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Critically ill polyneuropathy associated with burns: a case report
Bums (1989)15, (3). 179-180
179
Printed in Great Britain
Critically ill polyneuropathy a case report
associated with burns:
N. Carver and A. Logan West Norwich
Hospital, Norwich, UK
A severe sensorimotor peri$eral neuropathy is reportedin a patient with burns covering 35 per cent of the body surface area, severe sepsis ad acute renal failure. It presentecIas dificulty in weaning the patient from a ventilator. Attenfion is drawn to the condition of cr&ally ill polyneuropathy which has been recently noted in severe illnesses ana’sepsis. from a search of the literafure we believe this to be thefirst report of this condition in association with thermal injury.
Introduction The incidence of peripheral neuropathy following bum injury has been quoted at between 15 per cent (Henderson et al., 1971) and 29 per cent (Helm et al., 1985). This may be a mononeuropathy due to local factors or a polyneuropathy due to general factors. Anastakis et al. (1987) provide a review of the aetiology of this condition. Critically ill polyneuropathy is a recently noted clinical entity (Bolton et al., 1986), observed in varying degree in patients suffering severe sepsis from a variety of causes. It usually begins within I month of admission at the peak of the critical illness. Early clinical signs include difficulty in weaning the patient from the ventilator as the severe illness resolves, limb weakness or complete loss of active movement, muscle wasting and reduced or absent deep tendon reflexes. Movement in the head, facial, jaw and tongue muscles are relatively preserved. Sensory signs are milder, consisting of loss of all modalities in a symmetrical glove and stocking distribution, and problems may be encountered with painless pressure sores. All signs are most marked in the distal parts.
Case report A 27-year-old man was admitted to our Burns Unit following a road traffic accident in which his vehicle caught fire. He had sustained deep bums involving muscle on his left side amounting to 35 per cent of his total body surface; he had also sustained considerable trauma to his left side including a closed disruption of his left knee. Inhalation injury was evident with soot found circumorally and in the nostrils. There was no history or evidence of head injury and the patient was fully orientated on admission. He had no significant past medical history and there was no family history 0 1989 Butteworth & Co (Publishers) Ltd 0305-4179/89/030179-02$03.00
of hereditary sensory neuropathy. A standard comprehensive neurological examination was undertaken on admission. There was no evidence of muscle wasting or sensory deficit. Motor power was full on the right side and active movement was present on the injured left side. Escharotomies were performed and intravenous methyl prednisolone was given. His resuscitation was uncomplicated and excision and closure of the bum wound was carried out at days 3,14 and completed on day 28. A high pyrexia of 40°C developed on day 4. Cultures of wound swabs grew Pseua&anas ueruginosa from day 6 whilst blood cultures grew Staph. epiabmidis from day 9. Treatment was initially with cefuroxime and metronidazole intravenously. The pyrexia continued and netilmicin was given in addition from day 10. Respiratory function deteriorated on day 11; the patient became severely tachypnoeic and distressed and artificial ventilation was instituted. Total parenteral nutrition via a central venous catheter was commenced on day 7. His pyrexia continued and 20 days postinjury acute renal failure developed. There had been no episodes of hypotension and prolonged septicaemia was felt to be the cause. Peritoneal dialysis was started. On day 41 pseudomonas contamination of the peritoneal dialysis catheter forced its removal and haemodialysis was commenced. As well as the foregoing description, a further deterioration in the patient’s condition was evident. The patient’s general ability to cooperate with staff in normal daily nursing procedures deteriorated. Ulceration and pressure sores developed at the elbow and heels. Little reaction was provoked by previously painful dressings of the burned and grafted areas and physiotherapy then became restricted to passive manoeuvres as active cooperation was not possible. At 7 weeks postburn, the pyrexia finally resolved and renal function gradually recovered. However, he was still unable to breathe spontaneously despite the improvement in his condition. There was severe muscle wasting and hypotonia in all muscle groups of both upper and lower limbs and there was no active movement in this distribution or in the trunk. He was also unable to move his neck and jaw. Voluntary eye movements were however intact, he was alert and could respond in this way to members of staff. The deep tendon reflexes were absent. A symmetrical sensory deficit was found in a glove and stocking distribution below the knees and in the fingers of both hands. These findings were consistent with a severe sensorimotor peripheral neuropathy. The condition continued unchanged for a further 4 weeks, but
180
Burns (1989) Vol. 15/No.
