Although extrapyramidal diseases are commonly thought to solely affect the extrapyramidal motor system, nonmotor symptoms such as behavioural abnormalities, dysautonomia, sleep disturbances and sensory dysfunctions are also frequently observed. Autonomic dysfunction as an important clinical component of extrapyramidal disease (idiopathic Parkinson’s disease, multiple system atrophy, progressive supranuclear palsy, dementia with Lewy bodies) is often not formally assessed and thus frequently misdiagnosed. Symptoms of autonomic dysfunction in general impact more on quality of life than motor symptoms. Appropriate symptom-oriented diagnosis and symptomatic treatment as part of an interdisciplinary approach can greatly benefit the patient. Unfortunately, double-blind, randomized, controlled studies are scarce with the consequence that most recommendations are not based on the highest level of evidence. This review elaborates a limited overview on the treatment of cardiovascular, gastrointestinal, urogenital and sudomotor autonomic dysfunction in various extrapyramidal syndromes.

Paraneoplastic neurological disorders (PNDs) are a rare and diverse group of neurological conditions that can involve any part of the nervous system. Diagnosis is facilitated by finding well-recognized autoantibodies directed against neural antigens in the sera and the cerebrospinal fluid. Identifying and eliminating the underlying malignancy is the mainstay of treatment. Immunomodulatory treatment is gaining more acceptance especially, where a malignancy could not be identified, oncology treatment is completed, or along with cancer treatment. Literature review shows only a handful of systematic prospective case series. Multicenter, prospective controlled clinical trials are needed for future therapeutic advances.

Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved.