Autoimmune encephalitis associated with antibodies (Abs) directed against the synaptic ligand-gated ion channel NMDA receptor (NMDAR) was first described as a paraneoplastic disorder in association with ovarian teratoma. Other forms of neoplasia have subsequently been reported although many patients do not have a tumour. Tumour removal, where applicable, and immunotherapy form the mainstays of treatment. We present a patient who developed NMDAR-Ab encephalitis despite being chronically immunosuppressed following organ transplantation, and who was eventually found to have an occult malignancy in the form of non-Hodgkin's lymphoma.

Objective To report a case of NMDAR encephalitis, possibly related to B Cell lymphoma despite being immunosuppressed post renal transplant. Results A 54-year-old woman presented with a three month history of a sub-acute brainstem syndrome with altered personality and auditory hallucinations. She was on long-term immunosuppression with mycophenolate/prednisone following renal transplantation. MRI showed brainstem lesions, and CSF had lymphocytic pleocytosis without cancer cells. CT of the chest, abdomen, and pelvis was also normal. Despite empirical corticosteroid treatment the patient’s conscious level deteriorated and she developed orofacial dyskinesia with autonomic fluctuation. An autoimmune encephalitis was strongly suspected. Indeed both serum and CSF NMDAR antibodies were positive. Intravenous immunoglobulin and cyclophosphamide were commenced but treatment was complicated by peritonitis secondary to caecal perforation. Shortly thereafter the patient died of sepsis. Surpisingly histology from the resected colonic segment demonstrated high grade non-Hodgkin B-cell lymphoma. Discussion Neoplasia due to reduced immune vigilance is a known complication. However development of autoimmune disorders is unexpected as immunosuppression is the treatment for many autoimmune disorders including encephalitis. To have both complications together was exceptional. Possible mechanisms and implications will be discussed. Sometimes Ockham’s razor simply doesn’t cut.

Objective Every neurologist has encountered patients who present a (long) written list of symptoms: la maladie du petit papier. The frequency and diagnostic utility of this sign was examined. Results Over 6-months (April–September 2015), la maladie du petit papier was observed in 17/508 (3.35%) consecutive new patient referrals (16 handwritten examples, 1 ipad). Referrals to general (9/339=2.65%) and cognitive disorders clinics (8/169=4.73%) showed no significant frequency difference (chi-square=1.07, p>0.1). Final diagnoses in the general clinic were chronic daily headache (3), multiple symptoms with no neurological explanation (5) and unexplained blackouts (1). In the cognitive clinic, diagnoses were subjective memory complaint (5), mild cognitive dysfunction secondary to either alcohol misuse (1) or mild traumatic brain injury (1), and depression (1); none had dementia. La maladie du petit papier had high specificity (0.94) but low sensitivity (0.03) for cognitive impairment. Conclusions La maladie du petit papier is an infrequent sign in both general and cognitive disorders clinics. In general clinics it was associated with chronic and intractable disorders (headache, multiple symptoms). In cognitive clinics it was associated with subjective memory complaint; like the “attended alone” sign, it may support a diagnosis of cognitive normality.

Background Prompt assessment for respiratory muscle weakness in patients with acute neuromuscular weakness is recommended by national guidelines. Failure to recognize this leads to increased morbidity and mortality. We set out to quantify the problem and suggest ways for improving care. Aim To examine adherence to national guidelines for the identification of respiratory muscle weakness in patients with acute neuromuscular disease. Method Retrospective analysis of anonymised electronic case notes for patients admitted to a district general hospital within the period 1st January 2015 to 1st January 2016. Patients were identified by coding criteria; diagnosis Guillain-Barré syndrome, Myasthenia gravis, Lambert-Eaton syndrome, or having received intravenous immunoglobulin. Results Forced vital capacity (FVC) was requested at diagnosis in 38%. FVC was performed after the request in 45%. FVC was requested at any point during admission in 82%. The longest duration between request and completion of FVC was 5 days. Conclusions There are significant delays in recognizing the need for FVC measurement, and in some cases is missed entirely. When identified, measurement does not take place in more than half the population, and there are serious delays. The potential for harm is great, and in two cases led to ITU admission.

A 67-year-old woman presented with a seven-month history of progressive psychiatric disturbance and sub-acute cognitive decline mimicking Creuztfeldt-Jakob Disease.She presented initially to Psychiatry with worsening anxiety, depression, lassitude, impaired concentration and insomnia. She was given a diagnosis of agitated depression.She subsequently experienced episodes of disorientation, indecision and inability to complete routine, learned tasks as well as visual hallucinations. Initial investigations for causes of subacute dementia were unremarkable including a non-diagnostic MRI. However, five months into her presentation, there was further rapid deterioration with fluctuating consciousness and deteriorating mobility. She became bedbound, incontinent and cortically blind. She had right hemiparesis, hemineglect, extrapyramidal features, and extensor plantars.A 4D computerised tomography angiogram demonstrated a dural arteriovenous (AV) fistula with retrograde filling of superior sagittal sinus and cortical venous congestion. She underwent emergency Onyx embolisation. Over a few weeks, there was gradual improvement in her anxiety, cognition and mobility. She had a mild residual right hemiparesis.Intracranial dural AV fistulae can present with a spectrum of neurological symptoms, including cognitive decline. A lowhigh index of suspicion combined with close liaison between Neuroradiology, Neurology and Neurosurgery ensures prompt diagnosis and maximises the potential for cognitive recovery.