BackgroundIntranasal administration of benzodiazepines has shown superiority over rectal administration for terminating emergency epileptic seizures in human trials. No such clinical trials have been performed in dogs.ObjectiveTo evaluate the clinical efficacy of intranasal midazolam (IN‐MDZ), via a mucosal atomization device, as a first‐line management option for canine status epilepticus and compare it to rectal administration of diazepam (R‐DZP) for controlling status epilepticus before intravenous access is available.AnimalsClient‐owned dogs with idiopathic or structural epilepsy manifesting status epilepticus within a hospital environment were used. Dogs were randomly allocated to treatment with IN‐MDZ (n = 20) or R‐DZP (n = 15).MethodsRandomized parallel‐group clinical trial. Seizure cessation time and adverse effects were recorded. For each dog, treatment was considered successful if the seizure ceased within 5 minutes and did not recur within 10 minutes after administration. The 95% confidence interval was used to detect the true population of dogs that were successfully treated. The Fisher's 2‐tailed exact test was used to compare the 2 groups, and the results were considered statistically significant if P < .05.ResultsIN‐MDZ and R‐DZP terminated status epilepticus in 70% (14/20) and 20% (3/15) of cases, respectively (P = .0059). All dogs showed sedation and ataxia.Conclusions and Clinical ImportanceIN‐MDZ is a quick, safe and effective first‐line medication for controlling status epilepticus in dogs and appears superior to R‐DZP. IN‐MDZ might be a valuable treatment option when intravenous access is not available and for treatment of status epilepticus in dogs at home.

General rightsThis document is made available in accordance with publisher policies. Please cite only the published version using the reference above. Full terms of use are available:

No abstract

This retrospective case series examined the effectiveness of spinal segmental stabilisation, with or without decompression, in nine dogs with neurological deficits associated with dorsal hemivertebrae. Data on signalment, preoperative neurological status, imaging findings, surgical techniques and outcome were evaluated. All cases occurred in young or adult, small-breed dogs with neurological signs ranging from progressive moderate pelvic limb ataxia to non-ambulatory paraparesis. Six dogs also showed urinary and faecal incontinence. In each dog, one or more dorsal thoracic hemivertebra(e) were detected by radiography and MRI. In all dogs, hemivertebra(e) were associated with kyphosis and reduced vertebral canal diameter. All dogs were surgically managed with spinal segmental stabilisation, using Steinmann pins and orthopaedic wires and/or sutures attached to the spinous processes. Three dogs also underwent additional decompressive surgery. Post-operative follow-up ranged from 1.5 to 5.5 years. Immediate or delayed post-operative complications occurred in three dogs, including implant migration or loosening. Eight dogs showed long-term gait improvement, with resolution of incontinence if previously present. At 2-6 years post-surgery, four dogs were neurologically normal, three had mild residual ataxia, one had moderate ambulatory paraparesis, and one dog relapsed 3.5 years after surgery, resulting in severe paraparesis. Spinal segmental stabilisation techniques, with or without decompression, can result in satisfactory outcomes in small dogs with hemivertebrae and mild to moderate neurological signs. Further adaptations might be required to avoid implant loosening and allow continued growth in immature dogs.