then with steady improvement, the patient was finally weaned from the ventilator at II weeks postburn; he had been ventilator dependent for 8 weeks. Electrophysiological studies in the 18 months of the illness confirmed the sensorimotor neuropathy. Needle sampling showed denervation in the right tibialis anterior, gastrocnemius and 1st dorsal interrosseus. It was not possible to obtain a conduction velocity in the lower limbs. The median and ulnar nerve conduction velocities were 48 m/s (normal range 48-70 m/s) with prolonged distal latencies. The ulnar sensory Jction potential and evoked muscle action potential were reduced in amplitude in keeping with axonal loss. Arthrodesis of the left knee was performed 6 months after the initial injury and after intensive rehabilitation, at 24 months, the patient was independently mobile with crutches. The peripheral neuropathy had gradually improved; that in the upper limbs had totally resolved while in the legs, a sensory deficit to alJ modalities remained distal to the ankles. Motor function continued to return; almost full power had been regained to the level of the knee below which hypotonia and wasting persisted movement was present only in the gastrocnemius and soleus muscles.
Discussion It was not possible ropathy
in this patient.
to find the aetiology of the polyneuThe condition could not be explained
on the basis of localized factors such as injury, escharotomies, debridement, splintage or dressings. There was no history of general metabolic disturbance such as diabetes mellitus, alcoholism, malnutrition or porphyria. Similarly, there was no history of neuromuscular disorder. Polyneuropathy has not been reported in association with netilmicin administration and neurotoxic side-effects were not a feature of any other drugs administered to this patient. Full nutritional support was provided and there was no evidence of specific vitamin deficiency. A low serum albumin was the only indication of non-specific nutritional deficiency. Although the patient was septic, organisms known to produce toxins affecting the peripheral nervous system were not cultured. Our patient suffered acute renal failure. However, with dialysis the serum creatinine was maintained below 500 pmol/l (normal 55-125 pmol/l) and with a maximum urea level of 40pmoVl (normal 1.7-6.7~oVl. Neuropsychiatric manifestations of acute uraemia are varied, including lethargy, confusion, stupor, coma, agitation, psychosis, asterixis, twitching myoclonus, hyperreflexia, restless legs and seizures. These are usually rapidly improved by dialysis. However, focal neurological deficit is rare (Brezis et al., 1986). Acute renal failure in this patient was recognized early, treated aggressively and had a duration of only 4 weeks. Although acute uraemia can affect peripheral nerves it must persist for some time before polyneuropathy develops (Locke et al., 1961). The neuropathy in this patient was out of proportion to the duration and severity of the acute uraemia. Furthermore, adequate haemodialysis prevents deterioration of uraemic neuropathy and induces improvement in the condition (lennekens, 1987). It is concluded, therefore, that the polyneuropathy originated from an unrelated cause. The clinical picture of critically ill polyneuropathy is indistinguishable from Guillain-Barre syndrome. However, critically ill polyneuropathy occurs at the peak of the severe illness rather than presenting after a period of recovery from a minor illness.
3
Bolton et al. (1986) reported 15 patients with critically ill polyneuropathy associated with a variety of conditions of severe illness and sepsis. They found lower cerebrospinal fluid protein levels and electrophysiological differences in critically ill polyneuropathy compared with Guillain-Barre syndrome, but large standard deviations in their results made these indices unreliable in distinguishing between the two conditions. The polyneuropathy of Guillain-Barre syndrome recovers and the prognosis is excellent. In critical illness, the neuropathy was unchanged, or became worse in 40 per cent of the patients reported. Post-mortem examination of those cases showed primary axonal degeneration of peripheral nerves with sparing of the central nervous system and acute denervation atrophy of muscle. This pattern suggests toxic or metabolic disturbance as a cause rather than an inflammatory immune-mediated demyelination which is found in Guillain-Barre syndrome. Peripheral polyneuropathy can significantly affect the rehabilitation of burn patients. Critically ill polyneuropathy may explain a hitherto unexplained and puzzling deterioration in a patient’s condition or its failure to improve. Recognition of the condition enables the patient to be managed more intelligently and an expectant policy adopted. Full supportive measures should be continued, including ventilator-y assistance, full nutritional support, passive physiotherapy to prevent joint contractures and nursing care to prevent pressure sores which are liable to develop. No specific medical treatment is known to be of value in the prevention or cure of this particular type of polyneuropathy and its cause has yet to be elucidated.
