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Stiff man syndrome

First described by Moersch and Woltman at the Mayo Clinic in 1956, stiff person syndrome (SPS) is a rare neurologic disorder of unknown etiology. Those with the illness experience progressive, fluctuating tonic contractions of all muscles, particularly the axial musculature. Inability to walk and paralysis quickly ensues; death usually occurs six to twelve months after diagnosis. more...

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Treatment is mostly palliative with muscle relaxants such as benzodiazepines, which lose their effectiveness as the illness progresses. Because many patients with SPS have circulating antibodies to glutamic acid decarboxylase, an autoimmune genesis to the disease has been postulated. In the absence of double-blind, placebo-controlled class A trials to determine treatment efficacy, some authorities recommend humane trials of immunosuppressive therapy, plasmapheresis or intravenous immunoglobulin infusion.

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A spontaneous compartment syndrome in a patient with diabetes
From Journal of Bone and Joint Surgery, 9/1/04 by Jose, R M

A compartment syndrome is an orthopaedic emergency which can result from a variety of causes, the most common being trauma. Rarely, it can develop spontaneously and several aetiologies for spontaneous compartment syndrome have been described. We describe a patient with diabetes who developed a spontaneous compartment syndrome. The diagnosis was delayed because of the atypical presentation.

Compartment syndrome is defined as an elevation of the interstitial pressure in a closed osteofascial compartment causing microvascular compromise. The common causes include trauma, arterial injury, limb compression and burns. Rarely, it can also occur spontaneously in association with type-I diabetes mellitus,1-4 hypothyroidism,1 influenza-virus-induced myositis,6 leukaemic infiltration, the nephrotic syndrome,8 a ruptured aneurysm,9 anticoagulation10 and a ganglion cyst.11 Four cases of spontaneous compartment syndrome in diabetics have been described previously and many theories regarding the aetiology have been advanced, including metabolic changes giving rise to increased fluid pressure in the osteofascial compartment, vascular occlusion and muscle necrosis.

Case report

A 47-year-old man of Asian origin developed pain in the anterolateral aspect of the left leg after a brief walk. It was moderate in intensity but was not relieved by rest. He had suffered from type-I diabetes mellitus, well controlled on insulin, for almost 20 years. He was also hypertensive and was undergoing laser treatment for diabetic retinopathy.

He attended the Emergency Department with a localised red, tender area over the upper lateral aspect of the left leg below the knee. No definite diagnosis was made and he was given analgesics and discharged. The pain was not relieved and he was prescribed stronger analgesics by his general practitioner. The pain increased in intensity over the next four days and he developed foot drop. He was seen again and referred for an orthopaedic opinion.

There was swelling, redness and tenderness over the anterolateral aspect of the left leg. He had normal sensation but was unable to dorsiflex his foot. Both the dorsalis pedis and posterior tibial pulses were present. The differential diagnoses were an intrafascial bleed, infection, spontaneous muscle necrosis or a compartment syndrome.

Haematological investigation revealed a mild leukocytosis (12.8 × 10^sup 9^/1). Biochemical analysis was normal except that the level of creatine kinase was increased to 4178 U/l, raising the suspicion of muscle necrosis and a compartment syndrome. Decompression of the anterior and lateral compartments was carried out. The muscles were found to bulge beneath the deep fascia and the compartmental pressure was raised. Both muscle groups appeared to be ischaemic and did not respond to pinching. The pain persisted and he was taken back to theatre after two days. Necrotic parts of tibialis anterior were excised and sent for histological examination. The wound was left open and dressed regularly. At one week it was closed secondarily, without a skin graft.

Histological examination of the excised specimen showed areas of devitalised skeletal muscle without evidence of inflammation. There were some viable atrophie muscle fibres (Fig. 1 ) with blood vessels showing thrombus and recanalisation (Fig. 2).

He was reviewed in the Outpatient Clinic after two weeks when his wound had healed. There has been no improvement in the foot drop. He continues to attend for physiotherapy and a tendon transfer is being considered.

Discussion

Spontaneous compartment syndrome has been reported in influenzal myositis, hypothyroidism, leukaemic infiltration, nephrotic syndrome, vascular anomalies, anticoagulant therapy and cystic lesions.5-11 There have been four other case reports of spontaneous compartment syndrome in diabetes mellites.1-4

In 1997 Chautems et al1 described a similar case when the patient was operated on within eight hours of the onset of symptoms. He suffered no neurological deficit. Smith and Laing2 reported a case of bilateral compartment syndrome in a diabetic patient who presented to the Emergency Department after four days. He was found to have muscle necrosis, a bilateral sensory deficit in the distribution of the deep peroneal nerve, and a foot drop. The delay in the diagnosis of compartment syndrome in our patient may be excused by its atypical presentation. Initially, he had localised swelling and only moderate pain. Absence of pain has been reported previously by Ciacci et al,12 who suggested a possible neurapraxic block of the deep peroneal nerve as an explanation.