Background: Generalized myokymia and neuromyotonia (M/NM) in Jack Russell Terriers (JRTs) is related to peripheral nerve hyperexcitability syndrome in humans, a symptom complex resulting from diverse etiologies.Objective: Clinical and electrodiagnostic evaluation is used to narrow the list of possible etiological diagnoses in JRTs with M/NM.Animals: Nine healthy JRTs and 8 affected JRTs. Methods: A prospective study was conducted comparing clinical and electrophysiological characteristics in 8 JRTs affected by M/NM with 9 healthy JRT controls.Results: All affected dogs except 1 had clinical signs typical of hereditary ataxia (HA). In 6 dogs, neuromyotonic discharges were recorded during electromyogram. Motor nerve conduction studies showed an axonal neuropathy in only 1 affected dog. Compared with controls, brainstem auditory-evoked potentials (BAEP) showed prolonged latencies (P o .05) accompanied by the disappearance of wave components in 3 dogs. Onset latencies of tibial sensory-evoked potentials (SEP) recorded at the lumbar intervertebral level were delayed in the affected group (P o .001). The BAEP and SEP results of the only neuromyotonic dog without ataxia were normal.Conclusions and Clinical Importance: The BAEP and spinal SEP abnormalities observed in JRTs with M/NM were associated with the presence of HA. Therefore, these electrophysiological findings presumably arise from the neurodegenerative changes characterizing HA and do not directly elucidate the pathogenesis of M/NM. An underlying neuronal ion channel dysfunction is thought to be the cause of M/NM in JRTs.

Purpose of reviewCongenital myasthenic syndromes (CMS) are a group of heterogeneous inherited disorders caused by mutations in genes encoding proteins whose function is essential for the integrity of neuromuscular transmission. This review updates the reader on the expanding phenotypic spectrum and suggested improved treatment strategies.Recent findingsAs next-generation sequencing is taken into the clinic, its use is both continuing to unearth new causative genes in which mutations underlie CMS and also broadening the phenotypic spectrum for known CMS genes. The number of genes in which mutations may cause neuromuscular transmission defects has now passed 30. The defective transmission may be part of an overall more complex phenotype in which there may be muscle, central nervous system or other involvement. Notably, mutations in series of genes encoding proteins located in the presynatic motor bouton have been identified. Rare cases of mutations in basal laminar proteins of the synaptic cleft are coming to light and additional mutations/phenotypic features have been located in some of the larger neuromuscular junction proteins such as AGRN and MUSK, where previously mutation screening by sanger sequencing was time consuming and costly. Finally, there are more reports of the beneficial effects of treatment with β2-adrenergic receptor agonists in patients, and the study of their action in disease models.SummaryRecent studies of the CMS illustrate the increasing complexity of the genetics and pathophysiological mechanisms involved. With therapy tailored for the underlying disease mechanism treatment, although incomplete, is usually life-transforming. However, treatment for newly identified conditions in which myasthenia is only one component within complex multisystem disorder will prove challenging.

Background: The intranasal (IN) route for rapid drug administration in patients with brain disorders, including status epilepticus, has been investigated. Status epilepticus is an emergency, and the IN route offers a valuable alternative to other routes, especially when these fail.Objectives: To compare IN versus IV midazolam (MDZ) at the same dosage (0.2 mg/kg) for controlling status epilepticus in dogs.Abbreviations: BBB, blood-brain barrier; IN, intranasal; MAD, mucosal atomization device; MDZ, midazolam.

SeSAME/EAST syndrome is a multisystemic disorder in humans, characterised by seizures, sensorineural deafness, ataxia, developmental delay and electrolyte imbalance. It is exclusively caused by homozygous or compound heterozygous variations in the KCNJ10 gene. Here we describe a similar syndrome in two families belonging to the Malinois dog breed, based on clinical, neurological, electrodiagnostic and histopathological examination. Genetic analysis detected a novel pathogenic KCNJ10 c.986T4C (p.(Leu329Pro)) variant that is inherited in an autosomal recessive way. This variant has an allele frequency of 2.9% in the Belgian Malinois population, but is not found in closely related dog breeds or in dog breeds where similar symptoms have been already described. The canine phenotype is remarkably similar to humans, including ataxia and seizures. In addition, in half of the dogs clinical and electrophysiological signs of neuromyotonia were observed. Because there is currently no cure and treatment is nonspecific and unsatisfactory, this canine translational model could be used for further elucidating the genotype/ phenotype correlation of this monogenic multisystem disorder and as an excellent intermediate step for drug safety testing and efficacy evaluations before initiating human studies.