Handbook of clinical neurology
-
This chapter considers the use of central thalamic deep brain stimulation (CT/DBS) to support arousal regulation mechanisms in the minimally conscious state (MCS). CT/DBS for selected patients in a MCS is first placed in the historical context of prior efforts to use thalamic electrical brain stimulation to treat the unconscious clinical conditions of coma and vegetative state. ⋯ The conceptual foundations for CT/DBS in selected patients in a MCS are then presented with consideration of both circuit and cellular mechanisms underlying recovery of consciousness identified from empirical studies. Directions for developing future generalizable criteria for CT/DBS that focus on the integrity of necessary brain systems and behavioral profiles in patients in a MCS that may optimally response to support of arousal regulation mechanisms are proposed.
-
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.
-
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.
-
End-of-life care practices and attitudes in Europe are highly diverse, which is unsurprising given the variety of cultural and religious patterns across this region. The most marked differences are in the legal and ethical stances towards assisted dying, although there are also variations in limitation of life-sustaining treatment and the authority of advance directives to decline such treatment. ⋯ Fueled by the politically led process of European harmonization, future policies and laws on end-of-life care might converge. However, at the base of many ethical conflicts there remain deeply rooted differences about promoting the sanctity of life, eradicating suffering, and respecting patients' autonomous wishes.
-
This chapter summarizes progress in the evaluation of peripheral nerve (PN) lesions and disorders by imaging techniques encompassing magnetic resonance imaging (MRI) and nerve ultrasound (US). Due to the radiation exposure and limited sensitivity in soft tissue contrast, computed-tomography (CT) plays no significant role in the diagnostic work-up of PN disorders. MRI and US are complementary techniques for the evaluation of peripheral nerves, each having particular advantages and disadvantages. ⋯ In experimental settings, novel molecular and cellular MRI contrast agents allow in-vivo assessment of nerve regeneration as well as monitoring of neuroinflammation. Depending on further clinical development, contrast-enhanced MRI has the potential to follow cellular responses over time in vivo and to overcome the current limitations of histological assessment of nerve afflictions. Further advances in contrast-enhanced US has the potential for developing into a tool for the assessment of nerve blood perfusion, paving the way for better assessments of ischemic neuropathies.