Motor Neuron Disease After Electric Injury
Total Page:16
File Type:pdf, Size:1020Kb
J Neurol Neurosurg Psychiatry 2001;71:265–267 265 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.71.2.265 on 1 August 2001. Downloaded from SHORT REPORT Motor neuron disease after electric injury H Jafari, P Couratier, W Camu Abstract spinal cord sequelae are usually seen when The occurrence of motor neuron disease weakness develops within days or years after after electrical injury in six patients is the shock.6 The spinal forms resemble either reported and compared with patients from lower motor neuron syndrome, amyotrophic the literature. The patients were five men lateral sclerosis, or transverse myelitis. Sensory with spinal onset and one woman with bul- changes in the aVected limb are common.7 bar motor neuron disease after electric Onset of the disease is at the site through which shock. Two patients were struck by light- the shock entered, and may spread or remain ning and four by industrial electric shock. localised.6 The interval between the electrical For all six of them, the disease started at injury and the onset of motor neuron disease 3 the site of the electrical trauma. The mean has no limits (days to decades). Recovery, delay for onset of motor neuron disease was when it occurs, is usually slow and incomplete. 44 months. In four of the spinal patients the We report six cases of motor neuron disease disease progressed slowly with mild handi- after electrical injury, and compare them with cap after several years. For the fifth patient, 65 cases from the literature. improvement was noted progressively. The patient with bulbar disease died 26 months Case reports after onset. A link between electric shock All patients underwent comprehensive biologi- and motor neuron disease is likely, given cal and radiological investigations to rule out the homogenous profile of the patients both diVerential diagnosis of degenerative motor in the five spinal cases presented here and neuron disease. They all had complete elec- in the literature. Bulbar onset has not been troneuromyography. In all cases, a denervation reported to date. However, in this patient pattern was found corresponding with motor the long delay between the electrical injury unit potentials (MUPs) with increased dura- and motor neuron disease, together with tion and amplitude associated with a high firing the rapid evolution may suggest a chance rate (>15 Hz) and decreased recruitment. association. (J Neurol Neurosurg Psychiatry 2001;71:265–267) PATIENT 1 http://jnnp.bmj.com/ A 56 year old man was struck by lightning in Keywords: motor neuron disease; electric injury the right hand while using an ice axe during a hiking trip in the mountains. The current made its exit through his right foot. Six months later Motor neuron disease is a rapidly progressive he complained of weakness in his right upper and fatal degenerative disorder involving either and lower limbs. Two years later there was dif- upper or lower motor neurons, or both.1 The fuse amyotrophy and weakness of all muscle aetiopathogeny of the disease is unknown. groups of the right upper limb without fascicu- on October 2, 2021 by guest. Protected copyright. Fédération de However, in a few cases the disease has been lations. Deep tendon reflexes were normal. The Neurologie, Hôpital linked with genetic abnormalities.2 Several plantar responses were both flexor. Pain and Gui de Chauliac, CHU reports have pointed out the possibility of paraesthesia appeared immediately after the de Montpellier, 34 295 motor neuron disease after electrical injury trauma and were still present in his right hand. Montpellier Cedex 5, 3 France such as industrial electric shock or lightning. There was sensory loss for temperature on the H Jafari As early as 1889, Charcot noted that the nerv- right upper limb. Neurography was normal W Camu ous system could be aVected by an electrical except for a decreased amplitude for the com- injury.4 Delayed neurological sequelae after pound muscle action potentials (CMAPs) on Service de Neurologie, electrical injuries have been reported in the lit- motor nerves on the right upper and lower CHU Dupuytren, 87000 - Limoges, erature and diVerent reports have focused on limbs. Myography disclosed a denervation pat- France the occurrence of motor neuron disease after tern on all four limbs. He was diagnosed as P Couratier electrical injury. The neurological sequelae having progressive lower motor neuron disease, after being struck by lightning are polysympto- which after 3 years had not progressed. Correspondence to: matic and those of industrial injuries are more Professor W Camu 5 [email protected] homogenous. Spinal cord damage is the most PATIENT 2 common of the permanent delayed sequelae of Seven months after having experienced a direct Received 29 December 2000 electrical injury and occurs when the path of electric shock (380 V) to the left hand by turn- and in revised form 27 March 2001 the current is either from arm to arm or from ing the door knob of a door which was touch- Accepted 2 April 2001 arm to leg. It has been stressed that permanent ing a short circuit in the wall, a 42 year old man www.jnnp.com 266 Jafari, Couratier, Camu J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.71.2.265 on 1 August 2001. Downloaded from complained of fasciculations and weakness of fasciculations. Deep tendon reflexes were nor- the left hand. Twenty two months after the mal. The left plantar response was extensor. trauma there was severe amyotrophy of the left Electroneuromyography showed a denervation hand associated with muscle weakness in the pattern on all four limbs. He was considered as left upper limb rated 1/5 on distal and 4/5 on having a possible amyotrophic lateral sclerosis/ proximal muscles (Medical Research Council motor neuron disease. The motor deficit (MRC) scale). Fasciculations were present on progressively extended and the patient died of the tongue. Deep tendon reflexes were brisk on respiratory failure after 107 months. all four limbs. Plantar reflexes were flexor. Sen- sory examination and neurography were nor- PATIENT 6 mal. Myography disclosed a denervation pat- A 67 year old woman presented with dysarthria tern on all four limbs. He was diagnosed with and dysphagia. Eighteen years earlier she had probable amyotrophic lateral sclerosis/motor been struck by lightning while talking on the neuron disease. After more than 3 years the phone, holding the receiver to her left ear with patient’s course showed slow progression of her left hand. The electric current came out of muscle weakness, but with a mild handicap her left thigh, causing superficial burning of her (ALS functional rating scale 35/40). skin. Physical examination showed brisk deep tendon reflexes on her four limbs, and also a PATIENT 3 brisk jaw jerk. Plantar responses were both Ten days after he had been shocked by 380 V extensor. She had dysarthric speech, dyspho- from an electric cable on the right hand, a 68 nia, and dysphagia, associated with tongue year old man complained of weakness in his atrophy and fasciculations. Neurography was right upper limb. Three months after the normal. Myography showed a denervation pat- trauma physical examination disclosed muscle tern on all four limbs, on the abdomen, and on weakness in the right hand rated 3/5 on the the tongue. She was considered as having defi- MRC scale. Deep tendon reflexes were absent nite amyotrophic lateral sclerosis/motor neuron on all four limbs but the plantar responses were disease. with bulbar onset. She died in her sleep both extensor. There were fasciculations on the 26 months after the onset. right deltoid. Sensory examination and neu- rography were normal and myography showed Review of the literature a denervation pattern on all four limbs and on A 6 year old boy injured by a 220 V power line the abdominal muscles. He was initially who developed amyotrophy of the traumatised upper limb and shoulder 15 months later, was diagnosed as having amyotrophic lateral sclero- 8 sis, but his condition progressively improved. reported on by Hoel. Physical examination After 12 years the patient had no complaints disclosed “lively” deep tendon reflexes, and a and physical examination showed a discrete stiV gait. The patient was considered as having weakness in the right hand rated 4+/5 on the a typical clinical picture of amyotrophic lateral MRC scale. We considered him to have an sclerosis, which continued to advance over sev- almost complete remission of a motor neuron eral years. disease after 12 years of follow up. Farrell and Starr described a 67 year old man who was injured in his right upper limb by 18 000 V from a high tension line. Immediately PATIENT 4 after the trauma, the patient complained of http://jnnp.bmj.com/ A 50 year old man had an electric shock to his pain in the right hand and left foot, where the right hand in April 1999, after coming into current had made its exit. Two years later he contact with a 380 V cable. Immediately after complained of weakness and numbness of his the trauma he complained of pain and paraes- left leg. Physical examination showed amyotro- thesia of the right hand. Sixteen months later, phy, fasciculations, and deficit of the left quad- there was a progressive motor deficit of the riceps. The left patellar tendon reflex was right arm associated with amyotrophy and fas- absent. There was decreased sensation to pin, ciculations. Deep tendon reflexes were present touch, temperature, and vibration on the left on October 2, 2021 by guest. Protected copyright. in the atrophied muscles of the right arm and thigh. Neurography was normal, whereas were brisk elsewhere. Plantar responses were myography showed a denervation pattern on both flexor.