Clinical data and the identification of special forms of multiple sclerosis in 1271 cases studied with a standardized documentation system

In a multicenter study the clinical data of 1271 patients with multiple sclerosis (MS) were recorded in a standardized manner and analysed by a computer program.

Some of the retrospective data are compared with previous reports. The frequency of optic nerve involvement in the present series was close to the Japanese figures.

The development of signs and symptoms during the course of MS was given for the 1271 patients and differences in the reversibility of symptoms are presented.

In this study, one of the chief purposes was the selection of groups of MS patients with particular symptomatology and course of the disease for prospective, detailed study.

The following groups were selected and are under further investigation: 109 patients with an exclusively spinal symptomatology throughout the course of their disease; 441 patients with optic neuritis as initial symptom; 110 patients with early brain-stem involvement; 64 benign cases (duration of the disease more than 14 years and disability not more than 3 according to Kurtzke); 35 malignant cases (duration of the disease under 5 years and disability grade of 7 or more); 83 families with more than one member with MS; 289 females with a history of pregnancy, childbirth and/or use of oral contraceptives.

In 339 patients a lumbar puncture was performed at the present examination. The parameters determined constitute a pathognomonic pattern highly indicative of the diagnosis of MS.     

Assessment of the technical usability and efficacy of a new portable dry-electrode EEG recorder: First results of the HOMEONE study

ObjectivesThe HOME project is intended to provide evidence of diagnostic and therapeutic yield of a patient-controlled EEG home-monitoring for neurological outpatients.MethodsThis study evaluated the technical and practical usability and efficacy of a new portable dry-electrode EEG recorder in comparison to conventional EEG devices based on technical assessments and inter-rater comparisons of EEG record examinations of office-based practitioners and two experienced neurologists.ResultsThe technical assessment was based on channel-wise comparisons of band power values derived from power spectra as observed in two recording modalities. Slight yet significant differences were observed only in the Delta-frequency band (1.5–4 Hz). The fraction of automatically detected artifact segments was larger in the new portable recordings than in conventional recordings (20% vs. 11%, median). Overall, 93% of raters’ stated diagnostic findings gathered from conventional devices were concordant with stated diagnostic findings gathered from the new portable device.ConclusionThe new EEG device was shown to have technical comparability to and a high concordance rate of diagnostic findings with conventional EEG devices.SignificanceThe new portable dry-electrode EEG device is suitable to meet the HOME projects’ goal of establishing a patient-controlled EEG home-monitoring in the routine care of neurological outpatients.Trial registrationDRKS DRKS00012685. Registered 09 August 2017, retrospectively registered.     

De novo variant in SCN4A causes neonatal sodium channel myotonia with general muscle stiffness and respiratory failure

Variants of the skeletal muscle sodium channel gene SCN4A are associated with different neuromuscular disorders including sodium channel myotonia. Here, we report an infant with a de novo variant in SCN4A presenting with neonatal onset of severe muscle stiffness with involvement of facial and eyelid muscles, and life-threatening events with respiratory failure due to severe apnoea and thorax rigidity. The boy dramatically improved in both respiratory and motor function under carbamazepine therapy.     

The impact of accelerated high frequency rTMS on brain neurochemicals in treatment-resistant depression: Insights from 1H MR spectroscopy

Objective

Although accelerated repetitive transcranial magnetic stimulation (rTMS) designs seem to be able to alleviate mood over a relatively short period of time, no studies yet examined the cellular effects on neurochemicals with regard to working mechanisms, safety and neural integrity.

Monaural 40-Hz auditory steady-state magnetic responses can be useful for identifying epileptic focus in mesial temporal lobe epilepsy

ObjectivePatients with mesial temporal lobe epilepsy (mTLE) often exhibit central auditory processing (CAP) dysfunction. Monaural 40-Hz auditory steady-state magnetic responses (ASSRs) were recorded to explore the pathophysiology of mTLE.MethodsEighteen left mTLE patients, 11 right mTLE patients and 16 healthy controls (HCs) were examined. Monaural clicks were presented at a rate of 40 Hz. Phase-locking factor (PLF) and power values were analyzed within bilateral Heschl’s gyri.ResultsMonaural 40-Hz ASSR demonstrated temporal frequency dynamics in both PLF and power data. Symmetrical hemispheric contralaterality was revealed in HCs. However, predominant contralaterality was absent in mTLE patients. Specifically, right mTLE patients exhibited a lack of contralaterality in response to left ear but not right ear stimulation, and vice versa in left mTLE patients.ConclusionThis is the first study to use monaural 40-Hz ASSR with unilateral mTLE patients to clarify the relationship between CAP and epileptic focus. CAP dysfunction was characterized by a lack of contralaterality corresponding to epileptic focus.SignificanceMonaural 40-Hz ASSR can provide useful information for localizing epileptic focus in mTLE patients.