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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.4.384 on 1 April 1985. Downloaded from Journal of , Neurosurgery, and Psychiatry 1985;48:384-389 Distal sensory action potentials were Letters absent in median and ulnar nerves. EMG of biceps, abductor pollicis brevis and first Painful arm and moving fingers interosseous muscle showed signs of active and chronic denervation. At rest there Sir: A syndrome of painful legs and moving were spontaneous discharges of motor unit toes has been known for many years.' We action potentials in interosseous muscles, report a patient with a painful arm and occuring in pseudorhythmical bursts, with a moving fingers due to a lesion of the mean frequency of one burst per second. brachial plexus. These bursts had a voltage of 200-800 ,uV In April 1982, at the age of 54 years, our and they lasted up to 600 ms. patient discovered a breast tumour on the This patient is suffering from a painful left side. At surgical exploration an arm and moving fingers, caused by brachial adenocarcinoma was found and one of the plexus lesion due to radiotherapy. This axillar lymph nodes showed metastasis. Six sensorimotor disorder resembles the syn- months after irradiation was started the drome of painful legs and moving toes.' patient noted numbness and paraesthesias Most authors suggest a peripheral genera- in the left upper arm, gradually extending tion of this syndrome;2-4 a persistent to the distal part of the arm. In November abnormality of the central 1982 she felt a continuous deep dull pain in may subsquently develop.5 Lance6 sup- her left arm. Sensation was clearly poses that the origin of the disorder is in diminished. In the spring of 1983 she the and . noticed involuntary movements of the Our patient, patients with pseudo- fingers of her left hand. Muscular strength athetosis due to sensory polyneuropathy was diminished especially in the hand. On and patients with painful legs and moving *I I admission to our department, induration toes have two things in common: (1) the was found in the supraclavicular and axillar more or less continuous involuntary region, with cutaneous changes due to movements are associated with a dysfunc- Protected by copyright. irradiation. There were no enlarged lymph tion of the peripheral sensory nervous sys- nodes. Spine function was good. Examina- tem and (2) the absence of evidence of a tion revealed a brachial plexus lesion with disorder, which is atrophy of the interosseous muscles. The normally the cause of similar athetoid left arm was generally weak. Sensory loss movements. Wilson" and Denny-Brown9 was indicative of a lesion of the medial assumed that spontaneous involuntary cord. There were no indications of tumour movements like athetosis are nothing else sorimotor interactions as in athetosis. metastasis; blood examination, CSF, cervi- than a succession of cortical reflexes (Wil- Because these movements are related to a cal myelography and cervical CT scan were son) or a conflict between tonic types of sensation of pain or paraesthesias, group II normal. automatic patterned reflex responses to and group III afferent fibres are probably The fingers of the left hand showed con- contact stimulation (Denny-Brown). How- the major contributors. Strong evidence in tinuous involuntary movements at the ever, our patient, patients with painful legs favour of this supraspinal hypothesis is metacarpophalangeal joints with extension and moving toes and patients with given by Marsden:7 thermocoagulation of at the interphalangeal joints. These con- pseudo-athetosis due to sensory nucleus ventralis intermedius abolished the tinuous movements consisted of flexion polyneuropathy all demonstrate that simi- movements and reduced the pain in a and extension combined with adduction lar movements can also be evoked by patient with a comparable sensorimotor

and abduction. Each finger moved inde- abnormal impulses from the peripheral disorder (case 1). http://jnnp.bmj.com/ pendently of the other fingers in a more or nervous system. These abnormal impulses Segmental cutaneomuscular reflex- less identical rhythm, similar to athetosis disturb the normal functional relationship es" 19 20 or segmental bombardment of t or pseudo-athetosis due to sensory between afferent information and motor motoneurons may of course be held polyneuropathy. The movements were control.'° " responsible for this , unlike those that originate in motoneurons The influence of afferent information on but considering the more elaborate type of (, ). Sometimes athetotic movements is well known.9 12 finger movements, in which each finger these movements were absent for a few Furthermore, cutaneomuscular reflexes are seems to move independently of the others, moments. The patient was unable to increased in athetosis.'6 1' It is suggested'6 we believe that these movements transcend

