Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study.

Objective: To clarify the role of electroencephalography (EEG) as a promising marker of severity in amyotrophic lateral sclerosis (ALS). We characterized the brain spatio-temporal patterns activity at rest by means of both spectral band powers and EEG microstates and correlated these features with clinical scores.

Methods: Eyes closed EEG was acquired in 15 patients with ALS and spectral band power was calculated in frequency bands, defined on the basis of individual alpha frequency (IAF): delta-theta band (1-7 Hz); low alpha (IAF - 2 Hz - IAF); high alpha (IAF - IAF + 2 Hz); beta (13 - 25 Hz).

Hyper-reflexia in Guillain-Barré syndrome: systematic review.

Areflexia or hyporeflexia is a mandatory clinical criterion for the diagnosis of Guillain-Barré syndrome (GBS). A systematic review of the literature from 1 January 1993 to 30 August 2019 revealed 44 sufficiently detailed patients with GBS and hyper-reflexia, along with one we describe. 73.

Acute motor axonal neuropathy and transverse myelitis overlap: the importance of history taking.

In young adults, acute motor axonal neuropathy and transverse myelitis rarely occur as associated conditions. Clinical reasoning, symptoms, laboratory and ancillary investigations (electroneurographic and radiological findings), should properly address the physician to the correct diagnosis.

Demyelinating Guillain-Barré syndrome recurs more frequently than axonal subtypes.

Guillain-Barré syndrome (GBS) is considered a monophasic disorder yet recurrences occur in up to 6% of patients. We retrospectively studied an Italian-Japanese population of 236 GBS and 73 Miller Fisher syndrome (MFS) patients and searched for factors which may be associated with recurrence. A recurrent patient was defined as having at least two episodes that fulfilled the diagnostic criteria for GBS and MFS with an identifiable recovery after each episode and a minimum of 2months between episodes.

PDCD10 gene mutations in multiple cerebral cavernous malformations.

Cerebral cavernous malformations (CCMs) are vascular abnormalities that may cause seizures, intracerebral haemorrhages, and focal neurological deficits. Familial form shows an autosomal dominant pattern of inheritance with incomplete penetrance and variable clinical expression. Three genes have been identified causing familial CCM: KRIT1/CCM1, MGC4607/CCM2, and PDCD10/CCM3.

Autoantibodies to neurofascin-186 and gliomedin in multifocal motor neuropathy.

We tested autoantibodies to neurofascin-186 (NF186) and gliomedin in sera from patients with multifocal motor neuropathy (MMN, n=53) and chronic inflammatory demyelinating polyneuropathy (CIDP, n=95) by ELISA. IgG antibodies to NF186 or gliomedin were found in 62% of MMN and 1% of CIDP sera, and IgM antibodies to the same antigens in 12% of MMN and 1% of CIDP sera. These autoantibodies activated complement.

Neuroprotective effect of cathodal transcranial direct current stimulation in a rat stroke model.

Experimental focal brain ischemia generates in the penumbra recurrent depolarizations which spread across the injured cortex inducing infarct growth. Transcranial direct current stimulation can induce a lasting, polarity-specific, modulation of cortical excitability. To verify whether cathodal transcranial direct current stimulation could reduce the infarct size and the number of depolarizations, focal ischemia was induced in the rat by the 3 vessels occlusion technique.

Local and remote effects of transcranial direct current stimulation on the electrical activity of the motor cortical network.

We systematically investigated the effects of cathodal and anodal Transcranial Direct Current Stimulation (CtDCS, AtDCS) on the electric activity of primary motor cortex during a motor task. High-density electroencephalography was used to define the spatial diffusion of tDCS after effects. Ten healthy subjects performed a finger tapping task with the right hand before and after three separate sessions of 20 minutes of Sham, AtDCS or CtDCS over left primary motor cortex (M1).

Natura non facit saltus in anti-ganglioside antibody-mediated neuropathies.

