Category Archives: Content Spotlight

Spotlight on Sleep-related movement disorders

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Movement disorders are classically thought to resolve during sleep. Sleep-related movement disorders, however, are a subset that are characterized by their presence in sleep.

In this article, Dr. Lauren Talman and Dr. Stephanie Bissonnette of Boston Medical Center discuss these sleep-related movement disorders based on the International Classification of Sleep Disorders-3rd Edition (ICSD-3). These disorders include restless legs syndrome, periodic limb movement disorder, sleep-related leg cramps, sleep-related bruxism, sleep-related rhythmic movement disorder, benign sleep myoclonus of infancy, propriospinal myoclonus at sleep onset, sleep-related movement disorders due to medical disorders, and sleep-related movement disorders due to medication or other substance.

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Spotlight on Meningiomas

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Meningiomas are the most common primary intracranial tumors. Many meningiomas can be followed clinically and radiographically and may not require therapeutic intervention. Although most are benign, morbidity can be high, and in some cases the tumor is fatal.

In this article, Dr. Karen Dixit of Northwestern University Feinberg School of Medicine reviews the pathophysiology, presentation, and treatment of this tumor, including new prognostic indicators in atypical and malignant meningiomas.

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Spotlight on Akathisia

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In this article, Dr. Stewart Factor of Emory University School of Medicine discusses akathisia, the abnormal state of motor restlessness; it is most commonly caused by neuroleptic therapy and is sometimes perceived as anxiety. Akathisia can be an acute side effect that improves with withdrawal of medication, or it can be tardive, which occurs after chronic therapy and worsens with drug withdrawal. Literature demonstrates that it occurs with atypical antipsychotics although perhaps less commonly than is seen with typical agents. It can also be caused by antidepressants. The treatment is different for acute and tardive forms. For the acute form withdrawal of the causative agent followed by use of propranolol, mirtazapine and trazodone may be used if necessary. These agents can be helpful for the tardive form, but other drugs for tardive dyskinesia may also be tried.

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Spotlight on Acromegaly and gigantism

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Acromegaly is a disease characterized by the gradual enlargement of the peaked (acral) parts of the body, including the nose, lips, tongue, lower jaw, hands, and feet and by hyperplastic alterations in the entire osseous system. Gigantism occurs before epiphyseal closure. AIP mutations (AIPmut) often predispose to familial isolated pituitary adenomas with clinical features that may negatively impact treatment efficacy.

In this article, Dr. Ryan Jacobson of Rush University discusses the clinical picture of acromegaly, which is influenced by many factors, including the levels of growth hormone and insulin-like growth factor, age, tumor size, and delay in diagnosis. Surgical excision of the adenoma by transnasal transsphenoidal approach or by endoscopic endonasal transsphenoidal surgery is recommended. In the case of radiotherapy, stereotactic radiosurgery with the use of the gamma knife is choice. Pegvisomant is a medical therapy that blocks growth hormone action at peripheral receptors, normalizes insulin-like growth factor-1 levels, reduces signs and symptoms, and corrects metabolic defects.

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Spotlight on Inadequate sleep hygiene

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Inadequate sleep hygiene entails the behaviors, practices, rituals, and habits that result in sleep onset or maintenance difficulties and unrefreshing sleep. It is prevalent across all age groups from young children to the elderly. In our present society, we embrace a culture of taking liberties with our sleep in order to improve academic performance and productivity or to fulfill social or official obligations. This leads to behaviors and habits that then make it difficult to fall asleep or stay asleep through the night. This can result in chronic sleep complaints as well as daytime fatigue and sleepiness. In addition, poor sleep hygiene has been shown to worsen other comorbid psychiatric and neurologic disorders. Electronic device use before or during bedtime, especially in young adults, is becoming more common and has been associated with higher risk of poor sleep. Recognition, counseling, and therapeutic strategies can result in increased sleep quantity, improved sleep quality, and improved daytime functioning.

In this article, Dr. Souzana Obretenova and Dr. Raman Malhotra of Washington University School of Medicine discuss the presentation, impact, and treatment of inadequate sleep hygiene.

