18
SOCIETI~ D ' E.E.G. ET DE NEUROPHYSIOLOGIE DE LANGUE FRANqAISE
Permanent contractore of the striated muscle : report of a peculiar case. F . SAVOLDI~
A.
MOGLIA, M . POLONI, G . BONO, R . SCELSI * a n d A . ARRIGO.
Clinica delle Malattie Nervose e Mentali dell'Universith di Pavia (Direttore : P~ P. PINELLI) et Isrituto di Anatomia Patologica dell'Univetsitgl di Pavia (Direttore : pr L. MoscA), Italia.
A m o n g the clinical criteria that m u s t be satisfied in order to diagnose > (SMS), the clinical entity isolated a n d described by MOERSH a n d WOLTMAN (1956), it a p p e a r s necessary to consider the absence o f neurological signs associated with the specific m u s c u l a r contracture. SIGWALO a n d GUILLEMINAULT (1971), in a review of the s y n d r o m e s o f p e r m a n e n t contracture, have recognized as > o f S M S all those cases characterizes either by the presence of atypical elements or by an incomplete s y m p t o m a t i c picture. W e wish to present here a case o f p e r m a n e n t contracture with s o m e peculiar features.
CLINICAL OBSERVATION. This case concerns a male patient 54 years old who presented stiffness in the lower limbs, impairment in walking and an acute retention of urine which necessitated hospitalisations. During his stay at the hospital the rigidity of the lower limbs was noted and diagnosed as osteoarthritis affecting knees and hips bilaterally. A week before admittance, the patient had become aware of the impossibility to look towards the left, while the lower limb disturbance worsened. U p o n entrance the most relevant symptom was the contracture of the lower limbs : this was asymmetrical, prevailed in the right limbs and extended both proximally and distally to the agonistic and antagonistic muscles, its intensity varied in time but was aggravated by voluntary or passive movement, by skin stimulation and by emotional stress. Sometimes the patient experienced intense paroxysmal painful spasms of brief duration. Walking was not possible without bilateral supports. The contracture disappeared during sleep, No objective clinical symptoms were found in the upper limbs (but we shall see later the E M G record was abnormal). Neuro-ophthalmological examination showed no impairment of vertical movements or convergence, but a paralysis of conjugate movements towards the left and a large, regular, slow, inexhaustible right lateral nystagmus. The EEG showed a dominant rhythm of 7,5 c/s. The vestibular investigation showed a suffering of the central vestibular fibres, in mesencephalic portion. A retrograde left brachial arteriogram showed no abnormality. Cystometry demonstrated only the absence of the stimulation to start miction. The remaining neurological examination and the usual laboratory blood and urine tests were normal. ELECTROMYOGRAPHIC STUDY. A t rest a m a r k e d discharge o f M . U . potentials was recorded f r o m the m u s c l e s e x a m i n e d b u t it was n o t possible to discord the presence of fibrillation potentials. Polyphasic M . U . potentials of higher d u r a t i o n attained a percentage o f 16-19 p. 100, their amplitude was n o r m a l or a b o v e average. W i t h the m a x i m a l voluntary effort, a discontinued interference pattern was obtained o f n o r m a l m e a n a m p l i t u d e with m a x i m a l sporadic points u p to 4-5 mV. M a x i m a l m o t o r c o n d u c t i o n velocity was within n o r m a l limits for the different nerves examined. Irregular M response with lower a m p l i t u d e was obtained. By E r b ' s point s t i m u l a t i o n a n M response
Tirds dpart : F. SAVOLDI,via Palestro, 3, 27 100 Pavia, Italia.
