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Postgrad Med J 2002;78:377–380 377 Postgrad Med J: first published as 10.1136/pmj.78.920.377-a on 1 June 2002. Downloaded from SELF ASSESSMENT ANSWERS Box 1: Aetiology of

• Trauma. Back and systemic frank septicaemia or those with gross neuro- logical signs do poorly.4 • . compromise • Ischaemia. Conclusion • Metabolic defects: glycogenoses, carni- Q1: What is the most likely clinical Spinal epidural abscess remains a clinical tine palmitoyltransferase deficiency. diagnosis? diagnosis. A high index of suspicion and rapid • Drugs: clofibrate, , epsilon- The triad of /tenderness, neurologi- neurosurgical attention are essential to mini- aminocaproic acid, , etretinate, cal deficits, and systemic illness are highly mise mortality and long term morbidity. suggestive of a spinal epidural abscess. high dose steroids. Final diagnosis • Alcohol. Q2: What does the MRI scan show? Lumbar spinal epidural abscess. • Infectious diseases. The MRI scan (see p 373) shows a large • Malignant hyperpyrexia: halothane, en- central disc prolapse at the L4/L5 level. There References flurane, isoflurane, succinylcholine, cal- is also, however, loss of cerebrospinal fluid 1 Calderone RR, Larson JM. Overview and cium channel blockers. signal behind the dura from the first lumbar classification of spinal . Orthop Clin • Malignant neuroleptic syndrome. vertebral level caudally, suggestive of a com- North Am 1996;27:1–8. Baker AS • imbalance: hypokalaemia, pressive lesion. 2 , Ojemann RG, Swartz MN, et al. Spinal epidural abscess. N Engl J Med hypomagnesaemia, 1975;293:463–8. hypophosphataemia. Q3: Discuss the management of this 3 Verner EF, Musher DM. Spinal epidural condition abscess. Symposium on infections in the Urgent laminectomy was performed. At opera- central nervous system. Med Clin North Am tion free pus was found in the muscular and 1985;69:375–84. 4 Danner RL, Hartmann BJ. Update of spinal always be considered (many would not neces- fascial layers. There was a large epidural epidural abscess: 35 cases and a review of sarily be relevant in this particular age group), abscess which had caused severe compression the literature. Rev Infect Dis 1987;9:265–74. although such an acute, de novo presentation of the lumbar thecal sac posteriorly. The would be unusual. epidural space was debrided and irrigated. In this particular patient, the aetiological Cultures of the specimens as well as blood cul- A bad dose of ’flu agent was a virus, influenza type B, serological tures revealed a staphylococcal . The testing indicating a rising antibody titre to 1 Q1: What is the most likely diagnosis patient was started on high dose intravenous in 320. flucloxacillin, metronidazole, and gentamicin. and how would you confirm it? Postoperatively, he was improved neurologi- The most likely diagnosis is one of acute Q3: What potential complications may cally with near normal power in the lower rhabdomyolysis together with , occur? limbs bilaterally and normal sensation includ- as demonstrated by the generalised oedema, There are several potential complications. ing an improvement in his perianal sensation. muscle tenderness and , and the Massive rhabdomyolysis may result in electro- His subsequent recovery, however, was compli- brown discoloration of the . To confirm lyte imbalance, particularly hyperkalaemia, cated by septicaemia, acute respiratory distress the diagnosis, the urine should be tested for hyperphosphataemia, and . syndrome, and disseminated intravascular (radioimmunoassay is the best Disseminated intravascular and coagulation, which were successfully treated. technique) and muscle should be venous thromboses may also occur. Most seri- measured. ously, severe myoglobinuria may cause renal Discussion In this patient, the urine was positive for damage and . The exact mechanism by The risks of developing an abscess in the spine myoglobin and had an acidic pH of 6.0. Mus- which this occurs is uncertain but hypotheses cle enzymes were grossly raised, with creatine are greater in those with diabetes mellitus, include renal tubular obstruction by precipi- http://pmj.bmj.com/ intravenous drug abuse, tuberculosis, malnu- kinase 30 000 U/l, 2900 tated myoglobin, reduction in renal blood trition, chronic renal failure, and cancer.1 U/l, and aspartate aminotransferase 710 U/l. flow, and direct toxic to the tubular Making the diagnosis of an epidural abscess The differential diagnosis of a patient epithelium. can be difficult. Reliance on imaging alone becoming weak on a background of such a may be misleading since the radiological prodromal illness as that described here Q4: How would you manage this changes, as in this case, may be subtle. would normally