Spotlight on Temporal arteritis

Temporal arteritis is an important cause of ischemic complications in elderly patients, and timely diagnosis can prevent important morbidity, most notably blindness from either anterior ischemic optic neuropathy or central retinal artery occlusion. These patients, left untreated, are also at increased risk for ischemic stroke. Treatment with high-dose corticosteroids is effective in preventing these complications, and initial treatment with intravenous pulse corticosteroids leads to more sustained clinical remission and lower subsequent oral corticosteroid dosage requirement. Other adjunctive immunosuppressant drugs are also currently being used in this setting as steroid-sparing agents.

In this article, Dr. Peter MacIntosh and Dr. Omar Solyman of the University of Illinois at Chicago cite studies showing increased risk for a variety of ischemic cardiovascular complications among patients with temporal arteritis and patients who were taking statin drugs, which had a somewhat lesser incidence of these complications. They also review advances in biological treatment of giant cell arteritis with monoclonal antibodies with and without concomitant corticosteroid treatment.

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Spotlight on West syndrome

West syndrome is a severe epileptic encephalopathy occurring in infancy that comprises specific seizure types consisting of spasms and interictal EEG pattern known as hypsarrhythmia, together with psychomotor regression. It may result from various causes, but maturation of the brain is a crucial component. Early identification and proper treatment are required, although not sufficient, to optimize the outcome and avoid long-term disabilities.

In this updated article, Dr. Puja Patel, Dr. Aristea Galanopoulou, and Dr. Solomon Moshé, all of Einstein College of Medicine, include information on diagnoses, recently identified genetic causes, drug treatments, and outcome.

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Spotlight on Cognitive aspects of central auditory disorders

Central auditory disorder is a continuum of dysfunction involving the processing and interpretation of sounds, ranging from detecting the presence of simple sounds to comprehending complex auditory stimuli such as environmental sounds and music. Central auditory disorders usually result from damage in the temporal regions near the auditory and auditory association cortex. Diagnosis must be comprehensive due to co-occurrence of other deficits and should include audiological as well as neuropsychological tests.

In this article, Dr. Douglas Lanska of the University of Wisconsin School of Medicine and Public Health and the Medical College of Wisconsin reviews the range of clinical syndromes occurring in both acquired and developmental cases and their associated pathophysiology.

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Spotlight on Kennedy disease

Kennedy disease is a rare, X-linked inherited, neurodegenerative disorder characterized by a progressive weakness of the proximal limbs and bulbar muscles, muscle atrophy, fasciculations (especially perioral), loss of reflexes, tremor, gynecomastia, and diabetes mellitus. It results from an excessive number of trinucleotide (CAG) repeats in the androgen receptor gene on the X chromosome. Due to its X-linked genetic association, males are predominantly affected. Particular clinical features and genetic testing can help distinguish Kennedy disease from amyotrophic lateral sclerosis. Patients with Kennedy disease generally live a normal lifespan, despite the fact there are no treatments currently available to halt the slow progression of the disorder.

In this updated article, Dr. Robert Pratt of the University of Colorado summarizes the latest research on Kennedy disease, with particular emphasis on insights into its natural history, pathophysiological mechanisms, and potential therapeutic strategies.

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Spotlight on Neuroacanthocytosis

In this article, Dr. William Stamey of Mid Coast Hospital in Brunswick, Maine discusses neuroacanthocytosis, a neurologic syndrome characterized by a broad spectrum of movement disorders that often share acanthocytes on the blood smear. In addition to a variety of hyperkinetic and hypokinetic movement disorders, behavioral and cognitive disturbances are common features. An autosomal recessive disorder, chorea-acanthocytosis overlaps clinically with McLeod syndrome, which is inherited as an X-linked disorder. Neuroacanthocytosis must be considered in the differential diagnosis of patients presenting with movement disorders and behavioral or cognitive findings. Treatments, including deep brain stimulation, are met with various levels of success.

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Spotlight on Neurosurgical management of chronic pain

Chronic pain encompasses a spectrum of clinical scenarios involving both peripheral and central pain syndromes. In this article, Dr. Richard Polin of George Washington University discusses the progression of understanding about the pathophysiology of pain, culminating in the gait theory hypothesis. Dr. Polin addresses pain from peripheral nerve injury, spinal cord injury, and brain injury and stroke and discusses the basic science behind the rationale for current management schemes and pain treatments (both pharmacological and surgical). Dr. Polin concludes by summarizing the success (or lack thereof) of surgical treatment modalities for chronic pain. The author updates the section on intrathecal opioid pain pumps.

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Spotlight on Hyperhomocysteinemia

Hyperhomocysteinemia is defined as elevation of plasma total concentration of homocysteine and disulfide adducts of homocysteine collectively termed “homocyst(e)ine” or “total homocysteine. In this article, Dr. Yongwoo Kim of Temple University Lewis Katz School of Medicine discusses hyperhomocysteinemia and reviews developments in the understanding of its relationship with cerebrovascular disease and dementia. Although folic acid, vitamin B12, and vitamin B6 lower homocysteine levels, many recent randomized, controlled trials suggest that treatment with these vitamins does not clearly lower the risk of vascular disease.

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Spotlight on Abdominal migraine

In this article, Dr. Shuu-Jiun Wang of the National Yang-Ming University School of Medicine and the Neurological Institute, Taipei Veterans General Hospital updates new findings for abdominal migraine as well as for a condition referred to as cyclic vomiting syndrome. New research done in Germany showed the following by using a claims dataset: (1) several episodic syndromes including abdominal migraine and cyclic vomiting were risk factors for later migraine; (2) the risk of migraine at primary school age in most exposed children is low, except for abdominal migraine (> 50%); and (3) the proportion of later migraine in children aged 6 to 10 years explained by pre-school episodic syndrome is lower than 3%.

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Spotlight on Hearing loss in infants and children

Congenital hearing loss is the most common congenital sensory impairment and the most prevalent health problem screened for at birth. Early diagnosis and intervention for hearing loss is essential to maximize speech, language, and cognitive development. Binaural hearing is critical for speech perception and communication, and the consequences of unilateral hearing loss on several performance factors can be significant in the developing child. Many types of tests, physiologic and behavioral, are available for diagnosing hearing loss and obtaining information about the auditory system. When hearing loss is confirmed, further diagnostic and genetic testing can often help identify the cause of hearing loss, with implications for prognosis and family health. Management options depend on the type and degree of hearing loss, and can include early intervention, speech therapy, sign language education, hearing aids, and surgical therapies such as tympanostomy tube insertion, cochlear implantation, and auditory brainstem implantation.

In this article, Dr. Stephanie Wong and Dr. Margo McKenna Benoit, both of the University of Rochester Medical Center, discusses the diagnosis and treatment of infants and children with hearing loss, including newborn hearing screening, audiologic testing and work-up, a brief discussion on the genetics of hearing loss, and the goals for early diagnosis and treatment.

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Spotlight on Takayasu arteritis

Takayasu arteritis, sometimes called “pulseless disease,” is a rare form of vasculitis affecting the aorta and its major branches, resulting in large-vessel occlusions. It typically occurs in young Asian women, but can be found in any ethnic group and in men. The disease is heterogeneous in presentation. Early symptoms are often constitutional, making diagnosis difficult. Subsequently, arterial occlusions occur, producing more specific ischemic symptoms such as claudication, stroke, or death.

In this article, Dr. James Brorson of the University of Chicago highlights the potential for vascular wall imaging methods to detect and monitor vascular inflammation before occlusion occurs and reviews the evidence for effective therapy with biological agents in the treatment of this uncommon disease.

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