Sirs,Limbic encephalitis (LE) associated with other autoimmune disorders is relatively rare. There is an evidence of LE with relapsing polychondritis [1,2] or Sjogren's syndrome [3], and also with myasthenia gravis [6]. We present a case of LE with an unusual association of different autoimmune conditions.A 73-year-old man developed headache and fever over 2 weeks and a transitory loss of consciousness. His wife reported episodes of confusion over the previous 2 months.He had a medical history of seropositive myasthenia gravis in 1996 requiring artificial ventilation, plasmapheresis, immunotherapy and thymectomy. Removed thymus showed atrophy without histopathological finding of thymoma or thymic hyperplasia. After 2 years, the patient was asymptomatic and decided to stop all treatment and follow up. His medical history also included acute myocarditis in 1972, asthma, gastric ulcer. He was followed up for bladder carcinoma without signs of activity since the operation in 1994 and recent biopsy revealed prostatic microcarcinoma.On admission, he was disoriented in place and person and was confabulating. He had painful erythema of his right auricle, which he had experienced in the past. Neurological examination was unremarkable except for slight gait instability.Laboratory results showed mild inflammatory changes (hyperleucocytosis, higher erythrocyte sedimentation rate 20 mm/h, and raised C reactive protein 20.3 mg/l). Thyroid function was normal. Serum auto-antibodies ANA, ANCA, GBMi and dsDNA were negative; only anti-striatal muscle antibodies were slightly positive. Cerebrospinal fluid analysis showed mild protein-cytological association (hyperproteinorachia 0.657 g/l and 89 mononuclear cells). Serology for HIV, Borrelia, HSV1,2, CMV, EBV, VZV and neurosyphilis was negative. Onconeural antibodies anti-Yo, Hu, Ri, anti-VGKC antibodies, anti-GAD, antiamphiphysin and anti-NMDA receptor antibodies were negative in serum and cerebrospinal fluid.MRI showed FLAIR and T2 hippocampal temporal hyperintensities, vascular white matter lesions and moderate atrophy (Fig. 1). EEG repeatedly showed slow waves in the temporal regions. Whole body PET scan was negative.Progressively, myoclonic jerks, cerebellar signs, aphasia and hallucinations appeared with worsening of cognitive impairment. Bilateral auricle swelling occurred with softening and loss of cartilage contour. Biopsy revealed nonspecific cartilage inflammation without signs of an acute vasculitis (Fig. 2b), compatible with criteria for relapsing chondritis. Parenteral bolus of methylprednisolone 1,500 mg over 3 days led to partial and transitory improvement, therefore a month later bolus of 3,500 mg was repeated followed by permanent treatment with oral methylprednisolone 24 mg daily. Painful swelling extended to elbows and knees, and partial complex seizures with orofacial automatisms developed. A third bolus of 1,500 mg methylprednisolone was administered and four cycles of plasmapheresis were performed with only limited effect, moreover, massive eruptions of herpes...

Frontotemporal lobar degeneration with transactive response DNA-binding protein 43 (FTLD-TDP) and progressive supranuclear palsy (PSP) are distinct neurodegenerations with different clinical presentations. We report two cases with FTLD-TDP and PSP in comorbidity: a patient with amnestic dementia developing frontal lobe dementia, Parkinsonism and supranuclear gaze palsy and a patient with cerebellar ataxia and nystagmus developing akinesia, rigidity, and subcortical dementia. Neuropathological examination revealed neuronal and glial tau pathology together with ubiquitin, and phospho-TDP-43-immunoreactivities in the hippocampus, striatum, mesencephalon, and frontal and temporal cortices. Clinical and neuropathological correlations in atypical neurodegenerations are crucial to describe new entities of overlapping syndromes.

No abstract