include an acute inflamma- patient? Furthermore, the condition may be masked by tory demyelinating polyneuropathy (or In general, good nursing and medical care other more common pathologies. In this case, Guillain-Barré syndrome), but the presence of with close monitoring of electrolyte balance, such oedema and muscle tenderness makes there was a large L4/L5 central disc prolapse, renal function, and urinary output are essen- on September 30, 2021 by guest. Protected copyright. although the neurological deficits were far this an unlikely diagnosis even prior to the tial. More specifically, a high fluid intake and more extensive than that expected from such a biochemical results. alkalinisation of the urine by infusion or disc prolapse. It is therefore important to pay A patient presenting in such a manner in ingestion of helps to pro- careful attention to the clinical findings. the postoperative period would lead to suspi- tect the by preventing the formation of Cardinal features of spinal epidural abscess cion of a diagnosis of malignant hyperther- myoglobin casts. This patient received3gof are , spinal tenderness, and neurological mia. This is a rare autosomal dominant condi- , every two hours, by deficit. Pain is the most consistent symptom tion, linked to a mutation in the ryanodine intravenous infusion, until the urine pH was 1 and together with fever often precedes the receptor gene on chromosome 19, in which >7.0. He was also treated with 2 mg/kg of development of hard neurological signs.2 This susceptible individuals are endangered by dantrolene by intravenous infusion, daily for natural history contrasts markedly to that of exposure to certain anaesthetic triggering five days. the acute or chronic degenerative pathologies agents (see box 1). of the spine which tend not to exhibit local tenderness or systemic compromise. Most Q2: What is the possible aetiology of patients are thought to have major neurologi- this condition? Box 2: Infectious agents implicated in cal signs prior to .2 When septicaemia The potential aetiology of rhabdomyolysis is rhabdomyolysis dominates the picture as in this case, the wide (see box 1). In this particular patient neurological symptoms may be missed. This is there would appear to be three main possibili- • A and B. especially true for those patients who may ties. Firstly given the nature of the prodromal • Coxsackie virus. have been confined to bed for some time and symptoms, any one of a range of infectious • Epstein-Barr virus. therefore not regularly assessed.2 agents could be implicated (see box 2). • Cytomegalovirus. Management includes surgical decompres- Secondly, rhabdomyolysis is well recognised sion, , and broad spectrum anti- as a of certain types of drug • Echovirus. microbials. Parenteral treatment should be therapy and this patient had been exposed to • Adenovirus. continued for at least four weeks and may be several agents over the 48 hours immediately • Legionella pneumophila. needed for eight weeks if is before admission, although not before the • Streptococcus pneumoniae. suspected.34 Prognosis is related to the delay time that he first became unwell. Thirdly, the • HIV. in presentation.24 Patients who present with possibility of a primary muscle disease should

www.postgradmedj.com 378 Self assessment answers Postgrad Med J: first published as 10.1136/pmj.78.920.377-a on 1 June 2002. Downloaded from myonecrosis but no viral particles, it is postu- Box 3: Learning points Box 1: Causes of osteolysis lated that the virus releases a circulating toxin or stimulates cytokine release. To date how- • Acute rhabdomyolysis represents a ever, no putative toxins have been identified. Trauma medical and neurological emergency. Dantrolene has been used to beneficial • Reflex sympathetic dystrophy. • Rhabdomyolysis may complicate even effect in rhabdomyolysis arising from malig- usually innocent infections. nant hyperpyrexia, malignant neuroleptic Skin • Psoriasis. • Maintenance of high fluid intake and syndrome, exertion, acute alcoholic intoxica- tion, and both and ecstasy overdose. It alkalinisation of urine are fundamental Infection is known to decrease the release of calcium • Direct. to preserving renal function. from the .6 Calcium • Intravenous dantrolene is a useful thera- may play a part in muscle pain as has been • Indirect—for example, leprosy. peutic option. suggested in McArdle’s disease and in the Tumour • Patients should be monitored for the development of malignant . • Plasmacytoma. possible development of a compartment is also explained by an • Lytic metastases. syndrome and these should be man- increased calcium influx into the cell, trigger- Collagen vascular diseases aged appropriately. ing a vicious cycle of