References Anastakis D. J., Peters W. J. and Lee K. C. (1987) Severe burn polyneuropathy: a case report. Bums 13,232. Bolton C. F., Laverty D. A., Brown J. D. et al. (1986) Critically ill polyneuropathy: electrophysiological studies and differentiation from Guillain-Barre syndrome. J. Neural. NeurosuTg. Psychiafr.49, 563. Brezis M., Rosen S. and Epstein F. H. (1986) Acute renal failure. In:
Brenner B. M. and Rector F. C. (eds), ‘Ibe Kidney, 3rd edn. Philadelphia: W. B. Saunders, p. 767. Helm P. A., Pondian G. and Heck E. (1985) Neuromuscular problems in the bum patient: causes and prevention. Arch. Phys.Med. Rehabil.66, 451. Henderson B., Koepke G. H. and Feller I. (1971) Peripheral neuropathy among patients with bums. Arch. Phys. Med. Rehabil. 52, 149. Jennekens F. G. I. (1987) Peripheral neuropathy in renal and hepatic insufficiency. In: Matthews W. B. (ed.), I;ladbmk 4 Clinical Neurology, vol. 7 (51). New York: Elsevier Science, pp. 355-366. Locke S., Merrill J. P. and Tyler H. R. (1961) Neurologic complications of acute uraemia. Arch. Intern. Med. 108,519.
Paper accepted 20 October
1988.
Correspondence should be aaiiresd to: Mr N. Carver, Regional Plastic Maxillofacial and Oral Surgery Centre, Mount Vernon Hospital, Northwood, Middlesex, HA6 2RN. UK.
179
Printed in Great Britain
Critically ill polyneuropathy a case report
associated with burns:
N. Carver and A. Logan West Norwich
Hospital, Norwich, UK
A severe sensorimotor peri$eral neuropathy is reportedin a patient with burns covering 35 per cent of the body surface area, severe sepsis ad acute renal failure. It presentecIas dificulty in weaning the patient from a ventilator. Attenfion is drawn to the condition of cr&ally ill polyneuropathy which has been recently noted in severe illnesses ana’sepsis. from a search of the literafure we believe this to be thefirst report of this condition in association with thermal injury.
Introduction The incidence of peripheral neuropathy following bum injury has been quoted at between 15 per cent (Henderson et al., 1971) and 29 per cent (Helm et al., 1985). This may be a mononeuropathy due to local factors or a polyneuropathy due to general factors. Anastakis et al. (1987) provide a review of the aetiology of this condition. Critically ill polyneuropathy is a recently noted clinical entity (Bolton et al., 1986), observed in varying degree in patients suffering severe sepsis from a variety of causes. It usually begins within I month of admission at the peak of the critical illness. Early clinical signs include difficulty in weaning the patient from the ventilator as the severe illness resolves, limb weakness or complete loss of active movement, muscle wasting and reduced or absent deep tendon reflexes. Movement in the head, facial, jaw and tongue muscles are relatively preserved. Sensory signs are milder, consisting of loss of all modalities in a symmetrical glove and stocking distribution, and problems may be encountered with painless pressure sores. All signs are most marked in the distal parts.
