In-House Intraoperative Monitoring in Neurosurgery in England - Benefits and Challenges.

Background: Intraoperative neurophysiological monitoring (IOM) is a valuable adjunct for neurosurgical operative techniques, and has been shown to improve clinical outcomes in cranial and spinal surgery. It is not necessarily provided by NHS hospitals so may be outsourced to private companies, which are expensive and at cost to the NHS trusts. We discuss the benefits and challenges of developing an in-house service.

Electrodiagnostic findings in facial onset sensory motor neuronopathy (FOSMN).

Objective: To determine the electrodiagnostic characteristics of facial onset sensory and motor neuronopathy (FOSMN).

Methods: Electrophysiological data from 10 FOSMN patients in Newcastle-upon-Tyne and Sydney were reviewed. Relevant literature was reviewed.

Mills' syndrome revisited.

Mills' syndrome is an idiopathic, slowly progressive, spastic hemiparesis. We describe three cases that have been under review for a minimum of 11 years (range 11-19). In all patients, symptoms started in a leg, with a mean age of onset of 59 years (range 53-63).

Electromyography and muscle biopsy in paediatric neuromuscular disorders - Evaluation of current practice and literature review.

The conduct and interpretation of electromyography in children is considered difficult and therefore often avoided. We assessed the diagnostic accuracy of the paediatric electromyography protocol used in our tertiary reference centre and compared it to muscle biopsy results and clinical diagnosis. Electromyography was performed in unsedated children with suspected neuromuscular diseases between January 2010 and September 2017 and was analysed quantitatively.

Intermuscular Coherence in Normal Adults: Variability and Changes with Age.

We investigated beta-band intermuscular coherence (IMC) in 92 healthy adults stratified by decade of age, and analysed variability between and within subjects. In the dominant upper limb, IMC was estimated between extensor digitorum communis and first dorsal interosseous as well as between flexor digitorum superficialis and first dorsal interosseous. In the ipsilateral lower limb, IMC was measured between medial gastrocnemius and extensor digitorum brevis as well as between tibialis anterior and extensor digitorum brevis.

Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy.

Three patients with the clinical and investigation features of facial onset sensory and motor neuronopathy (FOSMN) syndrome are presented, one of whom came to a post-mortem examination. This showed TDP-43-positive inclusions in the bulbar and spinal motor neurones as well as in the trigeminal nerve nuclei, consistent with a neurodegenerative pathogenesis. These data support the idea that at least some FOSMN cases fall within the spectrum of the TDP-43 proteinopathies, and represent a focal form of this pathology.

A multiple regression model of normal central and peripheral motor conduction times.

Introduction: The effects of age, height, and gender on magnetic central and peripheral motor conduction times (CMCT, PMCT) were analyzed using a multiple regression model.

Methods: Motor evoked potentials were recorded in 91 healthy volunteers. Magnetic stimulation was performed over the primary motor cortex (cortical latency) and over the cervical and lumbar spines (spinal latency).

Mutations in the SPG7 gene cause chronic progressive external ophthalmoplegia through disordered mitochondrial DNA maintenance.

Despite being a canonical presenting feature of mitochondrial disease, the genetic basis of progressive external ophthalmoplegia remains unknown in a large proportion of patients. Here we show that mutations in SPG7 are a novel cause of progressive external ophthalmoplegia associated with multiple mitochondrial DNA deletions. After excluding known causes, whole exome sequencing, targeted Sanger sequencing and multiplex ligation-dependent probe amplification analysis were used to study 68 adult patients with progressive external ophthalmoplegia either with or without multiple mitochondrial DNA deletions in skeletal muscle.

Clinical reasoning: a 39-year-old man with abdominal cramps.

A 39-year-old lawyer presented with intermittent spasms and pain in his abdominal muscles, particularly the right upper quadrant. These had occurred since his mid-20s and there had been long asymptomatic periods, including 8 years prior to the most recent 4-month exacerbation. Trivial movement triggered a spasm of the abdominal muscles, leading to severe pain, which made breathing uncomfortable and interfered with sleep.

Western driving regulations for unprovoked first seizures and epilepsy.

Purpose: To review the legislation for non-commercial driving licenses in the Western world for unprovoked first seizures (UFS) and recurrence of established epilepsy, and to examine available evidence on the road traffic accident (RTA) risk in people with seizures.

Methods: Regulations for non-commercial driving licenses were sought from appropriate national or state authorities and epilepsy societies. The literature was searched for consensus guidelines and data relevant to risk analysis, including an appropriate seizure-free period (SFP).

Copper deficiency myelopathy.

Acquired copper deficiency has been recognised as a rare cause of anaemia and neutropenia for over half a century. Copper deficiency myelopathy (CDM) was only described within the last decade, and represents a treatable cause of non-compressive myelopathy which closely mimics subacute combined degeneration due to vitamin B12 deficiency. Here, 55 case reports from the literature are reviewed regarding their demographics, aetiology, haematological and biochemical parameters, spinal imaging, treatment and outcome.

Copper deficiency myelopathy and subacute combined degeneration of the cord - why is the phenotype so similar?

Copper deficiency myelopathy (CDM) is an increasingly recognised mimic of subacute combined degeneration (SCD) of the cord due to cobalamin (vitamin B(12)) deficiency. It has been suggested that copper deficiency induces myelopathy through dysfunction of cytochrome oxidase, which is known to be copper-dependent. However, cytochrome oxidase is not cobalamin-dependent, so this hypothesis fails to explain the phenotypic similarity between CDM and SCD.