Handbook of clinical neurology
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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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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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Neuropathic pain is the most common type of pain in neuropathy. In painful polyneuropathies the pain usually has a "glove and stocking" distribution. The pain may be predominantly spontaneous, e.g., with a burning, pricking, or shooting character or characterized by evoked pain such as mechanical or cold allodynia. ⋯ Peripheral edema, weight gain, nausea, vertigo, asthenia, dry mouth, and ataxia may also occur. Topical treatments are better tolerated due to lack of systemic side-effects but there is still limited evidence for the long-term efficacy of these drugs. With available drugs, the average pain reduction is about 20-30%, and only 20-35% of the patients will achieve at least 50% pain reduction, which stresses the need of a multidisciplinary approach to pain treatment.
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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.
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Deep brain stimulation for seizures has been applied to cerebellum, caudate, locus coeruleus, subthalamic nucleus, mammillary bodies, centromedian thalamus, anterior nucleus of thalamus, hippocampus and amygdala, hippocampal commissure, corpus callosum, neocortex, and occasionally to other sites. Animal and clinical studies have primarily investigated seizure prevention and, to a lessersmaller extent, seizure interruption. No studies have yet shown stimulation able to cure epilepsy. ⋯ We do not know the mechanisms, the best stimulation parameters, the best patient population, or how to predict benefit in advance. We do not know why benefit of neurostimulation for epilepsy seems to increase over time or whether there are long-term deleterious effects. All of these questions may be answerable with a combination of laboratory research and clinical experience.