F-waves responses derived from low-intensity electrical stimulation: A method to explore split-hand pathogenesis.

Objectives: The "split-hand syndrome" is a common clinical sign in amyotrophic lateral sclerosis (ALS), being characterized by more severe atrophy of the hand muscles on the radial side of the hand compared to the ulnar side. We aimed to investigate possible physiological differences between relevant hand muscles using low-intensity F-wave stimulation to assess spinal motoneuron excitability.

Methods: We recruited 36 healthy volunteers.

Hughlings Jackson's Second Thoughts on Mental States in Epilepsy.

John Hughlings Jackson (1835-1911) was the pre-eminent British neurologist of the last 3 decades of the 19th century whose most seminal contributions related to the understanding of epileptic seizures. Jackson instructed that his personal papers should be destroyed at his death, and consequently, few examples of his handwriting now survive. We discovered a series of marginalia in Jackson's handwriting annotating one of his papers, "On temporary mental disorders after epileptic paroxysms," first published in 1875 in the .

Revisiting the compound muscle action potential (CMAP).

The compound muscle action potential (CMAP) is among the first recorded waveforms in clinical neurography and one of the most common in clinical use. It is derived from the summated muscle fiber action potentials recorded from a surface electrode overlying the studied muscle following stimulation of the relevant motor nerve fibres innervating the muscle. Surface recorded motor unit potentials (SMUPs) are the fundamental units comprising the CMAP.

A brief physiology and pathophysiology of the anorectum based on the Integral Theory paradigm.

The remit of this review is confined to the experimental scientific works and surgeries based on the Integral Theory paradigm. The video abstract summarizes the anorectal function, how ligaments cause dysfunction and cure of fecal incontinence and obstructed defecation by ligament repair. Anorectal function is reflex and binary, with cortical and peripheral components.

Underactive bladder, Fowler's syndrome are potentially curable by uterosacral ligament repair.

Underactive bladder (UAB) is essentially an inability of the bladder to properly empty. UAB symptoms, when they co-occur with posterior fornix syndrome (PFS) symptoms (urge, frequency, nocturia, chronic pelvic pain), can be cured/improved, surgically by uterosacral ligament (USL) repair, non-surgically, by devices which give mechanical support of the USLs or strengthening pelvic muscle and ligaments with squatting-based exercises. The pathogenic pathway from weak USLs to UAB (and PFS) is that, when the muscles which externally open the posterior wall of the urethra contract against lax USLs, their contractile force weakens, and they cannot open the urethra adequately.

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Accurate and rapid diagnosis of amyotrophic lateral sclerosis (ALS) is essential in order to provide accurate information for patient and family, to avoid time-consuming investigations and to permit an appropriate management plan. ALS is variable regarding presentation, disease progression, genetic profile and patient reaction to the diagnosis. It is obviously important to exclude treatable conditions but, in most patients, for experienced neurologists the diagnosis is clear-cut, depending on the presence of progressive upper and lower motor neuron signs.

Segmental motor neuron dysfunction in amyotrophic lateral sclerosis: Insights from H reflex paradigms.

Introduction/aims: In amyotrophic lateral sclerosis (ALS), the role of spinal interneurons in ALS is underrecognized. We aimed to investigate pre- and post-synaptic modulation of spinal motor neuron excitability by studying the H reflex, to understand spinal interneuron function in ALS.

Methods: We evaluated the soleus H reflex, and three different modulation paradigms, to study segmental spinal inhibitory mechanisms.

A neurophysiological approach to mirror movements in amyotrophic lateral sclerosis.

Objective: To investigate mirror activity in amyotrophic lateral sclerosis (ALS) patients, using a simple paradigm of signal quantification.

Methods: Patients were asked to perform a brief isometric maximum contraction of the abductor digiti minimi (ADM) or tibialis anterior (TA) on one side, while relaxing the contralateral side of the body. Both sides were investigated.

Current challenges in primary lateral sclerosis diagnosis.

Introduction: Primary lateral sclerosis (PLS) is a rare, adult-onset and slowly progressive motor neuron disorder whose clinical core is characterized by upper motor neuron (UMN) dysfunction. Its formal diagnosis is clinically based and disease duration-dependent. Differentiating PLS from other disorders involving UMN can be challenging, particularly in the early stages.

Impact of diabetes mellitus on the respiratory function of amyotrophic lateral sclerosis patients.

Background And Purpose: Respiratory insufficiency and its complications are the main cause of death in amyotrophic lateral sclerosis (ALS). The impact of diabetes mellitus (DM) on respiratory function of ALS patients is uncertain.

Methods: A retrospective cohort study was carried out.

Upper and lower motor neuron neurophysiology and motor control.

This chapter considers the principles that underlie neurophysiological studies of upper motor neuron or lower motor neuron lesions, based on an understanding of the normal structure and function of the motor system. Human motor neurophysiology consists of an evaluation of the active components of the motor system that are relevant to volitional movements. Relatively primitive motor skills include locomotion, much dependent on the spinal cord central pattern generator, reaching, involving proximal and distal muscles activation, and grasping.

Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience.

: Motor Neuron Diseases (MND) have a large clinical spectrum, being the most common amyotrophic lateral sclerosis (ALS) but there is significant clinical heterogeneity. Our goal was to investigate this heterogeneity and any potential changes during a long period. : We performed a retrospective cohort study among a large Portuguese cohort of MND patients ( = 1550) and investigated changing patterns in clinical and demographic characteristics over the 27-year period of our database.

Exploring the split hand phenomenon with the neurophysiological index.

In 164 subjects of different age groups, we studied the neurophysiological index (NI) ([CMAP amplitude/Distal motor latency] *[F-wave frequency]; CMAP=compound muscle action potential) for three hand muscles (APB= abductor pollicis brevis; FDI= first dorsal interosseous; ADM= abductor digiti minimi). A split hand index based on CMAP amplitude (SHI_CMAP) and NI (SHI_NI) were calculated ([APB CMAP amplitude or NI * FDI CMAP amplitude or NI]/[ADM CMAP amplitude or NI]). All these neurophysiological measurements differed between age groups (p<0.

Mirror movements - A simple algorithm for mirror activity signal processing and normative values.

Mirror activity is an involuntary activation of a muscle when the respective contralateral muscle is contracting. This phenomenon has been described primarily in children and in disease states, and, more recently, also in healthy adults. Different ways of assessing mirror activity have been described.

Respiratory phenotypes in amyotrophic lateral sclerosis as determined by respiratory questions on the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised and their relation to respiratory tests.

Background And Purpose: Respiratory insufficiency and its complications are the main cause of death in amyotrophic lateral sclerosis (ALS). Respiratory symptoms are scored in questions Q10 (dyspnoea) and Q11 (orthopnoea) of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R). The association of respiratory test alterations with respiratory symptoms is unclear.

Trends in the diagnostic delay and pathway for amyotrophic lateral sclerosis patients across different countries.

Background: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease with a median survival of 2-5 years. An early diagnosis is essential for providing ALS patients the finest management possible. Studies from different countries report a similar median diagnostic delay of around 12 months, which is still far from desirable.

The cutaneous silent period as a measure of upper motor neuron dysfunction in amyotrophic lateral sclerosis.

Objectives: We investigated the cutaneous silent period (CutSP) as a measure of upper motor neuron (UMN) dysfunction in amyotrophic lateral sclerosis.

Methods: The onset latency, duration, and amount of EMG suppression of the CutSP were compared with clinical UMN signs in 24 patients with amyotrophic lateral sclerosis (ALS). UMN signs were quantified using a clinical index and transcranial magnetic stimulation (TMS).

The flail-arm syndrome: the influence of phenotypic features.

The flail-arm syndrome (FAS), one of the Amyotrophic lateral sclerosis (ALS) phenotypes, is characterized by slow progression and predominantly lower motor neuron (LMN) involvement with proximal upper limb (UL) weakness. We aim to characterize the clinical features, progression and survival of FAS associated with distal or proximal onset and presence or absence of upper motor neuron signs (UMN) signs at diagnosis. Data from 704 ALS patients was analyzed.

Transcranial magnetic stimulation to monitor disease progression in ALS: a review.

: Transcranial magnetic stimulation (TMS) is a technique to assess motor system function which has been used extensively in amyotrophic lateral sclerosis (ALS). Information on the changes during disease progression is scarce. We aimed to collect this information in a single source.

Wasted leg syndrome: An atypical slowly-progressive form of lower motor neuron disease.

We describe four male patients with wasted-leg syndrome, with predominant asymmetric thigh atrophy and weakness that stabilized after a period of slow progression (follow-up 7-18 years). Two patients had an Indian ethnic background and two were Portuguese, without known Indian ancestry. Other mimicking disorders were excluded, but one Indian patient was later diagnosed with CADASIL.

Finding Common Ground on the Site of Onset of Amyotrophic Lateral Sclerosis.

The fundamental origin of amyotrophic lateral sclerosis (ALS) has remained an enigma since its earliest description as a relentlessly progressive degeneration with prominent neuromuscular manifestations that are associated with upper and lower motor neuron dysfunction. Although this remains the hallmark of ALS, a significant proportion of patients will also demonstrate one or more features of frontotemporal dysfunction, including a frontotemporal dementia (FTD). Understanding whether these 2 seemingly disparate syndromes are simply reflective of the co-occurrence of 2 distinct pathologic processes or the clinical manifestations of a common pathophysiologic derangement involving the brain more widely has gripped contemporary ALS researchers.

Mild dysphagia does not influence survival in ventilated amyotrophic lateral sclerosis patients.

Background: Some amyotrophic lateral sclerosis (ALS) patients on continuous non-invasive ventilation (NIV) develop dysphagia demanding a clinical decision regarding gastrostomy.

Material And Methods: We have analyzed a cohort of seventy-four ALS patients dependent on continuous NIV (>22 h/day) and without feeding tube.

Results: Three patients underwent to gastrostomy due to dysphagia progression.