mitochondrial overload- ing and energy depletion, which leads to • Rheumatoid arthritis, usually the lateral hypercontraction and finally, to cell . end of the clavicle. Experiments with dantrolene in cases of • . Discussion exertional rhabdomyolysis have shown an Endocrine 83% reduction in intracellular calcium levels, In the period immediately after admission, which has been associated with marked • Primary hyperparathyroidism. the patient continued to deteriorate. There improvements in both clinical symptoms • Secondary hyperparathyroidism. was progression in with (muscle stiffness, rigidity and pain) and labo- Chronic renal failure lower limb muscle strength ranging between ratory values (83% reduction in creatine 0/5 at knee flexion and 3/5 at knee extension. kinase by day 4).7 Neurology In the upper limb, weakness was less marked, This case demonstrates the potentially • Neuroarthropathy. muscle strength generally 3–4/5. He also acute course of rhabdomyolysis in even developed decreased sensation on the dorsum healthy individuals, highlights the potential of the right foot and increased tension in the complications arising from this condition and Q2: What are the other causes of anterior tibial compartments of both legs. emphasises the routine but effective methods Consideration at this stage was given to of avoiding these. osteolysis? surgical release. The causes of osteolysis are shown in box 1. The level continued to rise, Final diagnosis peaking at 140 000 U/l 48 hours after admis- Acute rhabdomyolysis with myoglobinuria. Q3: What are the other clinical sion, before declining rapidly thereafter. Elec- features of this disease? tromyography and nerve conduction studies References can be associated with Chiari I showed clear myopathic changes in all sam- 1 MacLennan DH, Duff C, Zorzato F, et al. and II malformations, Dandy-Walker malfor- pled muscles with evidence of moderate bilat- Ryanodine receptor gene is a candidate for mation, and basilar invagination. Frequently, eral carpal tunnel compression and moderate- predisposition to . the in children and ado- 343 severe right peroneal and tibial nerve lesions. Nature 1990; :559–61. lescents may include scoliosis, dissociated The neuropathy was attributed to oedema and 2 Gabow PA, Kaehny WD, Kellcher SP. The spectrum of rhabdomyolysis. Medicine sensory loss, neck pain, , and lower compression. 1982;61:141–52. motor neuron signs in the upper limbs. In In the days after the decline in creatine 3 Morton SE, Mathai M, Byrd Jr RP, et al. adults, the clinical presentation is more

kinase, the patient’s condition stabilised and Influenza A pneumonia with rhabdomyolysis. variable, but usually point towards dysfunc- http://pmj.bmj.com/ then gradually improved. Four weeks after the South Med J 2001;94:67–9. tion of the cervical cord. It is worth noting onset of illness he was discharged home, 4 Baine WB, Luby JP, Martin SM. Severe illness that the classic “dissociated sensory loss” is mobilising with the aid of a Zimmer frame. At with influenza B. Am J Med 1980;68:181–9. present in only 49% of patients with a Singh U the most recent follow up several months 5 , Scheld WM. Infectious etiologies of syrinx.2 More commonly, lower motor neuron later, he was mobilising independently, had rhabdomyolysis: three case reports and review. Clin Infect Dis 1996;22:642–9. signs are seen in the upper limbs and long full return of upper limb strength, and had 6 Morgan KG, Bryant SH. The mechanism of tract signs are seen in the lower limbs. Other full power in the lower limb, apart from the action of dantrolene sodium. J Pharmacol Exp findings include dizziness, nystagmus, scolio- right ankle where dorsiflexion and plantar Ther 1977;201:138–47. sis, and even brain stem symptoms like López JR flexion were 4/5. 7 , Rojas B, Gonzalez MA, et al. dysphagia, facial numbness, and vertigo when on September 30, 2021 by guest. Protected copyright. Infectious agents are believed to be impli- Mycoplasmic Ca2+ concentration during there is extension of the syrinx into the cated in only 5% of cases of rhabdomyolysis.2 exertional rhabdomyolysis. Lancet medulla. The association with influenza infection, 1995;345:424–5. although uncommon, is well described in the Discussion literature. A recent review listed 22 cases of A case of acute swelling of Syringomyelia is a cavitation within the spinal rhabdomyolysis associated with generalised, cord appearing in the third to fourth decade. non-pneumonic influenza A infection.3 Of the left shoulder Familial cases have also been described.3 The these, 12 developed acute renal failure (55%) Arnold-Chiari malformation with herniation and four of these died. Influenza type B is Q1: What disease process are the x of the