Seminars in neurology
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Diabetes is associated with a variety of chronic and acute neuropathies. In this article, the authors summarize the clinical features of the most common diabetic neuropathies, focusing on those for which therapy is available or under active investigation. ⋯ Potential treatments for DSP are discussed in four broad themes: (1) medication and lifestyle therapy to improve hyperglycemia, insulin resistance, and attendant features of metabolic syndrome, including obesity and dyslipidemia; (2) pharmacologic therapy to alter neuropathy natural history aimed at rational targets from known pathophysiology; (3) symptomatic relief of neuropathic pain; and (4) treatment to prevent complications of neuropathy, including stasis ulcers and falls. The approach to the most common acute diabetic neuropathies is also reviewed.
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Seminars in neurology · Feb 2012
ReviewPelizaeus-Merzbacher disease, Pelizaeus-Merzbacher-like disease 1, and related hypomyelinating disorders.
The purpose of this article is to present contemporary information on the clinical and molecular diagnosis and the treatment of Pelizaeus-Merzbacher's disease (PMD) and related leukodystrophies. Various types of mutations of the X-linked proteolipid protein 1 gene (PLP1) that include copy number changes, point mutations, and insertions or deletions of a few bases lead to a clinical spectrum from the most severe connatal PMD, to the least severe spastic paraplegia 2 (SPG2). Signs of PMD include nystagmus, hypotonia, tremors, titubation, ataxia, spasticity, athetotic movements and cognitive impairment; the major findings in SPG2 are leg weakness and spasticity. ⋯ A similar constellation of signs and pattern of hypomyelination lead to the autosomal recessive disease called Pelizaeus-Merzbacher-like disease 1 (PMLD1) and the less-severe spastic paraplegia 44 (SPG44), caused by mutations of the gap junction protein, gamma-2 gene (GJC2), formerly known as the gap junction protein, α-12 gene (GJA12). Magnetic resonance spectroscopy (MRS) and brainstem auditory evoked potentials (BAEP) may assist with differential clinical diagnosis of PMD and PMLD1. Supportive therapy for patients with PMD/SPG2 and PMLD1/SPG44 includes medications for seizures and spasticity; physical therapy, exercise, and orthotics for spasticity management; surgery for contractures and scoliosis; gastrostomy for severe dysphagia; proper wheelchair seating, physical therapy, and orthotics to prevent or ameliorate the effects of scoliosis; special education; and assistive communication devices.
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The rising popularity of regional anesthesia in the last several decades has greatly changed the experience of labor. Although the use of regional anesthesia has aided in decreasing maternal morbidity and mortality, a new dimension of neurologic issues, particularly headache and peripheral neuropathy, is apparent. Obstetric anesthesiologists frequently encounter patients with preexisting neurologic disease. ⋯ Early consultation with an obstetric anesthesiologist, discussion with a neurologist, and communication with the obstetrician allows for the education and discussion of the risks and benefits of both the mode of delivery and anesthetic options. This multidisciplinary approach is crucial in forming reasonable expectations for the patient. The aim of this discussion is to provide an obstetric anesthesiologist's perspective on regional anesthesia and its implications in obstetrics, and to enhance communication between our specialties.
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The timing and continuity of sleep in healthy individuals is regulated by the synchronous function of the sleep homeostasis and the endogenous circadian rhythms. Multiple factors affect these two processes and the way they interact. Sleep disorders may manifest differently in men and women and these differences are particularly notable during pregnancy, lactation, and menopause. ⋯ Factors associated with increased RLS in pregnancy may be related to iron and folate metabolism. Risk for OSA increases after menopause and presentation with insomnia can delay the diagnosis of OSA. Various treatment options for sleep disorders in women are discussed.
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Headaches are a common complaint for most women during their lifetime, including gestation and puerperium. Tension-type headaches and migraine flare-ups are the most common headache complications after delivery, but the differential diagnosis of postpartum headache is broad. The clinician must distinguish common headache syndromes from dangerous causes of postpartum headache. We will focus on early postpartum headaches in the form of case discussions that will include clinical presentation, diagnostic criteria, and treatment.