Clinical characteristics and outcome of patients with autoimmune encephalitis: clues for paraneoplastic aetiology.

BACKGROUND AND PURPOSE: Autoimmune encephalitis (AE) represents a complex syndrome with diverse clinical manifestations and therapeutic outcomes. The aim of this study was to report the clinical characteristics and the long-term outcome of patients with paraneoplastic and idiopathic AE. METHODS: All patients with subacute encephalopathy admitted to the Neurology Department of our Institution from January 2012 to May 2019 were consecutively enrolled. Patients' serum and cerebrospinal fluid were tested for neural-specific autoantibodies by indirect immunofluorescence assays on mouse brain, rat neurons, cell-based assays and immunoblots. Outcome was assessed by the modified Rankin Scale score. RESULTS: From 107 adult patients with subacute encephalopathy, 50 patients were finally diagnosed with AE. Neural antibodies (Abs) were detected in 45/50 patients (90%). Leucine-rich glioma-inactivated protein 1 immunoglobulin G was the most frequent (6/50, 12%) Ab specific to neural surface antigens detected in adults with AE. Paraneoplastic encephalitis was diagnosed in 16/50 patients (32%). The presence of bilateral temporal lobe lesions on magnetic resonance imaging and cerebrospinal fluid restricted oligoclonal bands was associated with a higher probability to detect cancer at the time of AE diagnosis. All patients with Abs to neural surface antigens had a good outcome at last follow-up. Severe disability at AE onset and the lack of long-term immunosuppression predicted a poor outcome. CONCLUSIONS: Leucine-rich glioma-inactivated protein 1 immunoglobulin G was the most frequent Ab detected. Patients with bilateral temporal lobe lesions and oligoclonal bands have a higher probability to harbour an occult tumour. In these patients, a strict surveillance and monitoring for cancer detection is recommended.

The detection of neural autoantibodies in patients with antiepileptic-drug-resistant epilepsy predicts response to immunotherapy.

BACKGROUND AND PURPOSE: The detection of antibodies binding neural antigens in patients with epilepsy has led to the definition of 'autoimmune epilepsy'. Patients with neural antibodies not responding to antiepileptic drugs (AEDs) may benefit from immunotherapy. Aim of this study was to evaluate the frequency of autoantibodies specific to neural antigens in patients with epilepsy and their response to immunotherapy. METHODS: Eighty-one patients and 75 age- and sex-matched healthy subjects (HS) were enrolled in the study. Two groups of patients were included: 39 patients with epilepsy and other neurological symptoms and/or autoimmune diseases responsive to AEDs (group 1) and 42 patients with AED-resistant epilepsy (group 2). Patients' serum and cerebrospinal fluid were evaluated for the presence of autoantibodies directed to neural antigens by indirect immunofluorescence on frozen sections of mouse brain, cell-based assays and a radioimmunoassay. Patients with AED-resistant epilepsy and neural autoantibodies were treated with immunotherapy and the main outcome measure was the reduction in seizure frequency. RESULTS: Neural autoantibodies were detected in 22% of patients (18/81), mostly from the AED-resistant epilepsy group (P = 0.003), but not in HS. Indirect immunofluorescence on mouse brain revealed antibodies binding to unclassified antigens in 10 patients. Twelve patients received immunotherapy and nine (75%) achieved >50% reduction in seizure frequency. CONCLUSIONS: A significant proportion of patients with AED-resistant epilepsy harbor neural-specific autoantibodies. The detection of these antibodies, especially of those binding to synaptic antigens, may predict a favorable response to immunotherapy, thus overcoming AED resistance.

Correlations between abnormalities on brain SPECT scan and interictal EEG-foci in children with 'intractable' partial epilepsy.

SPECT scanning with Tc99mHM-PAO was performed on 40 children with 'intractable' partial epilepsy; 27 patients had abnormalities on SPECT images consisting of areas of decreased cerebral blood flow. In 18 of these 27 subjects there was a good correlation between site of the abnormalities on SPECT scan and interictal EEG foci. 12 patients with regionally decreased CBF had also neuroimaging (C.T. and/or R.M.I.) abnormalities. The brain SPECT provides more precise detection of epileptogenic foci, when used to complement EEG, also in childhood-onset epilepsy.

Brain SPECT and migraine in childhood.

The recent development of SPECT has introduced a new procedure to evaluate neurological diseases. By mean of Tc99mHM-PAO we studied a group of 19 pediatric subjects (7 males and 12 females) with different form of migraine, within five days after the last headache attack. Postictally SPECT shows regionally decreased CBF in 3/4 of HM, in 5/6 of BAM, in 5/5 of CM and in 2/4 of M.

[Ochronotic arthropathy. A case report: radiographic and scintigraphic comparative study (author's transl)]. / Presentazione di un caso di artropatia ocronotica: studio comparativo radiografico e scintigrafico.

After description of etiopathogenetic, anatomopathological and clinical aspects of the disease, the authors relate about a case of ochronotic arthropathy, lining on its typical radiological patterns. Scintigraphic study with 99mTc-pertechnetate and with 99mTc-pyrophosphate, excluding any phlogistic component, agree with dysmetabolic and degenerative nature of this arthropathy.

99mTc-pyrophosphate kinetics in man (relationship between bone uptake and mineral content).

Both the kinetics and the regional distribution of 99mTc-PPi were studied in the skeleton of healthy subjects of various ages by surface counting using a scintillation counter placed over different skeletal areas. The results were compared with bone mineral content as determined by the photonic absorption technique. Kinetic data of bone uptake were obtained by subtracting the counts measured in blood from the counts recorded over each body surface after making proportional corrections. The surface-counting patterns show a higher generalized 99mTc-PPi bone uptake in children and adolescents than in adults. The relative regional distribution of the tracer shows no difference between the two groups. The tracer accumulated more rapidly in spongy bone than in compact bone, and the fixation process was carried out during the first 30 min for both spongy and compact bone. In youths, however, higher uptake values corresponded to lower values of mineral content. The findings enable the normal surface-counting pattern to be constructed and confirm that the 99mTc-PPi uptake is related to bone metabolism; they also suggest the application of external measurements for the study of metabolic bone diseases.