imitate the movements nor could she stop that these reflexes are related to other sen- the simpler flexion-extension reflexes of on September 28, 2021 by guest. or initiate them. sorimotor reflexes'3-5 which have a sup- spinal origin. However, Fleshman2' The electromyogram showed a markedly raspinal pathway along the lines proposed demonstrated subtle peripheral influences reduced motor conduction velocity of the by Phillips" and Tatton." We suppose that on movement. These partly cutaneous brachial plexus (CV of lateral fascicle from in our patient (and in patients with com- influences have a segmental pathway and Erb's point to axilla: 6 m/s) as well as parable syndromes) the abnormal move- exert different effects on muscle that have slowed motor conduction of median nerve ments, which look so much like athetosis, essentially identical mechanical actions. (33 m/s) and ulnar nerve (47 m/s), both are caused by a peripheral sensory distur- Therefore, a spinal pathway of the move- nerves showing normal distal latencies. bance of the same supraspinal sen- ment discussed cannot be ruled out on the 384 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.4.384 on 1 April 1985. Downloaded from

Letters 385 ground of the elaborate and athetoid 16 Horstink MWIM. De Cutaneomusculaire reflexes were absent. Sensation and coor- phenomenology alone. reflexen. Een onderzoek aan de hand van dination were normal. WIM VERHAGEN EMG-registratie van de flexiereflex. (with a examination and radiographs of the skull MWIM HORSTINK summary in English). Thesis, University of and spine were normal. Electromyographic Dept Neurology Nijmegen, 1982. 7 Duensing F. Zur Pathologie der exteroceptiven examination of the right biceps brachii, first SLH NOTERMANS Reflexe des Menschen. J Nerv Ment Dis dorsal interossei, and rectus femoris Dept Clinical Neurophysiology, 1952; 116:973-87. showed potentials, positive St Radboud Hosptial, 18 Phillips CG. Motor apparatus of the baboon's sharp waves, an increase in potential amp- University of Nijmegen, hand. Proc Soc Biol 1969; 173:141-74. litudes and polyphasic potentials with a The Netherlands "Eccles RM, Lundberg A. Synaptic actions in decrease in the maximum interference pat- motoneurones by afferents which may evoke tern. Pulmonary function tests showed that References the flexion reflex. Arch Ital Biol his vital capacity was 49%. A diagnosis of 1959;97: 199-221. 'Spillane JD, Nathan PW, Kelly RE, Marsden 20 Stuart DG, Binder MD, Botterman BR. Fea- amyotrophic lateral sclerosis was made. CD. Painful legs and moving toes. Brain tures of segmental motor control revealed in The patient became almost aphagic and 1971;94:541-56. single-unit recordings during natural move- apnoeic by June 1982 and required the use 2 Nathan PW. Painful legs and moving toes: evi- ments. In: Talbott RE, Humphrey DR, eds. of a feeding tube and a respirator. Muscle dence on the site of the lesion. J Neurol Posture and Movement. New York. Raven weakness also progressed rapidly. Neurosurg Psychiatry 1978;41:934-9. Press, 1979:281-94. Neurological examination in April 1983 3Barrett RE, Singh N, Fahn S. The syndrome of 21 Fleshman JW, Lev-Tov A, Burke RE. showed that the patient was alert and painful legs and moving toes. Neurology Peripheral and central control of Flexor (NY) 1981;31:(2):79. orientated but was totally aphagic, Digitorum Longus and Flexor Hallucis Lon- aphonic, dyspnoeic and quadriplegic in 4Montagna P, Cirignotta F, Sacquegna T, Mar- gus motoneurons: the synaptic basis of func- tinelli P, Ambrosetta G, Lugaresi E. "Pain- tional diversity. Exp Brain Res extension. Supranuclear ophthalmoplegia ful legs and moving toes": associated with 1984;54: 133-49. and facial were noted. Forced cry- polyneuropathy. J Neurol Neurosurg ing was frequently seen. Deep tendon Psychiatry 1983;46:399-403. Accepted 1 September 1984 reflexes were symmetrically depressed but ' Schott GD. "Painful legs and moving toes": the sustained jaw clonus and head- the role of trauma. J Neurol Neurosurg retraction reflex were elicited. Tactile

