The Stiff-Man Syndrome: Report of a Case Bzl FLETCHER S. STUART,MARGARET HENRYAND HOWARD L. HOLLEY An additional case of the stiff-man syndrome manifested by extremely painful, fluctuating muscular spasm and rigidity is reported. Eighteen cases previously reported in the literature are reviewed, and the accumulated indications for a metabolic defect and/or myopathy are presented. Es reportate un caso additional del siappellate syndrome de homine rigide ( = “stiff-man syndrome”), manifestate per extrememente dolorose e fluctuante spasmos e rigiditate muscular. Dece-octo casos previemente reportate in le litteratura es revistate, e le accumulate evidentia pro un defect0 metabolic e/o myopathia es presentate. T HE STIFF-MAN SYNDROME of progressive fluctuating muscular rigidity and spasm was first described and named by Moersch and Woltman’ (1956) at the Mayo Clinic. Subsequently, cases have been reported by Bruckner,2 Asher,:’ and Price,4 and an additional case has been mentioned by O’Connor.:’There seems little doubt that the present case represents the same condition described by Moersch and Woltman. CASEREPORT The patient,’) ii 42 year old white male cattleman, complained of pain, stiffness and rigidity of the niuscles of the upper arms and hips, which had been fluctuating and progressive over the previous two years. The onset of his illness was in October 1957, 22 months prior to admission. At that time, while on a temporary construction job, he slipped and injured his lower back. Following the injury, he had sharp pains well localized in the lnnibosacral region. He was placed in traction with some relief and was subsequently operated on for a ruptured intervertebral disc. Shortly after discharge following surgery ( approxiniately one inonth after his injury) he noted gradual onset of pain in both shoulders. The pain was localized in the deltoid muscles and was constant, aching and squeezing in character. Associated with this was stiffness and hardness of the deltoid muscles, which was brought on with any movement of the arms involving use of these muscles. When stiffness and induration were present, the pain was bTeatly increased. Approximately two weeks after onset of symptoms in the deltoids, similar involvement was noted in the gInteal muscles bilaterally. These complaints have persisted with fluctuating intensity and progressive severity. Since August 1958, the patient has had intermittent pain in or around the right elbow with some swelling (not observed by a physician), but no redness or heat. During these episodes he has had a tendency to drop even light objects, he states, because of pain. In October 1958, he had an attack beginning with cramping pain in his right foot and pain and stiffness in the right calf and thigh. One day later he noted a severely pruritic From the Section oil Rheumntk Diseuses, Department of Medicine, Medical College of Alabama, Birminghum, A h . Supported by the John R. Irby Fund for the Study of Arthritis, and the Natioml Institute of Arthritis and I\f etuhlic Diseuses, National Institutes of’ Health, Bethesda, iMd. 4Acknowledgeinent ia made to Dr. William Tucker, Mobile, Alabama, Dr. H. L. Elmorc, Linden, Ala. and Dr. Bushnell Smith, of the National Institute of Neurological Diseases and Blindness, Bethesda, hld., for their assistanoe in the care of this patient. 229 230 STUART, HENRY AND HOLLEY papulo-vesicular rash which first appeared above the right lateral malleolu5 and quickly spread to the lateral aspect of the lower right leg. Three days later the rash spread over the lateral aspect of the right thigh. The rash clear’ed spontaneously after ten days, but pain and stiffness of the right leg persisted. During this time the patient first noted a feeling of tightness in the chest, accompanied by a dry cough and shortness of breath not increased by exercise, but relieved momentarily by deep sighing respirations. In January 1959, the chest pain became much more severe, involving the entire chest wall with a feeling of superficial constriction aggravated by deep inspiration. Subsequently, pain in the upper arms and chest occurred periodically with increasing frequency and severity, and during June and July 1959, the patient received almost daily narcotic injections for relief of this pain. Because of these complaints, the patient has had six hospital admissions. The first was in December 1957, approximately two months after appearance of his first symptoms. No records of this admission are available. The second admission was in January 1958. At that time skin and muscle biopsy from the left deltoid muscle suggested dermatomyositis. He was admitted to University Hospital, Birmingham, in October 1958, for further diagnostic studies. Physical examination was remarkable in that the deltoid and gluteal muscles were in a state of sustained contraction causing them to feel “rock-like” on palpation. Stimulation of these muscles caused pain and even more firm contraction. Neurologic examination was normal except for the contraction of the deltoid and gluteal muscles. No sensory deficits were detected. The total white blood count was 12,500 with a normal differential and a corrected sedimentation rate of 28 mm. per hour. Urinalysis was normal, and the serologic test for syphilis was negative. Blood chemical stiidies included a blood urea nitrogen of 17 mg. per cent, a fasting blood sugar of 66 mg. per cent, calcium 9.6 mg. per cent, phosphate 3.2 mg. per cent, and a normal alkaline phosphatase value. The x-rays of the chest were normaI. An electrocardiogram showed nonspecific ST changes. He was admitted to University Hospital again in December 1958. Physical examination revealed marked induration of the deltoid and gluteal muscles and firmness of the pectoral muscles. A review of the original biopsy was made and a report of nonspecific changes was rendered. These changes included some edema and a moderate degree of infiltration around small blood vessels and pilosebaceous glands and occasional sweat glands composed of largely mononuclear cells together with some eosinophils. The perivasculnr infiltration described in the superficial dermis was also seen at the inner edge of the dermis, as well as in the panniculus. The portions