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Spotlight on Personalized neurology

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Personalized neurology is the application of principles of personalized medicine, ie, the prescription of specific therapeutics best suited for an individual taking into consideration both genetic and environmental factors that influence response to therapy. The aim is to improve the efficacy and reduce the adverse effects of various therapies. Several biotechnologies are being integrated to develop personalized medicine. Besides omics, eg, neurogenomics and neuroproteomics, nongenomic technologies such as cell therapy and nanobiotechnology are also used. Biomarkers and integration of diagnostics with therapeutics are important for the selection and monitoring of treatments.

Dr. K K Jain is a Consultant in Neurology in Basel, Switzerland, and in this article, he provides examples of personalized approaches in the management of particular neurologic disorders.

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Spotlight on Fabry disease

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In this article, Dr. Raphael Schiffmann of the Institute of Metabolic Disease at Baylor Research Institute discusses Fabry disease, an X-linked disorder of glycosphingolipid metabolism that is caused by deficiency of alpha-galactosidase A. As a result of the disorder, patients have a markedly increased risk of developing common-looking small fiber peripheral neuropathy, ischemic stroke, myriad cardiac manifestations, and chronic renal disease. Some studies have found that about 0.5% of patients with stroke have GLA gene mutations.

Specific therapy for Fabry disease now exists, including enzyme replacement and pharmacological chaperones. Modified enzyme replacement therapy with a long circulation half-life and substrate synthesis inhibitors are being tested. Current enzyme replacement therapy does not lower the risk of stroke. Clinical experience suggests that antiplatelet agents that are ADP-receptor blockers markedly reduce the risk of stroke in Fabry disease patients.

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Spotlight on Cerebellar infarction and cerebellar hemorrhage

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Cerebellar infarct and hemorrhage account for about 2% of all strokes. Both conditions are critical neurologic disorders with potential catastrophic outcomes. They frequently present with headache, nausea and vomiting, dizziness, and a striking difficulty standing or walking. Their management often requires surgical intervention, and the prognosis is usually good when the intervention is performed before the patient becomes comatose.

In this article, Dr. Winnie Lau and Dr. J Dedrick Jordan of the University of North Carolina School of Medicine discuss the clinical presentations associated with the different vascular territories within the cerebellum, the potential to confuse its presentation with peripheral causes of vertigo, and the usefulness of the head thrust test to differentiate between peripheral and central causes of dizziness.

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Spotlight on Acquired human cytomegalovirus

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Human cytomegalovirus, also known as human herpesvirus 5, is a common pathogen that infects a majority of the adult population. After acute infection, the virus remains in a latent state but can reactivate to generate viremia or viruria, typically in the setting of immune system dysfunction. Human cytomegalovirus infection in immunocompetent hosts is often asymptomatic or self-limited whereas immunocompromised individuals can experience a more severe, life-threatening clinical course. Human cytomegalovirus infection can have a variety of clinical neurologic presentations, including retinitis, encephalitis, or radiculitis, and has also been implicated in cancer and vascular disease in immunocompetent individuals. Diagnosis is made through serological testing and molecular assays among individuals with a compatible clinical presentation, and current treatments are typically effective at controlling clinical symptoms.

In this article, Dr. Diana Vargas, Taylor Harrison, and Dr. William Tyor of Emory University School of Medicine discuss the manifestations, diagnosis, and management of human cytomegalovirus infection, which is a public health concern due to its widespread prevalence and the increasing number of immunosuppressed individuals.

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Spotlight on Sciatic neuropathy

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Sciatic neuropathy is an important consideration in the patient with foot drop. Causes of this mononeuropathy include total hip arthroplasty, unusual forms of leg or buttock compression, neoplasms, and a heterogeneous group of other disorders. Partial sciatic nerve injuries often affect the fibular (peroneal) division out of proportion to the tibial division. Electrodiagnostic testing is crucial to evaluate for subclinical involvement of tibial innervated muscles in sciatic neuropathy, which may be only subtly involved on clinical examination.

In this article, Dr. Olivia Tong and Dr. Steven Herskovitz, both of Montefiore Medical Center, summarize the clinical, etiologic, diagnostic, and management issues regarding sciatic neuropathy.

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