cerebellar tonsil is found in more than much less commonly implicated in rhab- ray and MRI findings diagnostic of? two thirds of patients. It may also be a late domyolysis. There has been a report of two The differential diagnosis of an acute monoar- consequence of trauma, with cases of influenza B in elderly men being thritis includes a pyogenic infection of the delayed onset observed in 5% leading to an associated with rhabdomyolysis, but each of joint. Tuberculosis should also be considered ascending spinal syndrome. As a result of these occurred in the context of hyperpyrexia in an individual who is PPD positive even with arachnoiditis, cerebrospinal fluid circulation and the clinical course was much milder with a normal chest radiograph. The resorption of is impaired. The syrinx mainly in the lower smaller rises than that documented the humeral head with a sharp line of demar- cervical region interrupts decussating spi- in our patient.4 cation, almost as if surgically excised, is diag- nothalamic fibres leading to a loss of pain and The mechanism by which viruses can cause nostic of this disease. Shards of bone can temperature in a shawl-like distribution with rhabdomyolysis is unclear, although at least sometimes be identified in the soft tissue and preservation of light touch, vibration, and two theories have been advanced.5 One theory was noted in a computed tomogram and MRI proprioception. Painless ulcers of the fingers is that there is direct viral invasion into mus- of the shoulder (not shown). This picture is may be a presenting feature. Extension of the cle fibres causing myonecrosis. This is sup- very characteristic of the atrophic form of a syrinx into the anterior horn results in a loss ported by findings of lym- neuropathic arthropathy of the shoulder1 with of motor neurons and an . It phocytic infiltrates, viral inclusions, and viral the most common cause being syringomyelia. begins in the small muscles of the hand with DNA identified by polymerase chain reaction. The MRI (fig 2; see p 374) confirms the diag- asymmetric weakness and early loss of muscle Conversely, where muscle biopsy is either nor- nosis of syringomyelia with a large syrinx in stretch reflexes in arms. Extension into the mal or shows hyaline degeneration and the spinal cord and all cultures were negative. lateral columns results in lower extremity

www.postgradmedj.com Self assessment answers 379 Postgrad Med J: first published as 10.1136/pmj.78.920.377-a on 1 June 2002. Downloaded from computed tomography. The patient’s risk 5 Quint HJ, Drach GW, Rappaport WD, et al. Key learning points factors, marked pyuria, foul smelling urine, Emphysematous cystitis: a review of the and predominant gas collection in the bladder spectrum of disease. J Urol 1992;147:134–7. • Recognise the radiographic appear- wall make a urinary tract infection most likely. ance of osteolysis and know its causes. The final diagnosis was emphysematous An eponymous reaction to • The most common cause of a neuro- cystitis. This is a rare form of urinary tract pathic joint of the shoulder is syringomy- infection in which fermentation of glucose by a knife elia. bacteria causes carbon dioxide production in the bladder wall, which is seen on a plain film Q1: Where is the anatomical site of • Resorption of the head of the humerus is as a radiolucency confined to the bladder wall. injury and which spinal tracts have a characteristic radiographic finding of Gas bubbles collect in the submucosa and been damaged? syringomyelia. eventually rupture, resulting in gas within the A left sided hemisection of the spinal cord at bladder lumen.1 Computed tomography is a T8 plus bilateral posterior column loss. This is very sensitive tool for demonstrating the gas due to the knife track coming obliquely from spasticity, paraparesis, brisk tendon reflexes, within the bladder wall and the extent and the right, across both posterior columns of the and a positive Babinski sign. location of the gas collection. spinal cord before hemisecting the left side of Patients may present with an acute enlarge- Patients may complain of lower abdominal the cord (see figs 1 and 2). The left sided tracts ment of the shoulder with destruction of the pain, dysuria, and pneumaturia or may have transected include the corticospinal tract, head of the humerus. Remarkable subcutaneous no symptoms. Likewise, severity of the illness dorsal column, and spinothalamic tract. oedema of the hands referred to as “main ranges from an asymptomatic condition to life succulente” has been described. Interruption of threatening cystitis. the central autonomic pathways results in hype- Q3: What are the predisposing rhydrosis. Resorption of the head of the hu- factors? merus with a