Case report A 27-year-old man was admitted to our Burns Unit following a road traffic accident in which his vehicle caught fire. He had sustained deep bums involving muscle on his left side amounting to 35 per cent of his total body surface; he had also sustained considerable trauma to his left side including a closed disruption of his left knee. Inhalation injury was evident with soot found circumorally and in the nostrils. There was no history or evidence of head injury and the patient was fully orientated on admission. He had no significant past medical history and there was no family history 0 1989 Butteworth & Co (Publishers) Ltd 0305-4179/89/030179-02$03.00
of hereditary sensory neuropathy. A standard comprehensive neurological examination was undertaken on admission. There was no evidence of muscle wasting or sensory deficit. Motor power was full on the right side and active movement was present on the injured left side. Escharotomies were performed and intravenous methyl prednisolone was given. His resuscitation was uncomplicated and excision and closure of the bum wound was carried out at days 3,14 and completed on day 28. A high pyrexia of 40°C developed on day 4. Cultures of wound swabs grew Pseua&anas ueruginosa from day 6 whilst blood cultures grew Staph. epiabmidis from day 9. Treatment was initially with cefuroxime and metronidazole intravenously. The pyrexia continued and netilmicin was given in addition from day 10. Respiratory function deteriorated on day 11; the patient became severely tachypnoeic and distressed and artificial ventilation was instituted. Total parenteral nutrition via a central venous catheter was commenced on day 7. His pyrexia continued and 20 days postinjury acute renal failure developed. There had been no episodes of hypotension and prolonged septicaemia was felt to be the cause. Peritoneal dialysis was started. On day 41 pseudomonas contamination of the peritoneal dialysis catheter forced its removal and haemodialysis was commenced. As well as the foregoing description, a further deterioration in the patient’s condition was evident. The patient’s general ability to cooperate with staff in normal daily nursing procedures deteriorated. Ulceration and pressure sores developed at the elbow and heels. Little reaction was provoked by previously painful dressings of the burned and grafted areas and physiotherapy then became restricted to passive manoeuvres as active cooperation was not possible. At 7 weeks postburn, the pyrexia finally resolved and renal function gradually recovered. However, he was still unable to breathe spontaneously despite the improvement in his condition. There was severe muscle wasting and hypotonia in all muscle groups of both upper and lower limbs and there was no active movement in this distribution or in the trunk. He was also unable to move his neck and jaw. Voluntary eye movements were however intact, he was alert and could respond in this way to members of staff. The deep tendon reflexes were absent. A symmetrical sensory deficit was found in a glove and stocking distribution below the knees and in the fingers of both hands. These findings were consistent with a severe sensorimotor peripheral neuropathy. The condition continued unchanged for a further 4 weeks, but
180
Burns (1989) Vol. 15/No.
then with steady improvement, the patient was finally weaned from the ventilator at II weeks postburn; he had been ventilator dependent for 8 weeks. Electrophysiological studies in the 18 months of the illness confirmed the sensorimotor neuropathy. Needle sampling showed denervation in the right tibialis anterior, gastrocnemius and 1st dorsal interrosseus. It was not possible to obtain a conduction velocity in the lower limbs. The median and ulnar nerve conduction velocities were 48 m/s (normal range 48-70 m/s) with prolonged distal latencies. The ulnar sensory Jction potential and evoked muscle action potential were reduced in amplitude in keeping with axonal loss. Arthrodesis of the left knee was performed 6 months after the initial injury and after intensive rehabilitation, at 24 months, the patient was independently mobile with crutches. The peripheral neuropathy had gradually improved; that in the upper limbs had totally resolved while in the legs, a sensory deficit to alJ modalities remained distal to the ankles. Motor function continued to return; almost full power had been regained to the level of the knee below which hypotonia and wasting persisted movement was present only in the gastrocnemius and soleus muscles.
Discussion It was not possible ropathy
in this patient.
