Introduction
Pathophysiology
Predisposing factors
Autonomic disorders without CNS or PNS involvement |
Pure autonomic failure (PAF) |
Autonomic disorders with brain involvement |
Multiple system atrophy (MSA) |
Wernicke Korsakoff syndrome |
Posterior fossa tumors |
Baroreflex failure |
Olivopontocerebellar atrophy |
Dementia with Lewy bodies |
Adult-onset autosomal dominant leukodystrophy (ADLD) |
Autonomic disorders with spinal cord involvement |
Traumatic tetraplegia |
Syringomyelia |
Subacute combined degeneration |
Multiple sclerosis |
Spinal cord tumors |
Autonomic neuropathies |
The acute autonomic neuropathies |
Autoimmune autonomic ganglionopathy (AAG; acute pandysautonomia) |
Acute paraneoplastic autonomic neuropathy |
Guillain–Barre syndrome |
Botulism |
Porphyria |
Drug induced acute autonomic neuropathies |
Toxic acute autonomic neuropathies |
The chronic peripheral autonomic neuropathies |
Pure adrenergic neuropathy |
Combined sympathetic and parasympathetic failure (autonomic dysfunction clinically important) |
Amyloid |
Diabetic autonomic neuropathy |
Paraneoplastic autonomic including panautonomic neuropathy |
Sensory neuronopathy with autonomic failure (most commonly associated with Sjogren’s syndrome) |
Familial dysautonomia (Riley-Day syndrome) |
Autoimmune autonomic neuropathy |
Dysautonomia of old age |
NOH in neurodegenerative disease
Evaluation
Step 1. Diagnosis of parkinsonian syndrome |
Bradikinesia (slowness of initiation of voluntary movement with progressive reduction in speed and amplitude or repetitive actions) |
And at least one of the following: |
Muscular rigidity |
4–6 Hz rest tremor |
Postural instability not caused by primary visual, vestibular, cerebellar, or proprioceptive dysfunction |
Step 2. Exclusion criteria for Parkinson’s disease |
History of repeated strokes with stepwise progression of parkinsonian features |
History of repeated head injury |
History of definite encephalitis |
Oculogyric crises |
Neuroleptic treatment at onset of symptoms |
More than one affected relative |
Sustained remission |
Strictly unilateral features after 3 years |
Supranuclear gaze palsy |
Cerebellar signs |
Early severe autonomic involvement |
Early severe dementia with disturbances of memory, language, and praxis |
Babinski signs |
Presence of a cerebral tumor or communicating hydrocephalus on CT scan |
Negative response to large doses of l-dopa (if malabsorption excluded) |
MPTP exposure |
Step 3. Supportive prospective positive criteria of Parkinson’s disease. Three or more required for diagnosis of definite Parkinson’s disease: |
Unilateral onset |
Rest tremor present |
Progressive disorder |
Persistent asymmetry affecting the side of onset most |
Excellent response (70–100 %) to l-dopa |
Severe l-dopa-induced chorea |
l-dopa response for 5 years or more |
Clinical course of 10 years or more |
Hyposmia |
Visual hallucination |