Psychiatry 1981;44:344-6. Protected by copyright. 6Lance JW, Andrews C. Dysaesthesia- Ihe arm-mouth reflex in a patient with stimulation of the tongue resulted in reflex : a syndrome of painful legs and amyotrohic lateral scderods masticatory movement. At this clinical moving toes. Proc Austr Assoc Neurol state, the arm-mouth reflex was observed 1973;9:87-90. Sir: Numerous pathological or primitive and consisted of a wide opening of the Marsden CD, Obeso JA, Traub MM, Rothwell reflexes may appear in a variety of diseases mouth upon passive flexion of the forearm JC, Kranz H, La Cruz F. Muscle of the central nervous system and could only be elicited by flexion of associated with Sudeck's atrophy after including injury. Br Med J 1984;288: 173-6. amyotrophic lateral sclerosis. We have either forearm. The response movement 'Wilson K. Disorders of motility and of muscle observed reflex opening of the mouth upon was restricted to the perioral musculature tone, with special reference to the corpus passive flexion of the forearm in a patient and was constantly elicited by repetitive . I. The voluntary motor system in with an advanced state of amyotrophic lat- stimulation. The appearance, latency and striatal disease. In: Modem Problems in eral sclerosis. To our knowledge, this duration of the response were absolutely Neurology. New York. William Wood, 1928. reflex, which we have called arm-mouth dependent upon the strength of the 9Denny-Brown D. The Basal Ganglia and their reflex, has never been described previ- stimulus (fig): faster and greater flexion of Relation to Disorders ofMovement. London. ously. the forearm resulted in more prolonged Oxford University Press, 1962. "° Adams JA. Feedback theory of how joint A 57-year-old Japanese man noticed and wider opening of the mouth at a shor- receptors regulate the timing and positioning wasting and weakness of both hands in ter latency, whereas slower flexion of the of a limb. Psychol Rev 1977;84:504-23. April 1981. During the next nine months forearm- elicited the response less often. "Tatton WG, Bruce IC. Comment: a schema for progressive weakness of lower limbs and Flexions and extensions of other joints and http://jnnp.bmj.com/ the interactions between motor programs difficulty in swallowing and speaking sensory stimuli including pain and tendon and sensory input. Can J Physiol Pharmacol developed. The patient s history was tap of the triceps brachii did not elicit open- 1981;59:691-9. otherwise normal. He was admitted to hos- ing of the mouth. 12 motor Twitchell TE. On the deficit in congeni- pital on 11 January 1982. Findings on gen- tal bilateral athetosis. J Ment Nerv Dis The reflex opening of the mouth by the 1959; 129:105-32. eral physical examination were normal as passive flexion of the forearm described '3 Marsden CD, Merton-PA, Morton HB. Servo was his mental status. Bilateral weakness here can be classified as a primitive brain- action and stretch reflex in human muscle was present mildly in facial muscles and stem reflex for the following reasons. and its apparent dependence on peripheral moderately in bulbar muscles with dys- Firstly, passive flexion of the forearm was sensation. J Physiol 1971;216:21P-23P. phagia and dysphonia. Atrophy and fas- the only stimulus that resulted in a on September 28, 2021 by guest. "Conrad B, Aschoff JC. Effects of voluntary ciculations in the tongue were observed. stereotyped response of the perioral mus- isometric and isotonic activity on late trans- Weakness was also present mildly in neck culature at distinct latent periods following cortical reflex components in normal sub- flexors, moderately in all limb girdle and jects and hemiparetic patients. Electroence- the stimulus. Secondly, the appearance, phalogr Clin Neurophysiol 1977;42: 107-16. proximal limb muscles and severely in latency and duration of the response were Jenner JR, Stephens JA. Cutaneous reflex bilateral hand muscles. Deep tendon dependent upon the strength of the responses and their central nervous path- reflexes were symmetrically hyperactive in stimulus. These physiological properties ways studied in man. J Physiol (Lond) association with Babinski' s reflex. Jaw jerk are in accordance with those of primitive 1982;333:405-19. was exaggerated and superficial abdominal brainstem reflexes.' 3 It has been demons-