SEANCE DU 5 JUIN 1974
19
20
SOCII~TI~ D'E.E.G. ET DE NEUROPHYSIOLOGIE DE LANGUE FRAN~AISE
of increased latence from deltoid muscles was recorded. In the presence of disturbances by muscular contraction it was impossible to carry out the determination for sensitive conduction velocity. Strength duration curve of the left median nerve and right SPE indicated a partial degeneration. In conclusion there were modest signs of peripheral neurogenic suffering. SPECIAL EXAMINATIONSWITH SIMULTANEOUSDERIVATIONS. a) Spontaneous record. The patient laid in a supine position, in a quiet environment. The presence of irregular asynchronous activity more or less rich, sometimes diffused to all the derivations, was evident. b) Voluntary movements (closure of the fist (fig. 1) controlateral to the nmscle examined or strong stimulus on the left foot (fig. 2) determined an increase and diffusion of the activity to non synergic muscles, which persisted for a few seconds after the cessation of the movement. c) The voluntary extension of the leg upon an acoustic signal (CARLS66 and coll., 1973) determined the appearance of marked activity interesting simultaneously either agonistic or antagonistic muscles. d) The passive slow flexion of the foot determined a marked increase of the activity either on the elongated muscle or the shortened one. The same thing was observed in the rapid passive flexions. The result was scarcely influenced by novocain injection around the nerve at the fossa poplitea.
FIG. 3. - - Silent period evoked by a supramaximal stimulus of the right sciatic nerve applied at the popliteal cavity. e) The duration of the silent period of the gastrocnemius (fig. 3) and of the brachial biceps was found to be about 100 msec., when obtained by stimulation of the motor nerve or by the deep tendon percussion and was reduced during the voluntary isometrical contraction although it was not observed during the maximal shortening of the muscle.
SEANCE DU 5 JUIN 1974
21
PHARMACOLOGICAL STUDY.
The same tests were done after administration of different substances : diphenglhydantoin (given for 7 days 300 rag/day per os) and carbamazepin (given for 8 days 600 mg/day p e r os) gave no substantial results. Both Diazepam and Lioresal 10 mg i. v. gave similar results. After treatment with Diazepam (20 nag/day per os and or by intramuscular injections) there was a marked reduction of spontaneous activity and of its increase to the various stimulations. Ro6-9098 (30 mg/day per os for 12 weeks) resulted in almost complete remission of the symptomotology. The contraction reappeared after a suspension of therapeutic for ten days. [-tYSTOPATHOLOGICAL OBSERVATIONS.
In the paraffine sections taken of the deltoid and the femoral quadriceps muscles we observed some sparse hypotrophic muscle fiber. Only at the quadriceps muscle level, some fibers assumed the nature of angled fiber. The histochemical preparations showed an homogeneous distribution of the two fibrocellular types. At the level of the femoral quadriceps, the hypotrophic phenomena seemed to mainly interest the fibers of type II. DISCUSSION.
Even if for the clinical and electromyographical characteristics of the permanent contracture this case can rightly be classified as SMS ; the presence of a nystagmus, a paralysis of conjugate eye movements and functional disturbances of the bladder raise physiopathological, topodiagnostic and nosographic problems. Two observations seem to be similar to the case we described : in the case of KASPEREKand ZEBROWSKt (1971) the contracture, the nystagmus and the oculomotor paralysis were associated with sensitive disturbances in the four limbs and absence of deep tendon reflexes ; besides the contracture affected largely only the superior limbs. LUERMrrTE and coll. (1973) reported a case where the upper limbs, which were the site of a peripheral neurogenic syndrome, were totally saved from disturbance of tone while the ophthalmoplegia was almost complete and there were disturbances of the sense of position of the extremities. In both cases the cerebrospinal fluid inflammatory signs were shown at least in certain phases of the disease, and the p o s t mortem examination showed an encephalomyelitis of probable viral origin. We wish to emphasize some peculiar features of the case described : the absence of sensitive disturbances and of alterations of the cerebrospinal fluid, the bladder disturbances and the effectiveness of Diazepam even in relatively small doses. Nervertheless our case could, seems to us, to be rightly included in the new entity distinguished by LHERMITTE and coll. (1973) if one keeps in mind the relatively precocious state at which the patient was examined. He is now being treated with a new Benzodiazepine (Ro6-9098 ; 20 m g / d a y p e r os) ; in addition due to a possible viral process, Amantidine was associated (Mantadan 3 g/day per os). The symptoms are actually under control, The patient is autonomous and can move without difficulty. The oculomotor paralysis is slightly improved.
22
SOCIt~TI~ D ' E.E.G. ET DE NEUROPHYSIOLOGIE DE LANGUE FRAN~AISE BIBLIOGRAPHY
CARLS66 (S.), FOHLIN (L.) and SKO~LLrND (G.). Studies of Co-Contraction of Knee Muscles. In :
SOCIETI~ D ' E.E.G. ET DE NEUROPHYSIOLOGIE DE LANGUE FRANqAISE
Permanent contractore of the striated muscle : report of a peculiar case. F . SAVOLDI~
A.