Natura non facit saltus (Latin for "nature does not make jumps") is a maxim expressing the idea that natural things and properties change gradually, in a continuum, rather than suddenly. In biomedical sciences, for taxonomic purposes, we make jumps that emphasize differences more than similarities. Among the dysimmune neuropathies, 2 disorders, characterized by the presence of antibodies to gangliosides GM1 and GD1a and a peculiar, exclusive motor involvement, have been identified: acute motor axonal neuropathy (AMAN) and multifocal motor neuropathy (MMN).

Electrophysiological comparison between males and females in HNPP.

Some evidences highlighted a higher clinical expression of hereditary neuropathy with liability to pressure palsy (HNPP) in males, and a higher load of traumatic nerve injuries due to different occupational activity has been invoked to explain this observation. It is unknown whether this increased clinical impairment corresponds to a greater electrophysiological involvement. Thus, we compared clinical and electrophysiological features between men and women in a large cohort of HNPP patients.

Pseudocortical and dissociate discriminative sensory dysfunction in a thalamic stroke.

In thalamic lesions a pseudocortical syndrome has been occasionally described but the effect of the lesion on the cortical network of tactile recognition has never been studied. We report a patient who developed tactile agnosia in the left hand after right thalamic stroke, configuring a pseudocortical sensory syndrome. The discriminative sensory dysfunction was dissociate because only tactile agnosia and mild pseudoathetosis were present.

Guillain-Barré syndrome associated with normal or exaggerated tendon reflexes.

Areflexia is part one of the clinical criteria required to make a diagnosis of Guillain-Barré syndrome (GBS). The diagnostic criteria were stringently developed to exclude non-GBS cases but there have been reports of patients with GBS following Campylobacter jejuni enteritis with normal and exaggerated deep tendon reflexes (DTRs). The aim of this study is to expand the existing diagnostic criteria to preserved DTRs.

Antiganglioside antibodies are associated with axonal Guillain-Barré syndrome: a Japanese-Italian collaborative study.

Background: Whether or not antiganglioside antibodies are related to axonal or demyelinating Guillain-Barré syndrome (GBS) is still a matter of controversy, as detailed in previous studies conducted in Western and Asian countries.

Objective: To clarify whether antiganglioside antibodies are associated with axonal dysfunction in Japanese and Italian GBS patient cohorts.

Methods: Clinical and electrophysiological profiles were reviewed for 156 GBS patients collected from Japan (n=103) and Italy (n=53).

Polymorphism of CD1 and SH2D2A genes in inflammatory neuropathies.

In the quest for susceptibility factors of inflammatory neuropathies, many genes implicated in the pathogenesis of autoimmune diseases have been investigated with negative or conflicting results. We studied, with a gene candidate approach, the CD1 system specialized in capturing and presenting glycolipids to antigen-specific T cells, and the SH2D2A gene encoding for a T-cell-specific adapter protein implicated in control of early T-cell activation. In Guillain-Barré syndrome, an initially positive association study with polymorphism of CD1A and CD1E genes was not confirmed.

Involvement of sensory fibres in axonal subtypes of Guillain-Barre syndrome.

Background: Acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN) are due to an antiganglioside antibody mediated attack, thought to be restricted to motor fibres in AMAN. Sensory symptoms and minor sensory conduction abnormalities, however, have been reported in some AMAN patients.

Objective: To verify whether sensory fibres are truly spared in AMAN and whether AMAN and AMSAN represent a continuum.

Motor and sensory conduction failure in overlap of Guillain-Barré and Miller Fisher syndrome: two simultaneous cases.

We report 2 patients diagnosed simultaneously with an overlap of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS), who had anti-GT1a, anti-GQ1b, anti-GD1a and anti-GD1b antibodies. There was no identifiable specific preceding infection. Both patients presented with upper and lower limb paresthesias and severe weakness, bulbar and facial weakness, ophthalmoparesis and areflexia.