Handbook of clinical neurology
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The few reported controlled studies show that bilateral stimulation of the globus pallidus interna (GPi) is a safe and effective long-term treatment for hyperkinetic disorders. However, the recently published data on deep brain stimulation (DBS) applied to different targets or patients (especially those with secondary dystonia) are mainly uncontrolled case reports, precluding a clear determination of its efficacy, and providing little guidance as to the choice of a "good" target in a "good" patient. This chapter reviews the literature on DBS in primary dystonia, paying particular attention to the risk:benefit ratio in focal and segmental dystonias (cervical dystonia, cranial dystonia) and to the predictive factors for a good outcome. ⋯ This chapter provides a comprehensive analysis of the use of the treatment in various types of secondary dystonia, with little to moderate benefit in most cases, based on single cases or small series. Beyond the reduction in the severity of dystonia, the global motor and functional outcome is difficult to determine owing to the paucity of adequate evaluation tools. Because of the large interpatient variability, different targets may be effective depending on the symptoms in each individual.
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Review Historical Article
The surgery of peripheral nerves (including tumors).
Surgical pathology of the peripheral nervous system includes traumatic injury, entrapment syndromes, and tumors. The recent significant advances in the understanding of the pathophysiology and cellular biology of peripheral nerve degeneration and regeneration has yet to be translated into improved surgical techniques and better outcome after peripheral nerve injury. ⋯ Surgery continues to be the primary treatment modality for peripheral nerve tumors and advances in adjuvant oncological treatment has improved outcome after malignant peripheral nerve tumors. The present chapter provides background knowledge of surgical peripheral nerve disease and some general and practical guidance toward its clinical management.
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Anorexia nervosa is a chronic and debilitating psychiatric disorder associated with one of the highest mortality rates of any psychiatric condition. Despite advances in neuroimaging, genetics, pharmacology, and psychosocial interventions in the last half-century, little progress has been made in altering the natural history of the condition or its outcomes. ⋯ Abnormal reward processing, compulsive hyperactivity, chronic anxiety, and depression, all suggest that anorexia nervosa shares much in common with other conditions, such as major depression and obsessive-compulsive disorder, for which surgical therapy with deep brain stimulation (DBS) has been tried, with promising results. As a result, the use of DBS in treatment-resistant anorexia nervosa should be evaluated in carefully designed, early-phase feasibility trials.
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Review
Peripheral nerve involvement in hereditary cerebellar and multisystem degenerative disorders.
Hereditary ataxias (HA) encompass an increasing number of degenerative disorders characterized by progressive cerebellar ataxia usually accompanied by extracerebellar semeiology including peripheral nerve involvement. Classically, HA were classified according to their pathological hallmark comprising three main forms: (1) spinal form predominantly with degeneration of spinocerebellar tracts, posterior columns, and pyramidal tracts (Friedreich's ataxia, FA); (2) olivopontocerebellar atrophy (OPCA); and (3) cortical cerebellar atrophy (CCA). In the 1980s Harding proposed a clinico-genetic classification based upon age of onset, modality of transmission, and clinical semeiology. ⋯ In this chapter we will review peripheral nerve involvement in classical pathological entities (OPCA and CCA), ARCA, ADCA, and ILOCA paying special attention to the most prevalent syndromes in each category. As a general rule, nerve involvement is relatively common in any form of ataxia except ILOCA, the most common pattern being either sensory or sensorimotor neuronopathy with a dying-back process. An exception to this rule is AR spastic ataxia of Charlevoix-Saguenay where nerve conduction studies show the characteristic pattern of intermediate neuropathy implying that sacsin mutation causes both axonal and Schwann cell dysfunction.
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Neuropathic pain is a clinical entity that presents unique diagnostic and therapeutic challenges. This chapter addresses the classification, epidemiology, pathophysiology, diagnosis, and treatment of neuropathic pain syndrome. Neuropathic pain can be distinguished from nociceptive pain based on clinical signs and symptoms. ⋯ Nonpharmacological treatments include psychological approaches, physical therapy, interventional therapy, spinal cord stimulation, and surgical procedures. Neuropathic pain is difficult to treat, but a combination of therapies may be more effective than monotherapy. Clinical practice guidelines provide an evidence-based approach to the treatment of neuropathic pain.