MOGLIA, M . POLONI, G . BONO, R . SCELSI * a n d A . ARRIGO.
Clinica delle Malattie Nervose e Mentali dell'Universith di Pavia (Direttore : P~ P. PINELLI) et Isrituto di Anatomia Patologica dell'Univetsitgl di Pavia (Direttore : pr L. MoscA), Italia.
A m o n g the clinical criteria that m u s t be satisfied in order to diagnose > (SMS), the clinical entity isolated a n d described by MOERSH a n d WOLTMAN (1956), it a p p e a r s necessary to consider the absence o f neurological signs associated with the specific m u s c u l a r contracture. SIGWALO a n d GUILLEMINAULT (1971), in a review of the s y n d r o m e s o f p e r m a n e n t contracture, have recognized as > o f S M S all those cases characterizes either by the presence of atypical elements or by an incomplete s y m p t o m a t i c picture. W e wish to present here a case o f p e r m a n e n t contracture with s o m e peculiar features.
CLINICAL OBSERVATION. This case concerns a male patient 54 years old who presented stiffness in the lower limbs, impairment in walking and an acute retention of urine which necessitated hospitalisations. During his stay at the hospital the rigidity of the lower limbs was noted and diagnosed as osteoarthritis affecting knees and hips bilaterally. A week before admittance, the patient had become aware of the impossibility to look towards the left, while the lower limb disturbance worsened. U p o n entrance the most relevant symptom was the contracture of the lower limbs : this was asymmetrical, prevailed in the right limbs and extended both proximally and distally to the agonistic and antagonistic muscles, its intensity varied in time but was aggravated by voluntary or passive movement, by skin stimulation and by emotional stress. Sometimes the patient experienced intense paroxysmal painful spasms of brief duration. Walking was not possible without bilateral supports. The contracture disappeared during sleep, No objective clinical symptoms were found in the upper limbs (but we shall see later the E M G record was abnormal). Neuro-ophthalmological examination showed no impairment of vertical movements or convergence, but a paralysis of conjugate movements towards the left and a large, regular, slow, inexhaustible right lateral nystagmus. The EEG showed a dominant rhythm of 7,5 c/s. The vestibular investigation showed a suffering of the central vestibular fibres, in mesencephalic portion. A retrograde left brachial arteriogram showed no abnormality. Cystometry demonstrated only the absence of the stimulation to start miction. The remaining neurological examination and the usual laboratory blood and urine tests were normal. ELECTROMYOGRAPHIC STUDY. A t rest a m a r k e d discharge o f M . U . potentials was recorded f r o m the m u s c l e s e x a m i n e d b u t it was n o t possible to discord the presence of fibrillation potentials. Polyphasic M . U . potentials of higher d u r a t i o n attained a percentage o f 16-19 p. 100, their amplitude was n o r m a l or a b o v e average. W i t h the m a x i m a l voluntary effort, a discontinued interference pattern was obtained o f n o r m a l m e a n a m p l i t u d e with m a x i m a l sporadic points u p to 4-5 mV. M a x i m a l m o t o r c o n d u c t i o n velocity was within n o r m a l limits for the different nerves examined. Irregular M response with lower a m p l i t u d e was obtained. By E r b ' s point s t i m u l a t i o n a n M response
Tirds dpart : F. SAVOLDI,via Palestro, 3, 27 100 Pavia, Italia.
