Further evidence for treatment of recalcitrant neuropathy of the upper limb with autologous vein wrapping.

The treatment of entrapment neuropathy in the upper extremity with surgical decompression has generally provided good results. Recurrence of symptoms, however, is not uncommon, and its management is both challenging and difficult. Autologous vein graft wrapping is an alternative technique, and it is recommended for the treatment of recalcitrant upper limb nerve compression in which 2 or more previous surgical procedures have failed to resolve the problem. Each patient underwent both subjective and objective evaluation. To our knowledge, this is the largest reported single series of vein wrapping cases in the upper limb outside the United States and the first to report results using the well-validated patient evaluation measure.

Guillain-Barré syndrome presenting with facial diplegia following COVID-19 vaccination in two patients.

In March 2020, the WHO declared COVID-19 to be a global pandemic and since December 2020, millions of vaccines have been administered. To date, cases of Guillain-Barré syndrome (GBS) following a COVID vaccine (Pfizer, Johnson & Johnson, Janssen, AstraZeneca) have been reported. A 61-year-old woman developed bilateral asymmetrical lower motor neuron (LMN) facial weakness followed by limb symptoms, 10 days after receiving the first dose of AstraZeneca COVID vaccine. The second patient was a 56-year-old man who, 9 days after receiving first dose of AstraZeneca COVID vaccine, developed bilateral asymmetrical LMN facial weakness with limb symptoms. Intravenous immunoglobulin was administered with rapid recovery. These cases of GBS following the AstraZeneca COVID vaccine add to cohort of patients reported. We flag up to raise awareness of this condition post-COVID-19 vaccine and highlight the prominent bifacial involvement. Early diagnosis and prompt treatment with intravenous immunoglobulin led to rapid recovery.

Bilateral vocal fold paresis: the only presenting sign of anti-MUSK antibody myasthenia gravis.

A previously fit and well 53-year-old man was referred to the otolaryngology clinic with intermittent stridor and was found to have bilateral vocal fold paresis. Subsequent airway compromise necessitated emergency surgical tracheostomy. The man was discharged home with tracheostomy in situ and a diagnosis of idiopathic bilateral vocal cord palsy, as all primary investigations were negative. Neurological disease was suspected following readmission to hospital several weeks later with diplopia. Electromyography and serum antibody testing confirmed a diagnosis of anti-muscle-specific tyrosine kinase antibody positive myasthenia gravis (MuSK-MG); a subset of MG where autoantibodies are directed against MuSK. Resolution of bilateral vocal fold paresis was found 8 months after a short course of immunoglobulin (intravenous immunoglobulin (IVIg)) and daily mycophenolate therapy was commenced. Multidisciplinary teamwork between ear, nose and throat surgeons, neurologists and speech therapists enabled successful decannulation of tracheostomy. The patient has recovered well and remains minimally symptomatic.

Seizure in benign epilepsy with centro-temporal spikes.

Rolandic epilepsy is frequent in children but seizures are rarely seen by the physician or captured by video-EEG monitoring. In most children the attacks are few and sporadic and generally occur at night. Also, a high percentage of children with benign epilepsy with centro-temporal spikes (BECTS) have characteristic centro-temporal spikes based on routine EEG monitoring and therefore do not require further tests such as telemetry or sleep studies. We report a video-EEG recording of a seizure in a patient with rolandic epilepsy which may be useful for educational purposes.

Multiple causes of apnea in 1p36 deletion syndrome include seizures.

INTRODUCTION: Apneic episodes have not previously been described in children with 1p36 deletion syndrome with seizures. Having encountered one such patient, we reviewed our experience of breathing difficulties in this syndrome, with particular attention to evidence of ictal apnea. We describe four children with 1p36 deletion syndrome, seizures and apneic episodes. METHOD: Retrospective analysis of clinical features, seizure semiology and video-EEG data. RESULTS: All patients showed characteristic craniofacial features, mental retardation, and diffuse hypotonia and apnea. Seizure semiology included focal motor, ± secondary generalized tonic clonic and tonic events. All had histories of status epilepticus; three showed clustering of their habitual seizures. Assessment of apnea was complicated by the presence of multiple other potential causes including obesity, reflux, respiratory, and cardiac problems Epileptic apneas were confirmed in one child by video-telemetry. In three other children, an epileptic basis for apneas was inferred from their clinical histories and treatment response supported by EEG findings. In three children, epileptiform discharges occurred over fronto-centro-temporal regions. CONCLUSION: Epileptic apnea is a feature of 1p36 deletion syndrome, though episodic apnea is multifactorial in these children, and may need repeated re-appraisal.

Efficacy of sildenafil in the treatment of female sexual dysfunction due to multiple sclerosis.

PURPOSE: We assessed the tolerability, safety and efficacy of sildenafil for the treatment of women with sexual dysfunction secondary to multiple sclerosis, as well as the role of somatosensory evoked potential neurophysiological testing. MATERIALS AND METHODS: We performed a double-blind, randomized, placebo controlled, crossover study investigating the effects of sildenafil in women with multiple sclerosis and sexual dysfunction. Assessments were done by validated questionnaires. Pudendal and tibial evoked potentials were also recorded. RESULTS: A total of 19 women completed the 2 arms of the double-blind phase and 12 completed the optional open label extension phase. Statistically significant improvement following sildenafil was only reported in the lubrication domain of sexual function during the double-blind phase. There was no overall change in quality of life after sildenafil. There was a significant correlation between the latency of tibial and pudendal evoked potentials. CONCLUSIONS: Sildenafil only appeared to produce limited benefit in certain individuals with female sexual dysfunction. Some measure of the extent of neurological deficit in these patients could be ascertained from the latency of tibial evoked potentials, which correlated with pudendal evoked potentials. However, it could not predict the extent of sexual dysfunction. Sildenafil is unlikely to help all patients with neurogenic female sexual dysfunction.