AMPA and NMDA receptor antibody autoimmune encephalitis preceded by ocular myasthenia gravis: a case report.

BACKGROUND: α-Amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) and N-methyl-D-aspartate (NMDA) receptors mediate excitatory neurotransmission in the brain and may be targeted by autoantibodies, leading to autoimmune synaptic encephalitis (AE). AE can be associated with other autoimmune diseases. However, the cooccurrence of anti-AMPA and NMDA receptor AE together with myasthenia gravis (MG) is unusual. CASE PRESENTATION: A 24-year-old previously healthy male presented with seronegative ocular MG, the diagnosis of which was supported by single-fiber electrophysiology findings. Three months later, he developed AE, initially being positive for AMPA receptor antibodies and subsequently for NMDA receptor antibodies. No underlying malignancy was found. In response to aggressive immunosuppressive treatment, he recovered (modified Rankin Scale (mRS) score change from 5 to 1). Despite some cognitive problems at the 1-year follow-up, which were not revealed using the mRS, he was able to return to his studies. CONCLUSIONS: AE may coexist with other autoimmune disorders. Patients with seronegative MG, including ocular MG, may develop autoimmune encephalitis with more than one cell-surface antibody.

[Subacute parkinsonism as first symptom of primary CNS lymphoma].

Secondary parkinsonism due to cerebral neoplasia is a rare condition. We present a case report of a 73-year-old woman with three weeks of progressing right-sided parkinsonism. Cerebral MRI arouse suspicion of left-sided primary CNS-lymphoma in the left basal ganglia. Cerebralspinal fluid analyzed with flow cytometry revealed diffuse B-cell lymphoma. The patient was transferred to the department of haematology and was treated by standard primary CNS-lymphoma protocol. Five-month clinical control revealed marked recovery. At nine-month control an MRI of cerebrum showed almost complete regression of tumour.

[Neurosarcoidosis].

Neurosarcoidosis (NS) affects 5-10% of sarcoidosis patients and can involve any part of the nervous system. The prognosis can be serious and relapse is common. The diagnostic work-up is difficult with many differential diagnoses in inflammatory, infective and malignant neurological diseases. There is no specific marker for NS. Recently proposed diagnostic criteria for NS emphasise that biopsy plays an important role. Infliximab appears to be effective when other treatments are inadequate. The review summarises the clinical features, imaging and laboratory findings, treatment and prognosis of NS.

Botulinum toxin treatment of spasticity targeted to muscle endplates: an international, randomised, evaluator-blinded study comparing two different botulinum toxin injection strategies for the treatment of upper limb spasticity.

OBJECTIVES: The therapeutic effects of botulinum neurotoxin (BoNT) are well documented in upper limb spasticity. However, several factors may influence treatment efficacy, including targeting of neuromuscular junctions (NMJs). We examined whether NMJ-targeted BoNT injections were non-inferior, in terms of efficacy, to current injection practices. DESIGN: Open-label prospective evaluator-blinded study. SETTING: Conducted across 20 medical centres in Denmark, Finland, Norway and Sweden (24 September 2012 to 11 March 2015). PARTICIPANTS: Aged ˃18 years with upper limb spasticity (Modified Ashworth Scale [MAS] score of 2 or 3) following stroke or traumatic brain injury, had received ≥2 consecutive BoNT-A treatment cycles (the latest of which was abobotulinumtoxinA [aboBoNT-A]) and needed BoNT-A retreatment (same modality as previous cycle). Patients requiring aboBoNT-A doses >800units were excluded. In total, 88 patients were randomised (intention-to-treat [ITT] population), most were male (n=58/88, 65.9%) and 54/88 (61.4%) completed the study (per protocol [PP] population). INTERVENTIONS: Randomisation (1:1) to receive a single dose of aboBoNT-A (≤800 U) according to either current clinical practice (300 U/mL) or as an NMJ-targeted injection (100 U/mL). PRIMARY OUTCOME MEASURE: Proportion of patients with a ≥1 level reduction from baseline in MAS score at week 4 post-injection (responders). RESULTS: In the ITT population, the proportion of responders at elbow flexors was 72.7% in the current practice group and 56.8% in the NMJ-targeted group (adjusted difference -0.1673 [95% CIs: -0.3630 to 0.0284]; p=0.0986). Similar results were observed in the PP population (69.0% vs 68.0%, respectively, adjusted difference 0.0707 [-0.1948 to 0.3362]; p=0.6052). CONCLUSIONS: Owing to the limited number of participants, non-inferiority of NMJ-targeted injections could not be determined. However, there was no statistical difference between groups. Larger studies are needed confirm whether the two techniques offer comparable efficacy. TRIAL REGISTRATION NUMBER: NCT01682148.

Involvement of distal sensory nerves in amyotrophic lateral sclerosis.

INTRODUCTION: The diagnostic criteria for amyotrophic lateral sclerosis (ALS) require normal sensory nerve conduction studies (NCS) or abnormal NCS only in the presence of neuropathy of identified etiology. In this study, we investigated the presence and extent of involvement of Aß sensory fibers in ALS. METHODS: Distal sensory NCS [antidromic dorsal sural (DS) and orthodromic medial plantar (MP)] and conventional sensory NCS (unilateral median sensory and bilateral sural nerves) were performed in 16 definite and 2 probable ALS patients (based on Awaji criteria) and 31 controls. RESULTS: Abnormal conventional sensory NCS were found in 8 (44.4%) ALS patients and 1 (3.2%) control subject (P = 0.002), whereas abnormal distal sensory NCS were found in 12 (66.7%) ALS patients and 3 (9.6%) controls (P < 0.0001). CONCLUSION: Distal sensory NCS were more often abnormal than conventional sensory NCS in ALS. Muscle Nerve 54: 1086-1092, 2016.