well defined margin is a character- istic radiographic finding of syringomyelia. More than 50% of patients with emphysema- tous cystitis have diabetes mellitus. Patients Final diagnosis with bladder outlet obstruction, neurogenic Syringomyelia with resorption of the humeral bladder, and recurrent urinary tract infections head representing a neuroarthropathy are at increased risk. Immunocompromised and debilitated patients are especially suscep- References tible. Females are two times more likely to be 1 Sequeira W. The neuropathic joint. Clin Exp affected than males.2 The condition most Rheumatol 1994;12:325–37. commonly results from infection with Es- 2 Soults CB, Wasenko J, Hodge C. cherichia coli but proteus, klebsiella, staphylo- Syringomyelia and related conditions. Clinical coccus, streptococcus, nocardia, and clostrid- neurology. Vol 3. Philadelphia: Harper and Row, 1996: 1–35 (chapter 45). ium have also been described in the 3 3 Zakeri A, Glasauer F, Egnatchik JG. Familial literature. Long term broad spectrum anti- syringomyelia: case report and review of the biotic therapy and indwelling Foley catheters literature. Surg Neurol 1995;44:48–53. constitute another risk group for candida related emphysematous cystitis.4 Abdominal pain in a Q4: How would you treat this diabetic myeloma patient condition? with cirrhosis Early diagnosis, strict control of the blood glucose level in diabetic patients, immediate Q1: What is the differential diagnosis? antibiotic therapy, and adequate continuous http://pmj.bmj.com/ Multiple myeloma (with low concentrations bladder drainage are the general treatment of uninvolved immunoglobulin concentra- principles. Patients must be admitted to tions), diabetes, cirrhosis, and hospitalisation for observation and proper intra- constitute major risk factors for severe infec- venous antibiotic therapy. After successful tions in this patient. Spontaneous bacterial elimination of infection, radiographic and peritonitis must always be considered for this clinical resolution usually occurs within a few Figure 1 kind of cirrhotic patient as well as the days, because carbon dioxide is readily ab- T2 weighted sagittal MRI image sorbed in human tissue.35 of the thoracic cord showing a mixed signal

possibility of a urinary tract infection, because on September 30, 2021 by guest. Protected copyright. of the marked pyuria and multiple risk It is important to differentiate emphysema- abnormality (arrowed) slightly to the left of factors. Although these two conditions are the tous cystitis from emphysematous pyelone- the cord at D10, representing the knife track most probable, all other causes of lower phritis, in which gas involves the renal paren- within the cord. abdominal pain—for example, intra- chyma, since the latter has a of abdominal abscess, perforated viscus, me- about 40% and generally requires nephrec- senteric vascular accident, and diverticulitis— tomy. In contrast surgical intervention is must be excluded, especially in this rarely needed in emphysematous cystitis uncooperative patient. except when an anatomical abnormality like an obstruction or stone is present.3 Q2: What abnormalities are seen on the radiographs? Final diagnosis The x ray film obtained after contrast en- Emphysematous cystitis. hanced computed tomography, while the References patient was excreting contrast material, shows 1 Biyani CS, Cornford PA, Scally JS, et al. the borders of the urinary bladder and gas Emphsematous cystitis. Scand J Nephrol bubbles within its wall clearly as a radiolucent 1997;31:309–10. line (fig 1; see p 375). The extent of gas collec- 2 Akalin E,HydeC,SchmittG,et al. tion can be better appreciated in the computed Emphysematous cystitis and pyelitis in a tomogram (fig 2; see p 375). The source of this diabetic renal transplant recipient. gas within the urinary tract may arise from Transplantation 1996;62:1024–6. infection, , gastrointestinal 3 Ankel F, Wolfson AB, Stapczynski JS. Figure 2 T2 weighted axial MRI image of fistulas or iatrogenic causes, such as diagnostic Emphysematous cystitis: a complication of the thoracic cord showing an area of high or surgical instrumentation. urinary tract infection occurring predominantly signal posteriorly and to the right of the cord, in diabetic women. Ann Emerg Med consistent with the track of the knife through Our patient gave no history of trauma, and 1990;19:404–6. no urinary instrumentation had been per- 4 Singh CR, Lytle WF. Cystitis emphysematosa the soft tissues (arrowhead). The lesion within formed previously. Fistulous tracts, abscess, caused by Candida albicans. J Urol the cord is seen again slightly to the left of and mesenteric occlusion can be excluded on 1983;130:1171–3. the midline (arrowed).

