Diffuse Autonomic Failure Pandysautonomia Central Preganglionic Disorders

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Multiple System Atrophy. Multiple system atrophy (MSA, Shy-Drager syndrome) is a degenerative disorder of the CNS that affects the extrapyramidal, cerebellar, and autonomic neurons (see Chaptei.34,). Autonomic dysfunction in patients with MSA is due to the loss of preganglionic neurons in the brain stem and spinal cord. Patients with MSA typically present with diffuse autonomic failure and parkinsonian, cerebellar, or pyramidal deficits in different combinations. y Autonomic features include orthostatic intolerance, erectile dysfunction in males, bowel hypomotility, urinary incontinence due to denervation of the external urinary sphincter, and respiratory disturbances (sleep apnea and laryngeal stridor). There is usually a poor response to levodopa. Pathologically, cell loss and gliosis in striatonigral, olivopontocerebellar, and autonomic neurons are evident, and intracytoplasmic oligodendroglial and neuronal inclusions are frequently present.

Parkinson's Disease. Autonomic dysfunction in patients with Parkinson's disease (see Cha.pter...34 ) is rarely as severe as that seen in patients with MSA. Autonomic failure frequently occurs late in the course of the illness and is often associated with levodopa and dopamine agonist therapy. In patients with Parkinson's disease, Lewy bodies are found not only in central but also in peripheral neurons, suggesting that autonomic dysfunction in this disorder may be caused by both preganglionic and postganglionic neuronal dysfunction.

TABLE 21-3 -- SELECTED ETIOLOGIES ASSOCIATED WITH DISORDERS OF THE AUTONOMIC NERVOUS SYSTEM

Etiological Category

Selected Etiologies

Chapter

STRUCTURAL DISORDERS

Developmental structural disorders

Arnold-Chian malformation

28

Synngomyelia

Normal pressure hydrocephalus

HEREDITARY AND DEGENERATIVE DISORDERS

Movement disorders

Multiple system atrophy

34

Degenerative motor, sensory, and autonomic disorders

Riley-Day syndrome

36

Pure autonomic failure

Inherited muscle, neuromuseular, and neuronal disorders

Amyloidosis

37

ACQUIRED METABOLIC AND NUTRITIONAL DISORDERS

Endogenous metabolic disorders

Diabetes

38

Nutritional deficiencies and syndromes associated with alcoholism

Wernicke's encephalopathy/thiamine deficiency

40

INFECTIOUS DISORDERS

Viral infections

Poliomyelitis

41

Transmissible spongiform encephalopathy

Fatal familial insomnia

43

HIV and AIDS

HIV-associated neuropathy

44

HIV-related Guillain-Barre syndrome

NEUROVASCULAR DISORDERS

Vertebrovbasilar ischemia

45

NEOPLASTIC DISORDERS

Pnmary neurological tumors

Brain stem glioma

46

DEMYELINATING DISORDERS

Demyelinating disorders of central nervous system

Multiple sclerosis with bladder dysfunction

48

Demyelinating disorders of the peripheral nervous system

Guillain-Barre syndrome

49

AUTOIMMUNE AND INFLAMMATORY DISORDERS

Lambert-Eaton myasthenic syndrome

50

TRAUMATIC DISORDERS

Spinal cord: blunt or penetrating trauma

51

EPILEPSY

Temporal/limbic seizures

52

HEADACHE AND FACIAL PAIN

Cluster headaches

53

DRUG-INDUCED AND IATROGENIC

Drugs: chemotherapeutic agents (vincritine)

55

NEUROLOGICAL DISORDERS

AIDS, Acquired immunodeficiency syndrome; HIV, human immunodeficiency virus.

AIDS, Acquired immunodeficiency syndrome; HIV, human immunodeficiency virus.

AUTONOMIC GANGLIONIC AND POSTGANGLIONIC DISORDERS

Pure Autonomic Failure. Pure autonomic failure (PAF) is an idiopathic, sporadic, degenerative disorder of the autonomic nervous system of adult onset. y Unlike MSA and Parkinson's disease, PAF has no motor manifestations. The disorder is slowly progressive and has a better prognosis than MSA (..Table.21-2. ).

Autonomic Peripheral Neuropathies and Neuropathies. Acute autonomic neuropathies with prominent sympathetic and parasympathetic involvement (i.e., acute pandysautonomia) occur in isolation or accompany the Guillain-Barre syndrome and acute paraneoplastic neuropathies. The CSF protein level may be elevated, and the autonomic features may respond to intravenous immunoglobulin. Paraneoplastic dysautonomia may occur in association with carcinoma of the lung, pancreatic carcinoma, Hodgkin's disease, testicular cancer, and other neoplasms. Paraneoplastic gastrointestinal dysfunction with constipation and intestinal pseudo-obstruction has been reported in patients with small-cell carcinoma of the lung and elevated titers of antineuronal nuclear (anti-Hu) antibodies. Acute autonomic dysfunction may also be a feature of porphyria and of the neuropathies due to drugs (e.g., vincristine, taxol, cis-platinum) and toxins (e.g., thallium, acrylamide). y

Dysautonomia is a prominent feature of some chronic neuropathies affecting small-diameter unmyelinated fibers. Diabetes mellitus is the most common cause of autonomic neuropathy in the developed world. Typical features of diabetic autonomic neuropathy are orthostatic intolerance, impotence, voiding difficulties, gastroparesis, constipation alternating with diarrhea, and gustatory sweating. Patients with primary or familial amyloid neuropathy typically present with distal sensory symptoms, although the autonomic manifestations may be the presenting feature (see Chapter.37.). Sensory neuronopathy with autonomic failure may be a paraneoplastic phenomenon that may be associated with Sjogren's syndrome (see C.h.ap.te.L.50 ) or may be idiopathic.y

Hereditary sensory and autonomic neuropathies (HSAN) are characterized by prominent sensory loss and dysautonomia. Autonomic manifestations are mild in patients with HSAN type I and II but prominent in those with HSAN type III (Riley-Day syndrome, familial dysautonomia) (see Chapter 36 ). Familial dysautonomia is an autosomal recessive disorder that is seen primarily in Ashkenazi Jewish

_TABLE 21-4 -- AUTONOMIC NERVOUS SYSTEM SYNDROMES_

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