Vasospastic dystrophy of the hand Case report JOHN L. BENDER, M . D .

Section of Neurology and Neurosurgery, Rockford School of Medicine, Rockford, Illinois

~," The author reports a patient with a history of left arm pain and wasting of the hand who had absent radial and ulnar pulses. Sympathectomy restored the arterial circulation to the hand with nearly complete return of function. KEY WORDS

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HE following reports the case of a patient who presented with an unusual form ofvasospastic dystrophy. Case Report

This 38-year-old woman had a 1-year history of intermittent left shoulder, arm, and hand pain which developed after a hysterectomy for fibroid tumors. The pain gradually worsened. One month before seeking medical attention, she developed weakness of lefthand grasp, wasting, and intermittent hand numbness. She noted no changes in the skin or nails. Temperature extremes had no apparent effect on the condition. She had had a 16-lb. weight loss following the hysterectomy and continued to have poor appetite. Past history revealed a cholecystectomy in 1965. She smoked 10 cigarettes a day, rarely consumed alcohol, and her only daily medication was Premarin. Examination. Examination revealed a mildly depressed woman whose left hand was clawed, with hyperextended metacarpophalangeal joints and moderately flexed in764

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terphalangeal joints. The hand was soft to palpation. The thenar, hypothenar, and interosseous muscles were atrophic with corresponding intrinsic hand weakness. The bulk of the distal left forearm was also diminished (Fig. 1). There was a periodic involuntary myoclonic twitching of the fingers. No indications of thoracic outlet syndrome, peripheral nerve entrapment, or cervical spine disease were found. The left biceps and brachioradialis reflexes were absent. Sensory examination was normal, and temperature and sweating capabilities of her hands were equal. Blood pressure was 100/76 in the right arm and 96/70 in the left. The left brachial pulse was normal, but the left radial and ulnar pulses were absent. All other pulses were normal. The patient was admitted to the hospital on October 15, 1972. Complete blood count, chemical screening, serological findings, sedimentation rate, protein electrophoresis, and lupus erythematosus preparation were normal. Chest and cervical spine x-ray films were normal. Electromyography of the left first dorsal interosseous, abductor pollicis J. Neurosurg. / Volume 43 / December, 1975

Vasospastic dystrophy of the hand

Ftc. 1. Photograph comparing the patient's dystrophic left hand with the normal one.

brevis, and flexor digitorum sublimis muscles revealed a decreased number of motor units, giant polyphasic potentials, and rare fibrillation potentials. Motor nerve conduction velocities were as follows: left ulnar, 48 m/sec; right ulnar, 58 m/sec; left median, 41 m/sec; right median, 53.5 m/sec. A cervical and upper thoracic myelogram was normal. Spinal fluid examination revealed no cells, protein 41 mg%, and nonreactive serological results. A femoral-left subclavian arteriogram showed that there was no arterial circulation beyond the lower forearm (Fig. 2 upper). A trial of therapy was begun using an alpha-adrenergic blocking agent, tolazoline HCI (Priscoline) 25 mg four times daily, along with hand exercises. Follow-up revealed palpable left ulnar and radial pulses, normal hand temperature, and improved extensibility of the interphalangeal joints; prior to sympathectomy, however, the left radial pulse disappeared again. Operation. A left cervical sympathectomy was performed on February 6, 1973. Through a midline posterior incision from C7 to T4, the tip of the left transverse process of T-2 was removed. The exposed T-2 sympathetic ganglion and rami communicantes were excised. Skin and muscle biopsy specimens were taken from the left thenar eminence. The cervical sympathetic and skin specimens were normal, while the muscle biopsy (Fig. 3) revealed neurogenic atrophy of the muscle fibers and no evidence of vasculitis. J. Neurosurg. / Volume 43 / December, 1975

FIG. 2. Angiograms of the left brachial artery circulation. Upper." Preoperatively, with absence of arterial feeders distal to the lower forearm. Lower: After sympathectomy.

