This article discusses three patients with likely Hirayama disease. They have no other significant past medical history and no personal or family history of other neurological disorders. Hirayama disease is a form of cervical myelopathy attributed to forward displacement of the posterior cervical dural sac on neck flexion with resultant cord compression and/or venous congestion. It is characterized by a pure motor focal amyotrophy in the distribution of C7, C8 and T1 spinal segmental-innervated muscles and differs from other motor neuron diseases by virtue of its ultimately non-progressive course.

Hemiplegic migraine (HM) is a rare migraine disorder with aura subtype including temporary weakness and visual, sensory, and/or speech symptoms. To date, three main genes—CACNA1A, ATP1A2, and SCN1A—have been found to cause HM. These encode ion channels or transporters, important for regulating neuronal ion balance and synaptic transmission, leading to HM being described as a channelopathy. However, <20% of HM cases referred for genetic testing have mutations in these genes and other genes with roles in ion and solute transport, and neurotransmission has also been implicated in some HM cases. In this study, we performed whole exome sequencing for 187 suspected HM probands referred for genetic testing, but found to be negative for CACNA1A, ATP1A2, and SCN1A mutations, and applied targeted analysis of whole exome sequencing data for rare missense or potential protein-altering variants in the PRRT2, PNKD, SLC1A3, SLC2A1, SLC4A4, ATP1A3, and ATP1A4 genes. We identified known mutations and some potentially pathogenic variants in each of these genes in specific cases, suggesting that their screening improves molecular diagnosis for the disorder. However, the majority of HM patients were found not to have candidate mutations in any of the previously reported HM genes, suggesting that additional genetic factors contributing to the disorder are yet to be identified.

We describe a case of headache and neurological deficits with cerebrospinal fluid (CSF) lymphocytosis in a patient presenting with a 3-week history of recurrent severe headaches associated with negative sensory symptoms and dysphasia. The patient had no cardiovascular risk factors and no family history of migraines. Neurological examination was unremarkable. Cerebral magnetic resonance imaging was unremarkable. CSF analysis revealed lymphocytosis (leucocytes 84 × 10(6)/L, 100% lymphocytes). Extensive laboratory investigations of CSF and serum did not reveal an infectious, autoimmune or metabolic cause. Visual evoked potentials were normal. Awake electroencephalogram revealed intermittent 3-5 Hz generalised slowing and frontal intermittent rhythmic delta activity, without epileptiform discharges. Repeat CSF analysis showed marked reduction of the total leucocyte count and remained negative for infectious aetiology. Propranolol was commenced, and no recurrence of headache or neurological symptoms was observed at follow-up. An extensive literature review on the topic is discussed.

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In recent years, a rapidly increasing number of scientific papers have been published that utilize zebrafish (Danio rerio) as an alternative model organism in the study of a wide range of biological phenomena from cancer to behavior. This is, in large part, due to the prolific nature, relative ease of maintenance, and sufficiently high genetic homology of zebrafish to humans. With the surge of zebrafish use in animal research, the variations in methodologies of breeding and husbandry of this species have also increased. Investigators usually focus on the development and implementation of rigorous laboratory control that is specific to their studies. We suggest that the same scrutiny and attention may be required for the methods of breeding and housing of zebrafish. This article reviews a variety of zebrafish husbandry and breeding techniques and conditions employed around the world. It discusses factors ranging from numerous aspects of rearing/housing conditions through the sex ratio of the breeding group to the composition of the diet of zebrafish that may vary across laboratories. It provides some feedback on the potential pros and cons of the different methods. It argues that there is a substantial need for systematic analysis of these methods, that is, the effects of environmental factors on zebrafish health and breeding. It also discusses the question as to whether some degree of standardization of these methods is needed to enhance cross-laboratory comparability of results.

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Personal FM systems may substantially improve speech-in-noise deficits in stroke patients who are not eligible for conventional hearing aids. FMs are feasible in stroke patients and show promise to address impaired AP after stroke. Implications for Rehabilitation This is the first study to investigate the benefits of personal frequency-modulated (FM) systems in stroke patients with disordered AP. All cases significantly improved speech perception in noise with the FM systems, when noise was spatially separated from the speech signal by 90° compared with unaided listening. Personal FM systems are feasible in stroke patients, and may be of benefit in just under 20% of this population, who are not eligible for conventional hearing aids.

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