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ulousness with activity of the limb, but, as discussed further on, it is the maintenance of a posture that truly exaggerates these tremors Clinically, one type of faster essential tremor is of the same frequency (6 to 8 Hz) as enhanced physiologic tremor, which has led several clinicians to declare their identity However, there are certain clinical and physiologic differences, as indicated below Familial or essential tremor most often makes its appearance late in the second decade, but it may begin in childhood and then persist A second peak of increased incidence occurs in adults over 35 years of age Both sexes are affected It is a relatively common disorder, with an estimated prevalence of 415 per 100,000 persons over the age of 40 years (Haerer et al) The tremor frequency, as remarked earlier, is 4 to 8 Hz, usually at the lower end of this range; it is of variable amplitude The frequency diminishes slightly with age while its amplitude increases, as described by Elble Aside from its frequency, the identifying feature is its appearance or marked enhancement with attempts to maintain a static limb posture Like most tremors, essential tremor is worsened by emotion, exercise, and fatigue The tremor practically always begins in the arms and is usually symmetrical; in approximately 15 percent of patients, however, it may appear rst in the dominant hand A severe isolated arm or leg tremor should suggest another disease (Parkinson disease or focal dystonia, as described further on) The tremor may be limited to the upper limbs, or a side-to-side or nodding movement of the head may be added or may occur independently Infrequently, the tremor of the head precedes that of the hands The head tremor is also postural in nature and disappears when the head is supported It has also been noted that the limb and head tremors tend to be muted when the patient walks In some of our patients whose tremor remained isolated to the head for a decade or more, there has been little if any progression to the arms and almost no wors-.

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of sweating in areas of sensory loss, but otherwise the autonomic nervous system is unaffected In distinction to other polyneuropathies, tendon re exes are usually preserved in leprosy despite widespread sensory loss Probably this is the result of sparing of most of the muscular and larger sensory nerves Because of widespread anesthesia, injuries may pass unrecognized, with resultant infections, trophic changes, and loss of tissue Variations in host immunity result in patterns of disease having both tuberculoid and lepromatous characteristics (dimorphous leprosy) Erythema nodosum occurs in a few cases The diagnosis can be made from a skin scraping or biopsy but often, multiple samples are required Treatment All forms of leprosy require long-term treatment with sulfones (dapsone being the most commonly used), rifampin, and clofazimine The skin lesions of lepromatous leprosy are responsive to thalidomide, which itself may cause a sensory neuropathy (page 1041; Barnhill and McDougall) Reactivation of disease, or a conversion from the tuberculoid to the lepromatous pattern, may occur during times of reduced immunity Polyneuropathy with Hypothyroidism Although characteristic disturbances of skeletal muscle are known to complicate hypothyroidism (see Chap 51), the demonstration of a de nite polyneuropathy has been infrequent However, a number of elderly myxedematous patients complain of weakness and numbness of the feet, legs, and, to a lesser extent, hands, for which no other explanation can be found Loss of re exes, diminution in vibratory, jointposition, and touch-pressure sensations, and weakness in the distal parts of the limbs are the usual ndings The neuropathic manifestations are seldom severe Nerve conduction velocities are slowed, and the protein content of the CSF is usually increased to more than 100 mg/dL Possibly the latter nding is a re ection of the increased protein content of the serum in the hypothyroid state The subjective improvement and complete or near-complete reversibility of neuropathic signs following treatment with thyroid hormones provides convincing evidence of a hypothyroid etiology In biopsies of nerve, an edematous protein in ltration of the endoneurium and perineurium, a kind of metachromatic mucoid material, has been seen Dyck and Lambert (who should be credited for drawing attention to this neuropathy) noted segmental demyelination in teased ber preparations In electron-microscopic sections, a slight increase in glycogen, acid mucopolysaccharides, and aggregates of glycogen and cytoplasmic laminar bodies in Schwann cells have been observed by others Polyneuropathy of sensorimotor type has also been observed in association with a syndrome of chronic lymphocytic thyroiditis and alopecia (Hart et al) Chronic Mild Sensory Polyneuropathy of the Elderly (Burning Feet Syndrome) All neurologists have observed numerous cases of a relatively nonprogressive sensory polyneuropathy in elderly patients Tingling paresthesias of feet and lower legs, sensory loss, and absent ankle re exes are the usual ndings The hands may be mildly affected but leg weakness and imbalance are absent or tri ing A painful variety is also known (see later) Laboratory studies may be normal or reveal minimally altered sensory conduction, but the evaluation has often not been extensive, and therefore it is dif cult to say if Sjogren syndrome, glucose intolerance, or another underlying disease is responsible Usually this chronic condition progresses little The most common variant of this form of chronic sensory polyneuropathy is one that affects mainly elderly women, who.

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Table 46-5 Causes of painful sensory neuropathy (Adapted from Mendell and Sahenk) Common Idiopathic in the elderly Diabetes Vasculitis Residue of Guillain-Barre syndrome Renal failure Connective tissue disease, especially Sjogren disease Human immune de ciency virus Less Common Amyloidosis, familial and primary Paraneoplastic Sarcoidosis Toxic neuropathy, esp arsenic poisoning Fabry disease Perineuritis

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