Postoperative Course. A postoperative femoral-left brachial catheter arteriogram revealed a large ulnar and smaller radial artery emptying into the hand (Fig. 2 lower). A pulsating ulnar artery was palpable and the hand was warm. Two months postoperatively, although the appearance of the hand was unchanged, it had recovered 90% of its power and use. The hand was warm with normal sensation and free of pain. The patient has since returned to full activity without further difficulty. Discussion

The blood vessels of the hand are innervated through fibers in the ulnar and median nerves. 7 Blood-flow regulation in the hand is achieved by release of constrictor tone, while forearm control is by an active vasodilator mechanism. Reflex vasospasm can be produced by many pathological conditions. Lesions at various levels of the nervous system including the parietal lobe of the brain may lead to trophic disorders.' The mechanism works through the sympathetic nerves to constrict the arterial walls and selectively reduce blood flow; the resulting ischemia can selectively involve just the skin or the muscles. 5 Appenzeller, et al., ~ found evidence in patients with migraine of a basic generalized abnormality in the blood-vessel neural control that prevented reflex vasodilatation. This would, of course, raise 765

J. L. B e n d e r The long-term effectiveness of sympathectomy for this patient seems good so far. Kirtley, et al., 6 reported good-to-improved results in 21 of 22 patients with idiopathic Raynaud's disease without vasculitis, while seven of their nine posttraumatic sympathetic dystrophy patients had good results. None of their 76 patients seemed to have a vasospastic disorder like that of our patient. Whether she will have further vasomotor difficulty remains to be seen. Acknowledgment

We wish to thank Dr. J. H. Van Landingham, who performed the surgery on this patient. References

1. Appenzeller O: The Autonomic Nervous System. An Introduction to Basic and Clinical Concepts. New York, American Elsevier, 1970, pp 75-76 2. Appenzelter O, Davison K, Marshall J: Reflex vasomotor abnormalities in the hands of migrainous subjects. J Neurol Neurosurg FIG. 3. Photomicrograph of muscle biopsy of Psychiatry 26:447-450, 1963 the left thenar eminence illustrating muscular 3. Edwards EA: Varieties of digital ischemia and atrophy. H & E, • 125. their management. N Engl J Med 250:709-716, 1954 4. French EB, Lassers BW, Desai MG: Reflex the question of a basic, generalized bloodvasomotor responses in the hands of migrainvessel abnormality in all such disorders, but ous subjects. J Neurol Neurosurg Psychiatry French, et al., 4 were unable to confirm 30:276-278, 1967 Appenzeller's observations in similar studies. 5. Gillilan LA: Clinical Aspects of the Autonomic Nervous System. Boston, Little, Brown, 1954, p The cause of our patient's vasospastic dis165 order could fall under one of several categories according to Edwards' classifica- 6. Kirtley JA, Riddell DH, Stoney WS, et al: Cervicothoracic sympathectomy in neurovascular tion for Raynaud's phenomenon, 8 which abnormalities of the upper extremities: extends to distinguish functional (or nonthromperiences in 76 patients with 104 sympathecbotic) vascular shutdown from the thromtomies. Ann Surg 165:869-877, 1967 botic occlusive state. If the vascular shutdown 7. Kuntz A: Autonomic Nervous System, ed 4. was functional, as it seems to have been in Philadelphia, Lea & Febiger, 1953, pp 154-155 this case, then it could have been a traumatic result of her hysterectomy, or else related to a toxic agent, tobacco. The loss of the radial pulse prior to the sympathectomy could have been caused by organic obstruction, in which Address reprint requests to." John L. Bender, the functionally constricted vessel throm- M.D., 2825 Glenwood Avenue, Rockford, Illinois bosed because of sludging of blood. 61103.

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J. Neurosurg. / Volume 43 / December, 1975

Vasospastic dystrophy of the hand. Case report.

The author reports a patient with a history of left arm pain and wasting of the hand who had absent radial and ulnar pulses. Sympathectomy restored th...
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