NOTES AND NEW DEVELOPMENTS MONOMELIC MUSCLE HYPERTROPHY FOLLOWING TRANSIENT ISCHEMIC ATTACKS: A CASE REPORT Edward R. Iraacs, MD, and Paul Larson, MD _ _ ~- -~ ~- Abstract: A 39-year-old right-handed male suffered several episodes of transient right-sided weakness, sensory loss, and Broca’s aphasia at age 31. Diagnostic studies failed to demonstrate any cause for these events. Following a complete recovery, he noted the onset of stiffness and increasing muscle bulk in his right arm, which gradually restricted its use. Simultaneous recordings from contracting biceps and triceps muscles in both arms suggested that the hypertrophy was physiologically induced by an abnormal mechanism providing resistance during phasing activity. MUSCLE 6 NERVE 1242-244 1978 It is a u n i q u e experience t o receive a p a t i e n t w h o complains of a progressive increase i n b u l k and stiffness involving t h e m u s c u l a t u r e of one a r m . M u s c l e h y p e r trophy associated w i t h d y s t o n i a or athetosis has been described , a s a physiologic consequence of a central nervous system disorder,j b u t it is n o t usually a m a j o r feature of such conditions. H y p e r t r o p h y a s a p a t h o logic manifestation of diseased muscle, s u c h as myotonia congenita or m u s c u l a r dystrophy, is usually symmetrical5 and rarely confined t o o n e extremity.’ Atrophy is a m o r e c o m m o n l y observed sequel t o a cerebrovascular event. CASE REPORT The patient, a 39-year-old right-handed male, was referred to neurology service at Keesler Air Force Base Medical Center, Biloxi, Mississippi, because of progressive enlarge- 242 Monomelic Muscle Hypertrophy ment and stiffness of the muscle of his right arm. At age 91 he experienced four episodes of light headedness, right-sided weakncss, and difficulty expressing himself. These episodcs began abruptly and lasted for approximately 30 min. During onc of these episodes, right-sided hcrniparesis with hyperreflcxia, hemianesthesia, and Broca’s aphasia were documented. A residual, mild, lower-right facial weakness persisted for several days and then cleared. During this period, electroencephalogram, brain scan, left carotid arteriogram, and cerebrospinal fluid evaluation werc reported as normal. The patient returned to active duty, his assignment confined to administrative responsibilities and his major physical activity restricted to a n occasional game of golf. H e denied participation in any formal exercise program. Tbvo years later he noticed mild aching pain and stiffness in his right arm. After that, the right arm became noticeably larger than the left, and because of associated stiffness and cramping, it was finally rendered ineffective for even the simplest of chores. Even a casual handshakc would cause the arm to go into painful spasms. O n examination, there was marked hypertrophy of the musculature of the right upper arm and forearm (fig. 1). Circumferential measurements of contracted musculature at symmetrical locations were: right biceps-33 cm, left biceps29 cm; right forearm-29 cm, left forearm-26 cm; wrist-18 cm bilaterally. At rest, awake and asleep, the patient held his right arm slightly flexed, adducted and internally rotated. When strength was tested, his muscles could not be overcome, but severe cramping with painful spasms quickly ensued and the involved muscles became quite firm. Passive From Richmond Neurological Consultants, Inc , Richmond, VA (Dr. Isaacs). and Louisiana State University Medical School, New Orleans (or Larson). Read by title at the lllrd International Congress on Muscle Disease, Newcastle upon Tyne, Great Britain. September 15-21. 1974. The authors wish to thank Dr. Donald C Oxenhandler tor his referral of this patient. Address reprint requests to Dr lsaacs at Richmond Neurological Consultants, Inc., 8919 Three Chopt Rd.. Richmond, VA 23229. Received for publication November 16, 1977; revision received February 1 5 , 1978 MUSCLE & NERVE May/Jun 1978 motion was slightly limited, with lead-pipe hypertonicity and occasional cogwheeling present in the biceps, triceps, and wrist extensors as well as in the flexors, pronators, and supinators of the right upper extremity. Deep tendon reflexes were depressed in the right arm but normal elsewhere. Percussion myotonia was not present. The remainder of the neurologic examination was normal. MATERIALS AND METHODS firtinent studies included X rays of the skull, cervical spine, and both arms and shoulders; electroencephalograms; brain scan; selective four-vessel arteriography; cerebrospinal fluid evaluation; and determination of serum creatine phosphokinase and serum T-3 and T-4 levels. Electrodiagnostic studies showed normal peripheral nerve conduction velocities, and needle electrode studies of the involved muscles showed electrical silence at rest but prolonged discharges of normalappearing motor units during