www.postgradmedj.com 380 Self assessment answers Postgrad Med J: first published as 10.1136/pmj.78.920.377-a on 1 June 2002. Downloaded from Q2: What is the name of this posterior column loss and the left sided after some lumbar punctures and can syndrome? Brown-Séquard syndrome, although the also occur spontaneously. The classical Brown-Séquard syndrome. point of knife entry was right sided. The features are a positional headache that is explanation is due to the oblique knife injury worse on sitting up; and photophobia Q3: Give three other causes of this with the track of the blade crossing the may also occur.4 Headaches of this nature syndrome posterior columns from the right and imping- may respond symptomatically to increased Other causes of Brown-Séquard syndrome ing in the left side of the cord (figs 1 and 2). fluid intake and the use of caffeine com- include multiple sclerosis, unilateral disc her- A classical presentation for Brown-Séquard is pounds. Occasionally epidural blood patches ipsilateral loss of the corticospinal, posterior may be useful in plugging the cerebrospinal niation, extrinsic cord lesions—for example, 4 metastases, epidural haematomata, and uni- column, sympathetic, and spinothalamic fluid leak. 1 lateral ischaemic lesions of the cord. tracts. Clinically this results in ipsilateral In this case, the patient’s low pressure pyramidal deficit with ipsilateral loss of joint headache resolved spontaneously without the Q4: What is the cause of his position, vibration, and soft touch at the level need for surgical exploration of his wound. headache? of the lesion. There is contralateral loss of The headache is a classical presentation of low pain and temperature sensation that mani- Final diagnosis pressure headache due to leakage of cerebro- fests itself a few segments below the level of Brown-Séquard syndrome. spinal fluid from a dural tear. the lesion because the decussating fibres enter the spinothalamic tract a few segments References Q5: How would you treat his rostrally to the level of entry of the nerve root. 1 Brown-Séquard CE. Recherches et headache if the symptoms persisted? Stab are a common cause of expériences sur la physiologie de la moelle 2 The recommended management for persist- Brown-Séquard syndrome with rarer causes épinière. Thèse de Paris 1846. ent low pressure headache in the context of a including primary or secondary cord tumours, 2 Koehler PJ, Endtz LJ. The Brown-Séquard of this nature would be surgical degenerative disc disease, cord ischaemia, syndrome. True or false? Arch Neurol exploration and repair of the dural defect with inflammatory or infectious conditions—for 1986;43:921–4. Peacock WJ increased fluid intake. A blood patch or oral example, herpetic infections or multiple 3 , Shrosbree RD, Key AG. A sclerosis and subdural/epidural haemorrhage review of 450 stab-wounds of the spinal cord. caffeine treatment could also be considered. 51 (reviewed in Peacock et al3). SAfrMedJ1977; :961–4. Discussion 4 Mokri B. Spontaneous cerebrospinal fluid The low pressure headache was as a leaks: from intracranial to The neurological injury is consistent with left result of continuing cerebrospinal fluid cerebrospinal fluid —evolution of 1 sided Brown-Séquard syndrome. One of the leakage from a dural tear. Low pressure a concept. Mayo Clin Proc points of interest in this case is the bilateral headaches of this nature are also reported 1999;74:1113–23. http://pmj.bmj.com/ on September 30, 2021 by guest. Protected copyright.

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