Postinfectious Myoclonus
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J7ournal of Neurology, Neurosurgery, and Psychiatry 1992;55: 1089-109 1 1 089 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.11.1089 on 1 November 1992. Downloaded from SHORT REPORT "Isolated" postinfectious myoclonus K Bhatia, P D Thompson, C D Marsden Abstract abnormality on clinical examination or inves- Two cases are reported who developed tigations and the myoclonus is usually short myoclonus as the only manifestation of a lasting. Although post-infectious encephalitis post-infectious syndrome without evi- as a cause of myoclonus has been included in dence of encephalitis or the opsoclonus- lists of aetiological classifications,3 12 13 the myoclonus syndrome. Case 1 had syndrome of isolated myoclonus following generalised myoclonus following an influ- infection has not been clearly defined. In the enza-like illness, while case 2 had right past such patients have been referred to ambig- upper limb segmental myoclonus follow- uously and one report'4 included them within ing uncomplicated chicken pox. Neither a group labelled "myoclonus without seizures had any localising neurological signs or or neurological or mental deficit." We report abnormality on investigation. Both recov- two cases with myoclonus as the sole manifes- ered completely within six months of the tation of a post-infectious syndrome, and have onset. Similar cases are reviewed from the reviewed the few similar cases from the lit- literature and it is suggested that such erature. cases be called "isolated" post-infectious myoclonus. Case reports (7 Neurol Neurosurg Psychiatry 1992;55: 1089-109 1) Case 1 A 23 year old woman, presented with a 14 week history of an influenza-like illness dominated by generalised body ache, lethargy Myoclonus can be a clinical feature of many and fever. Two weeks after the onset she infectious encephalitides and was notable in developed episodic generalised jerks of the the acute and chronic phases of epidemic arms and legs, each lasting for up to 30 encephalitis lethargica.' More recently it has seconds, and occurring every two hours or so, been recognised as a pathognomonic feature of without loss of consciousness, or other epi- subacute sclerosing panencephalitis2 and leptic phenomena. She was otherwise normal Creutzfeldt-Jacob disease.2 Myoclonus has between attacks. Treatment with clonazepam also been described in acute encephalitis due improved the spasms but made her very to a variety of viral24 and non-viral agents` drowsy. When discontinued 2 weeks later, the http://jnnp.bmj.com/ which either directly invade the CNS, or by an spasms became more intense and responsive to immunological process cause an acute dis- noise, sudden movement and startle. She also seminated encephalomyelitis (also called post- developed constant twitching of her arms and infectious encephalitis). In either case, the legs making it difficult for her to use her hands, clinical picture is of an acute illness charac- walk, and speak. She was investigated else- terised by seizures, alterations in conscious- where at this stage. An EEG carried out ness, and focal neurological signs. Myoclonus, showed runs of polyspike/spikes at about 5/6 on September 30, 2021 by guest. Protected copyright. when present, is usually only a minor feature of per second, associated with the episodes of the overall clinical syndrome. If the patient jerking. CT scan of the head and CSF exam- survives the acute illness the myoclonus usually ination at that time were normal. She was disappears. Segmental myoclonus following treated with clobazam which benefitted her herpes zoster infection is also well known8 9 initially, but she developed tolerance to it, as and is usually transient. The opsoclonus- she did to propranolol which was tried next. At myoclonus or the "dancing eyes and dancing this stage, when seen in our department, she The Institute of is a brain stem had the attacks only once or twice a Neurology and The feet" syndrome encephalitis jerking National Hospital for preceded or associated with a variety of known day. However, she had spontaneous, repetitive Neurology and and often unknown infectious agents.'o " This wriggling jerks of arms, legs, and head, lasting Neurosurgery, Queen self limiting condition is easily recognised many minutes and increasing on stress. At the Square, London, UK K Bhatia because of the association of the myoclonus beginning of her examination, she could not P D Thompson with the characteristic eye signs. walk because of the jerking of her legs. She also C D Marsden Here, we draw attention to patients who had almost continuous rhythmic multifocal Correspondence to: or focal as an myoclonus ofboth arms, and occasional bursts Professor Marsden, Institute develop generalised myoclonus, of Neurology, Queen isolated phenomenon, after a mild illness of titubation of the head. Twenty minutes later Square, London WCIN (probably viral) without any features of ence- the large jerks gradually settled and dis- 3BG, UK or of the syn- appeared. She could then walk normally heel Received 28 November 1991 phalitis opsoclonus-myoclonus -Accepted 23 January 1992 drome. These patients have no other to toe and hop on either leg. The rest of the 1 090 Bhatia, Thompson, Marsden J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.11.1089 on 1 November 1992. Downloaded from CNS examination revealed no abnormality in liver function tests, calcium, copper and caer- tone, power, and deep tendon reflexes with the uloplasmin, thyroid function, ANF and plantar responses being flexor and sensation autoantibodies were all normal or negative. A normal. She was advised to take sodium CSF examination was not done because the valproate 500 mgs, to which she showed an patient was unwilling to undergo a lumbar excellent response and the myoclonus subsided puncture. A CT scan of the head was normal. almost completely in a few months. The patient recovered completely within 6 months. Case 2 A 14 year old girl presented with myoclonic jerking of the right arm, two weeks after the onset of uncomplicated chicken pox Discussion which resolved in about 10 days. The jerking of Generalised myoclonus developed in case 1 the arm progressively got worse and reached its following an influenza-like illness whilst case 2 peak after three weeks making the use of her developed segmental myoclonus following right hand difficult, especially for writing. The uncomplicated chicken pox. Neither patient jerking worsened when she was anxious or had altered consciousness, seizures, or other tired. She had no weakness or sensory symp- clinical signs to suggest an acute encephalitis toms in that arm. Five weeks after the onset or myelitis. Myoclonus was the sole manifesta- when seen by us she had begun to notice some tion of their post-infectious syndrome. We improvement. Her past medical history was suggest that the term isolated post-infectious unremarkable. myoclonus is appropriate to describe the On examination higher mental function was myoclonic syndromes exhibited by these two normal as were the cranial nerves. There were patients and in others who satisfy the following occasional jerks of the right triceps, brachior- criteria: 1) A sudden onset of generalised, adialis, wrist and finger extensors and biceps multifocal or segmental myoclonus; 2) a his- with sparing of the spinati and pectorals. Some tory of a recent preceding infectious illness; 3) jerks appeared myoclonic while others were no features of encephalitis or the opsoclonus- longer lasting and resembled tonic spasms. The myoclonus syndrome; 4) a non-progressive jerks increased on sustained posture. There course without seizures, ataxia, or dementia was no stimulus or action sensitivity. Occa- and 5) recovery in a short but variable period sional dystonic posturing of the right hand was of time. also noted. On one occasion she was seen to A number of similar cases are described in have some jerks of the left upper and lower the literature where myoclonus developed fol- limbs but this was not a repeated observation. lowing presumed viral infections of the upper There was no wasting, and the tone, power and respiratory tract or following an influenza-like reflexes were normal. The deep tendon reflexes illness (table'4"7). The interval between the and plantar responses were normal and there initial illness and the onset of myoclonus was was no sensory deficit. General examination short. None of these cases had any focal was normal except for a mild thoracic scoliosis neurological signs. The course of the condition with convexity to the left. was benign and the prognosis good. Both our Electrophysiological investigation showed a cases recovered in about six months and most single long duration burst and short trains of 3 in the literature recovered over a period of bursts betwen 50 and 100 milliseconds in the weeks. Silfverskiold'7 described three young right brachioradialis and triceps muscles. girls with the subacute onset of rhythmic http://jnnp.bmj.com/ Back-averaging the EEG activity preceding the myoclonus of upper and lower limbs who had jerks did not show any time locked cerebral no preceeding illness. Although there was no events. Cortical somatosensory evoked respon- definite evidence of previous infection, they ses were of normal latency and morphology. were presumed to be post-infectious on epide- Magnetic stimulation of the motor cortex miological grounds, because all three lived in produced normal responses in the right arm. the same area, and developed a similar benign There was no stimulus sensitivity to percussion myoclonic syndrome in the autumn of 1958. on September 30, 2021 by guest. Protected copyright. or electrical stimulation of the median nerves. All recovered over 6 to 18 months of onset. EEG showed some asymmetry in the activity There are other cases in the literature of between the hemispheres with better formed myoclonus attributed to preceding infec- " alpha activity on the right. In addition, there tions." In a large survey of myoclonus, were some runs of slow activity on the right.