cramping spasms. No myotonic or bizarre high-frequency discharges were seen even after cooling with ice packs. Using a four-channel polygraph adapted from a Grass Model 6 Electroencephalograph (Quincy, MA), needle electrode recordings of the right and left triceps and biceps muscles were made simultaneously (fig. 2). Cocontraction with failure of antagonistic muscles on the right to relax was demonstrated. In addition, cocontraction of the right biceps and triceps was seen when either the left biceps or left triceps was maximally contracted. After 1% xylocaine block to the right brachial plexus, there was a dramatic relaxation of the musculature of the right arm for approximately 30 min. There was no concomitant loss of strength or sensation for position, touch, or vibration. Adequate trials with various medications, such as diphenylhydantoin, levodopa, and amantadine, failed to afford any relaxation. Partial relaxation of the patient’s right a r m was finally achieved with diazepam, but the dosage had to be increased frequently. A combination of dantrolene sodium 200 mg/day and diazepam 20 mg/day, in divided doses, provided complete sustained relaxation. After three months of this regimen, tone returned to normal and the patient was again using his right arm without difficulty or pain. The following measurements were obtained: right biceps-relaxed 27 crn, contracted 31.5 cm; left biceps-relaxed 26 cm, contracted 29.5 c m ; right forearm-relaxed 27 cm, contracted 28 cm; left forearmrelaxed 26 cm, contracted 27 cm. DISCUSSION T h e muscle hypertrophy observed in this patient was probably physiologic in that it represented true work hypertrophy. With delayed relaxation of antagonists as well as cocontraction of both agonists and antagonists, there was in effect both static and dynamic exercise against ever-increasing resistance. Both maneuvers are responsible for muscle hypertrophy,6,10 but the latter may have the greatest influence.‘j The patient’s past history of a transient left cerebral dysfunction, along with the tendency for nonvoluntary muscle contractions in the right arm,8.9 suggests that a left basal ganglion lesion is responsible for the basic abnormality in tone. Relaxation following a 1% xylocaine block suggests an increased contribution from the gamma motor system,” either as a direct reflection of a dystonic state or as an aggravating factor resulting from increased feedback from the contracted muscle. Figure 1 . Patient demonstrating marked hypertrophy of the muscles of the right arm and shoulder. Monornelic Muscle Hypertrophy MUSCLE & NERVE May/Jun 1978 243 Figure 2 Simultaneous EMG polygraph recordings of right biceps (RB). nght triceps (RT), left biceps (LB). and left triceps (LT) Tracings show the effect of voluntary contractions of (A) left biceps, (6) left triceps, (C) right biceps, and (0)right triceps The action of dantrolene2.4 appears to be mainly peripheral. Its ameliorating effect when coupled with diazepam was apparently capable of reversing a positive feedback system underlying or aggravating the patient’s condition. The failure of levodopa, amantidine, or diphenylhydantoin to successfully treat this condition suggests that neither dopominergic nor peripheral nerve influences were responsible. REFERENCES 1. Celesia GG, Andermann F, Wiglesworth FW, Kobb J P : Monomelic myopathy. Arch Neurol 17:69-77 1967. 2. Chyatte SB, Birdsong J H , Koberson DL: Dantrolene sodium in athetoid cerebral palsy. Arch Phys Med Rchabil54:365-368, 1973. 3. Denny-Brown D: Experimental studies pertaining to hypertrophy, regeneration and degeneration. Recenf Pub Assoc New Men1 Diseases 38 :147- 196, 196 1. 4. Dykes M H M : Evaluation of a muscle relaxant: dantrolene sodium (Dantriuni). J A M A 231 :862-864, 1975. 5. Gardner-Medwin D, Walton JN: A classification of the neuromuscular disorders and a note on the clinical examination of the voluntary muscles. In Walton JN (Editor): Disorders OJ Volunfary Muscle, 2nd ed. London, J & A Churchill Ltd, 1969. 6. G o d o n EE: Anatomical and biochemical adaptations of muscle 244 Monomelic Muscle Hypertrophy to excessive use in compensatory hypertrophy and increased phasic activity. Physiol Bohcmoslou 20:205-212, 1971. 7. Gordon EE, Januszko D M , Kaufman L: A critical survey of stiffman syndrome. Am J Mcd 42:582-599, 1967. 8. Green JB: An electrornyographic study of mirror movements. .Vcurulology (Minneap) 17:91-94, 1967. 9. Gregg RA, Mastellone AF, Gersten JW: Cross-exercise-a review of the literature and study utilizing electromyographic techniques. Am J Plys Mcd 36:269-289, 1957. 10. Gutmann E, Hajek I: Differential reaction of muscle to excessive use in compensatory hypertrophy and increased phasic activity. Physiol Bohcmoslou 20:205-2 12, 197 1. 11. Matthews PBC: Mammalian Muscle Rccepfors and T h e v Cenlral Acfionr. London, Edward Arnold Publishers, Ltd, 1972. MUSCLE & NERVE May/Jun 1978