. Goldman et al. 1980 [27] 13: 1 noted with 8/M All with spiking fever, 1 woman described to have erosion of the No clear association with AOSD    neurological 5/F    polyarthrocytes, leucocytoses    sella tursica who died with status    and status epilepticus    symptoms    and negative RF    epilepticus followed by cardiovascular    collapse (no autopsy)