Lancet neurology
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Review
Approach and management to patients with neurological disorders reporting sexual dysfunction.
Sexual difficulties are common in patients with neurological disorders, and different domains of sexual function-desire, arousal, orgasm, and ejaculation-can be affected. Advances in the past 7 years in structural and functional neuroimaging have contributed to a greater understanding of the neural pathways involved in the regulation of sexual functions in health and disease, and this increased knowledge might help with development of future therapeutic strategies. ⋯ PDE5A inhibitors and intracavernosal injections of the prostaglandin alprostadil are effective for treating erectile dysfunction; however, options for managing other domains of sexual dysfunction in men and women remain poor. Research into different domains of sexual dysfunction is likely to lead to additional therapeutic strategies in the future.
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CDKL5 deficiency disorder (CDD) was first identified as a cause of human disease in 2004. Although initially considered a variant of Rett syndrome, CDD is now recognised as an independent disorder and classified as a developmental epileptic encephalopathy. It is characterised by early-onset (generally within the first 2 months of life) seizures that are usually refractory to polypharmacy. ⋯ Gastrointestinal, sleep, and musculoskeletal problems are common in CDD, as in other developmental epileptic encephalopathies, but the prevalence of cerebral visual impairment appears higher in CDD. Clinicians diagnosing infants with CDD need to be familiar with the complexities of this disorder to provide appropriate counselling to the patients' families. Despite some benefit from ketogenic diets and vagal nerve stimulation, there has been little evidence that conventional antiseizure medications or their combinations are helpful in CDD, but further treatment trials are finally underway.