SEANCE DU 5 JUIN 1974
19
20
SOCII~TI~ D'E.E.G. ET DE NEUROPHYSIOLOGIE DE LANGUE FRAN~AISE
of increased latence from deltoid muscles was recorded. In the presence of disturbances by muscular contraction it was impossible to carry out the determination for sensitive conduction velocity. Strength duration curve of the left median nerve and right SPE indicated a partial degeneration. In conclusion there were modest signs of peripheral neurogenic suffering. SPECIAL EXAMINATIONSWITH SIMULTANEOUSDERIVATIONS. a) Spontaneous record. The patient laid in a supine position, in a quiet environment. The presence of irregular asynchronous activity more or less rich, sometimes diffused to all the derivations, was evident. b) Voluntary movements (closure of the fist (fig. 1) controlateral to the nmscle examined or strong stimulus on the left foot (fig. 2) determined an increase and diffusion of the activity to non synergic muscles, which persisted for a few seconds after the cessation of the movement. c) The voluntary extension of the leg upon an acoustic signal (CARLS66 and coll., 1973) determined the appearance of marked activity interesting simultaneously either agonistic or antagonistic muscles. d) The passive slow flexion of the foot determined a marked increase of the activity either on the elongated muscle or the shortened one. The same thing was observed in the rapid passive flexions. The result was scarcely influenced by novocain injection around the nerve at the fossa poplitea.
FIG. 3. - - Silent period evoked by a supramaximal stimulus of the right sciatic nerve applied at the popliteal cavity. e) The duration of the silent period of the gastrocnemius (fig. 3) and of the brachial biceps was found to be about 100 msec., when obtained by stimulation of the motor nerve or by the deep tendon percussion and was reduced during the voluntary isometrical contraction although it was not observed during the maximal shortening of the muscle.
SEANCE DU 5 JUIN 1974
21
PHARMACOLOGICAL STUDY.
The same tests were done after administration of different substances : diphenglhydantoin (given for 7 days 300 rag/day per os) and carbamazepin (given for 8 days 600 mg/day p e r os) gave no substantial results. Both Diazepam and Lioresal 10 mg i. v. gave similar results. After treatment with Diazepam (20 nag/day per os and or by intramuscular injections) there was a marked reduction of spontaneous activity and of its increase to the various stimulations. Ro6-9098 (30 mg/day per os for 12 weeks) resulted in almost complete remission of the symptomotology. The contraction reappeared after a suspension of therapeutic for ten days. [-tYSTOPATHOLOGICAL OBSERVATIONS.
In the paraffine sections taken of the deltoid and the femoral quadriceps muscles we observed some sparse hypotrophic muscle fiber. Only at the quadriceps muscle level, some fibers assumed the nature of angled fiber. The histochemical preparations showed an homogeneous distribution of the two fibrocellular types. At the level of the femoral quadriceps, the hypotrophic phenomena seemed to mainly interest the fibers of type II. DISCUSSION.
Even if for the clinical and electromyographical characteristics of the permanent contracture this case can rightly be classified as SMS ; the presence of a nystagmus, a paralysis of conjugate eye movements and functional disturbances of the bladder raise physiopathological, topodiagnostic and nosographic problems. Two observations seem to be similar to the case we described : in the case of KASPEREKand ZEBROWSKt (1971) the contracture, the nystagmus and the oculomotor paralysis were associated with sensitive disturbances in the four limbs and absence of deep tendon reflexes ; besides the contracture affected largely only the superior limbs. LUERMrrTE and coll. (1973) reported a case where the upper limbs, which were the site of a peripheral neurogenic syndrome, were totally saved from disturbance of tone while the ophthalmoplegia was almost complete and there were disturbances of the sense of position of the extremities. In both cases the cerebrospinal fluid inflammatory signs were shown at least in certain phases of the disease, and the p o s t mortem examination showed an encephalomyelitis of probable viral origin. We wish to emphasize some peculiar features of the case described : the absence of sensitive disturbances and of alterations of the cerebrospinal fluid, the bladder disturbances and the effectiveness of Diazepam even in relatively small doses. Nervertheless our case could, seems to us, to be rightly included in the new entity distinguished by LHERMITTE and coll. (1973) if one keeps in mind the relatively precocious state at which the patient was examined. He is now being treated with a new Benzodiazepine (Ro6-9098 ; 20 m g / d a y p e r os) ; in addition due to a possible viral process, Amantidine was associated (Mantadan 3 g/day per os). The symptoms are actually under control, The patient is autonomous and can move without difficulty. The oculomotor paralysis is slightly improved.
22
SOCIt~TI~ D ' E.E.G. ET DE NEUROPHYSIOLOGIE DE LANGUE FRAN~AISE BIBLIOGRAPHY
CARLS66 (S.), FOHLIN (L.) and SKO~LLrND (G.). Studies of Co-Contraction of Knee Muscles. In :