of muscle were normal except for dense eosinophilic staining of som’e individual muscle fibers. No significant myositis or inflammation of blood vessels was noted. The patient was subsequently hospitalized at the National Institute of Neurological Diseases and Blindness, Bethesda, Maryland. At this institution the physical examination revealed prominence and extreme hardness of the sternocleidomastoid, deltoid and gluteal muscles, which, on movement, were extremely painful. There was no associated weakness, but there was a transient sensory loss over these muscles. Hematologic studies, including a complete blood count, platelet counts, blood indices, L.E. cell preparations and total eosinophil counts were normal except for a white blood count of 11,000 and corrected sedim’entation rate of 17 mm. per hour. Fasting blood sugar, blood urea nitrogen, acid and alkaline phosphatase, total protein and albnniin/globulin ratio, cholesterol, bromsulphalein determination, serum electrolytes, C-reactive protein, bentonite flocculation, and antistreptolysin 0 titer were all within normal limits. During an acute exacerbation a basal metabolic rate of plus 35 per cent was recorded, but subsequent values of plus’ 1 per cent and minus 3 per cent were obtained. The protein-bound iodine was 8.5 micrograms per cent and radioactive iodine uptake was 16 per cent. The twenty-four hour urine creatine was 137 mg. and creatinine 1,351 mg., with a total urine volume of 2,400 ml. X-rays of the skull, spinal column, shoulders, and pelvis were not remarkable. Barium enema. intravenous pyelogrrun, upper gastrointestinal series, proctoscopy, and electrocardiogram were normal. Electromyogram revealed clear decrease in action potential duration (to 40 per STIFF-MAN SYNDROME: CASE REPORT 231 cent of normal ) consistent with myopathy. Injection of tubocurarine chloride produced some softening of the deltoids with some relief from pain. Muscle biopsy was not diagnostic. He was discharged with a diagnosis of myositis (rheumatoid fibrositis) of the deltoid and gluteal muscles. He was last admitted to University Hospital in August, 1959, for possible trial on Tubadil as suggested by the National Institutes of Health. Again, there was slight elevation of the white blood count and corrected sedimentation rate. A three-hour glucose tolerance test showed a diabetic type curve with glucosuria. Concomitant serum phosphorus determinations did not vary except for an 0.9 mg. per cent fall in two hours. The serum calcium was normal. The patient was most unhappy because of refusal to give him narcotics and left the hospital after 36 hours against medical advice. There was no history of preceding infectious illness, chills or fever, or exposure to heavy metals or other toxic substances. There was no history of tetanus immunization. His past history was remarkable only in that h e had poliomyelitis in childhood; this left him with a small but functionally adequate left leg. The family history was not significant. DISCUSSION From a total of 19 cases to date there has been no evidence of the etiology and pathologic physiology in this condition. Because of the fluctuating intensity of symptoms and the occasional presence of reducing substances in the urine (4 of 14 cases), Moersch and Woltmanl suggsted that there might be a metabolic basis for this malady. These two conditions were evident in our patient and in the two patients of Ashers and Price.4 In our patient glucosuriii was noted only during a glucose tolerance test. In addition, one patient from the Mayo series’ was found to be diabetic after extensive follow-up, and our patient exhibited a diabetic type curve on a glucose tolerance test. In the case presented by Price, there was an abnormal inorganic phosphate response to a carbohydrate load. Following administration of glucose, glucose and insulin or adrenalin, he found a consistent rise in the level of serum inorganic phosphate. In our case there was a slight fall in serum phosphorus following administration of glucose as would be expected with a diabetic-type glucose tolerance curve. Electromyographic studies were performed on five patients in the Mayo series,l on the patients of Price4 and O’Connor? and on our patient; all indicated prolonged volitional contraction of muscle. In our case, myopathy also was suggested by a decrease in action potential duration. Necropsy findings in Asher’s3 case suggested myopathy, but muscle biopsies in two cases from the Mayo series and in four subsequent cases have shown essentially normal muscle. Treatment of this condition including nonnarcotic analgesics, tranquilizers, antihistaminics, muscle relaxants and narcotics has been essentially ineffective in our patient as in those previously reported. Bruckner2 reported that Flexin was of value in preventing severe spasms but that it did not relieve the sustained muscle contraction. D-tubocurarine was shown to relieve the muscle pain and induration in our patient but was not tried in a slow release preparation. SUMMARY Another case of the stiff-man syndrome is described. The indications of a metabolic abnormality and/or myopathy are reviewed. 2-32 STUART, HENRY AND HOLLEY REFERENCES 1. h4oersch, F. P., and Woltman, H. \V.: 1957. Progressive fluctuating muscular rigid3. Asher, R. A.: A woman with the stiff-man ity and spasm (“stiff-man” syndrome) : Report of a case and some observations in 13 other cases. ProcStaff Meet.Mayo Clinic 31:421, 1956. 2. Bruckner, W. J.: “Stiff-man” syndroineprogressive fluctuating miisciilar rigidity and spasm. California Med. 87:336, syndrome. Brit.M.1. 1:265, 1958. 4. Price, T. M. L., and Allot, E. N.: The stiff-man syndrome. Preliminary report of a case. E3rit.M.J. 1:682, 1958. 5. O’Connor, D. C. J.: Stiff-man syndrome. l3rit.M.J. 1:645, 1958. Fletcher S . Stuart, AS., Student Fellow, Section on Rheumatic Diseases, Department of Medicine, The Medical Collegc of Alabama, Birmingham, Ala. Margaret Henry, M.D., Trainee of the National Institute of Arthritis and Metabolic Diseases, Department of Medicine, The Medical College of Alabama, Birmingham, Ala. Howard L. Holley, M.D., Professor of Medicine, Chief of Section on Rheumatic Diseases, The Medical College of Alabama, Birmingham, Ala.