to find the aetiology of the polyneuThe condition could not be explained
on the basis of localized factors such as injury, escharotomies, debridement, splintage or dressings. There was no history of general metabolic disturbance such as diabetes mellitus, alcoholism, malnutrition or porphyria. Similarly, there was no history of neuromuscular disorder. Polyneuropathy has not been reported in association with netilmicin administration and neurotoxic side-effects were not a feature of any other drugs administered to this patient. Full nutritional support was provided and there was no evidence of specific vitamin deficiency. A low serum albumin was the only indication of non-specific nutritional deficiency. Although the patient was septic, organisms known to produce toxins affecting the peripheral nervous system were not cultured. Our patient suffered acute renal failure. However, with dialysis the serum creatinine was maintained below 500 pmol/l (normal 55-125 pmol/l) and with a maximum urea level of 40pmoVl (normal 1.7-6.7~oVl. Neuropsychiatric manifestations of acute uraemia are varied, including lethargy, confusion, stupor, coma, agitation, psychosis, asterixis, twitching myoclonus, hyperreflexia, restless legs and seizures. These are usually rapidly improved by dialysis. However, focal neurological deficit is rare (Brezis et al., 1986). Acute renal failure in this patient was recognized early, treated aggressively and had a duration of only 4 weeks. Although acute uraemia can affect peripheral nerves it must persist for some time before polyneuropathy develops (Locke et al., 1961). The neuropathy in this patient was out of proportion to the duration and severity of the acute uraemia. Furthermore, adequate haemodialysis prevents deterioration of uraemic neuropathy and induces improvement in the condition (lennekens, 1987). It is concluded, therefore, that the polyneuropathy originated from an unrelated cause. The clinical picture of critically ill polyneuropathy is indistinguishable from Guillain-Barre syndrome. However, critically ill polyneuropathy occurs at the peak of the severe illness rather than presenting after a period of recovery from a minor illness.
3
Bolton et al. (1986) reported 15 patients with critically ill polyneuropathy associated with a variety of conditions of severe illness and sepsis. They found lower cerebrospinal fluid protein levels and electrophysiological differences in critically ill polyneuropathy compared with Guillain-Barre syndrome, but large standard deviations in their results made these indices unreliable in distinguishing between the two conditions. The polyneuropathy of Guillain-Barre syndrome recovers and the prognosis is excellent. In critical illness, the neuropathy was unchanged, or became worse in 40 per cent of the patients reported. Post-mortem examination of those cases showed primary axonal degeneration of peripheral nerves with sparing of the central nervous system and acute denervation atrophy of muscle. This pattern suggests toxic or metabolic disturbance as a cause rather than an inflammatory immune-mediated demyelination which is found in Guillain-Barre syndrome. Peripheral polyneuropathy can significantly affect the rehabilitation of burn patients. Critically ill polyneuropathy may explain a hitherto unexplained and puzzling deterioration in a patient’s condition or its failure to improve. Recognition of the condition enables the patient to be managed more intelligently and an expectant policy adopted. Full supportive measures should be continued, including ventilator-y assistance, full nutritional support, passive physiotherapy to prevent joint contractures and nursing care to prevent pressure sores which are liable to develop. No specific medical treatment is known to be of value in the prevention or cure of this particular type of polyneuropathy and its cause has yet to be elucidated.
References Anastakis D. J., Peters W. J. and Lee K. C. (1987) Severe burn polyneuropathy: a case report. Bums 13,232. Bolton C. F., Laverty D. A., Brown J. D. et al. (1986) Critically ill polyneuropathy: electrophysiological studies and differentiation from Guillain-Barre syndrome. J. Neural. NeurosuTg. Psychiafr.49, 563. Brezis M., Rosen S. and Epstein F. H. (1986) Acute renal failure. In:
Brenner B. M. and Rector F. C. (eds), ‘Ibe Kidney, 3rd edn. Philadelphia: W. B. Saunders, p. 767. Helm P. A., Pondian G. and Heck E. (1985) Neuromuscular problems in the bum patient: causes and prevention. Arch. Phys.Med. Rehabil.66, 451. Henderson B., Koepke G. H. and Feller I. (1971) Peripheral neuropathy among patients with bums. Arch. Phys. Med. Rehabil. 52, 149. Jennekens F. G. I. (1987) Peripheral neuropathy in renal and hepatic insufficiency. In: Matthews W. B. (ed.), I;ladbmk 4 Clinical Neurology, vol. 7 (51). New York: Elsevier Science, pp. 355-366. Locke S., Merrill J. P. and Tyler H. R. (1961) Neurologic complications of acute uraemia. Arch. Intern. Med. 108,519.
Paper accepted 20 October
1988.
Correspondence should be aaiiresd to: Mr N. Carver, Regional Plastic Maxillofacial and Oral Surgery Centre, Mount Vernon Hospital, Northwood, Middlesex, HA6 2RN. UK.