There are two conflicting views regarding the development of spontaneous compartment syndrome in diabetics. One suggests that metabolic disturbances cause osmotic accumulation of fluid in the muscle which may be the primary event leading to increased pressure.1 The muscle necrosis develops as a result of the ischaemia.14 The other view is that spontaneous muscle infarction, because of microvascular blockage, is the primary event and that compartmental pressures rise subsequent to that."'4 We prefer the latter explanation since our patient had a localised swelling initially and the symptoms progressed over several days. The histopathology of the excised muscle showed thrombi in the small blood vessels with attempts at recanalisation (Fig. 2). A relevant coincidence is that our patient, and two other reported patients, had diabetic retinopathy which suggests coexisting microvascular disease. There have been other recorded cases of spontaneous muscle infarction in diabetics. They are common in type-I diabetes and are strongly associated with other microvascular complications such as neuropathy, retinopathy and nephropathy. 15 The usual presentation has been a swelling in the muscles of the thigh and the treatment has mostly been conservative.16,17 Since the compartment in the calf is smaller and tighter, swelling within it can easily result in a compartment syndrome. Early surgery is more likely to be curative.

No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.

References

1. Chautems RC, lrmay F, Magnin M1 Morel P, Hoffmeyer P. Spontaneous anterior and lateral tibial compartment syndrome in type 1 diabetic patient: case report. J Trauma 1997;43:140-1.

2. Smith AL, Laing PW. Spontaneous compartment syndrome in Type 1 diabetes mellitus. Diabet Med 1999; 16:168-9.

3. Lecky B. Acute bilateral anterior tibial compartment syndrome after caesarian section in a diabetic. J Neuml Neumsurg Psychiatry 1980:43:88-90.

4. Parmoukian VN, Rubino F, Iraci JC. Review and case report of idiopathic lower extremity compartment syndrome and its treatment in diabetic patients. Diabetes Metab 2000:26:489-92.

5. Hsu SI, Thadhani RI, Daniels GH. Acute compartment syndrome in a hypothyroid patient. Thyroid 1995:5:305-8.

6. Paletta CE, Lynch R, Knutsen AP. Rhabdomyolysis and lower extremity compartment syndrome due to influenza B virus. Ann Plast Surg 1993:30:272-3.

7. Veeragandham RS, Paz IB, Nadeemanee A. Compartment syndrome of the leg secondary to leukemic infiltration: a case report and review of literature. J Surg Oncol 1994:55:198-200.

8. Sweeney HE, O'Brien F. Bilateral anterior tibial compartment syndrome in association with nephrotic syndrome: report of a case. Arch Intern Med 1965:116:487-90.

9. Hasaniya N, Katzen JT. Acute compartment syndrome of both lower legs caused by ruptured tibial artery aneurysm in a patient with polyarteris nodosa: a case report and review of literature. J Vase Surg 1993:18:295-8.

10. Griffiths D, Jones DH. Spontaneous compartment syndrome in a patient on longterm anticoagulation. J Hand Surg [Br] 1993;18:41-2.

11. Ward WG, Eckardt JJ. Ganglion cyst of the proximal tibiofibular joint causing anterior compartment syndrome. J Bone Joint Surg [Am] 1994;76-A:1561-4.

12. Ciacci G, Federico A, Giannini F, et al. Exercise-induced bilateral anterior tibial compartment syndrome without pain, Ital J Neurol Sci 1986:7:377-80.

13. Coley S, Situnayaki RD, Alien MJ. Compartment syndrome, stiff joints, and diabetic cheiroarthropathy. Ann Rheum Dis 1993:52:840.

14. Chester CS, Banker BWQ. Focal infarction of muscle in diabetics. Diabetic Care 1986:9:623-30.

15. Grigoriadis E, Fam AG, Starok M, Ang LC. Skeletal muscle infarction in diabetes mellitus. J Rheum 2000:27:1063-8.

16. Lauro GR, Kissel JT, Simon SR. ldiopathic muscular infarction in a diabetic patient. J Bone Joini Surg [Am] 1991:73-A:301 -4.

17. Banker BQ, Chester CS. Infarction of the thigh muscle in the diabetic patient. Neurology 1973:23:667-77.

R. M. Jose, N. Viswanathan, E. Aldlyami, Y. Wilson, N. Moiemen, R. Thomas

From Department of Plastic Surgery, Selly Oak Hospital, Birmingham, UK

* R. M. Jose, MB BS, MCh, FRCS, Senior House Officer

* N. Viswanathan, MB BS, FRCS, Registrar

* E. Aldlyami, MBChB, MRCS, Senior House Officer

* Y.Wilson, MBChB, FRCS, Consultant

* N. Moiemen, MBBCh, FRCS, Consultant

Department of Plastic Surgery, Selly Oak Hospital, Birmingham B29 6JD, UK.

* R.Thomas, MBBS, MRCS, LRCP, Consultant

Department of Trauma and Orthopaedics, New Cross Hospital, Wolverhampton WV10 0QP, West Midlands, UK.

Correspondence should be sent to Mr R. M. Jose.

©2004 British Editorial Society of Bone and Joint Surgery

doi:10.1302/0301-620X.86B7. 14770 $2.00

J Bone Joint Surg IBr] 2004;86-B:1068-70.

Received 9 July 2003; Accepted after revision 16 October 2003

Copyright British Editorial Society of Bone & Joint Surgery Sep 2004
Provided by ProQuest Information and Learning Company. All rights Reserved

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