1. Neuromuscular disorder in Diabetes mellitus
R3 Kim Hyun Soo

2. Differential diagnosis of painful swelling of an extremity in a patient with diabetes
Infection
Inflammatory
Vascular
Neoplastic
Miscellaneous
Cellulitis
Polymyositis dermatomyositis
Deep venous thrombosis
Benign: lipoma, fibroma, or leiomyoma
Diabetic muscle infarction
Soft tissue abscess
Myositis, inclusion body
Haemorrhage (haematoma)
Malignant: liposarcoma
Calcific uraemic arteriolopathy
Necrotising fasciitis
Thrombophlebitis
Neurological
Trauma
Osteomyelitis with soft tissue involvement
Arterial occlusion
Diabetic
lumbosacral radiculoplexopathy (amyotrophy)
Drug induced (statins)
Parasitic infestation
Post traumatic pseudoaneurysm
Bruns-Garland syndrome
Rhabdomyolysis
BMJ 2009;338

3. Diabetes muscle infarction

4. Epidemiology
The first case was reported in 1965 by Angervall and Stener.
Reported 86 cases till 2001.
65 patients : type 1 diabetes, 19 patients: type 2 diabetes, patients: undetermined
The male/female ratio: equal (44/42), In some article more common in female
Age range of 19–81 years
Longstanding diabetes and extensive end-organ damage due to microvascular disease
Ann Rheum Dis 2001;60:310–312
Am J Med. 1996; 101:

5. Clinical presentation
Atraumatic swelling of the limb, most commonly the thigh. Quadriceps(83.7%), Vastus lateralis(24%), vastus medialis(22%), calf involvement(19.28%)
The onset of pain is usually abrupt, but can be gradual.
The local swelling, palpable painful mass
Fever was present in 10%
No skin color change
The white cell count and the level of CK are normal or slightly raised.
Often associated with microvascular complications (nephropathy, retinopathy, or neuropathy) of diabetes
Ann Rheum Dis 2001;60:310– DIABETES CARE, 26, NUMBER 1, JANUARY 2003

6. Clinical presentation
DIABETES CARE, 26, NUMBER 1, JANUARY 2003

7. pathophysiology Unclear,, several hypothesis
Most likely hypothesis- vascular disease such as arteriosclerosis and diabetic microangiopathy
Alteration in the coagulation-fibrinolysis system, in the form of hypercoagulability
Palmer and Greco
Gargiulo et al.
Galtier-Dereure et al.
hypoxia-reperfusion injury- Silberstein, Britton, Marsh, et al.
antiphospholipid antibodies
DIABETES CARE, 26, NUMBER 1, JANUARY 2003/ Ann Rheum Dis 2001;60:310–312

8. Investigations and diagnosis
Combination of clinical presentation and radiological imaging
The most valuable diagnostic technique is MRI.
Increased signal intensity in T2-weighted MRI images
with marked muscle oedema extending into the perifascicular and subcutaneous tissues
Radiographic films, US, CT, gallium scintigraphy
Needle electromyography of the muscle
Biopsy
Figure 3. The group of necrotic skeletal muscle fiber (left) and
regenerating myofibers within fibrovascular connective tissue
DIABETES CARE, 26, NUMBER 1, JANUARY 2003

9. prognosis
The short-term prognosis of diabetic muscle infarction is good
Recurrence has been reported in a total of 55 cases (47.82%): 10 cases (8.69%) involving the originally affected muscle and 45 cases (39.13%) involving another muscle.
Long term prognosis is poor
DIABETES CARE, 26, NUMBER 1, JANUARY 2003

10. management Bed rest and analgesics
Nonsteroidal anti-inflammatory drugs, glucocorticoids, aspirin, and pentoxyphylline
Some authors- anticoagulant agents
DIABETES CARE, 26, NUMBER 1, JANUARY 2003

11. Diabetic Muscle Infraction: An unusual cause of muscle pain in a diabetic patient on hemodialysis
A 36-year-old diabetic woman referred to our clinic with severe pain in the left anteromedial thigh. She had a 15-year history of Type 2 diabetes mellitus (DM). She was complicated by diabetic nephropathy and requiring hemodialysis.
Clinical and laboratory evaluation, and muscle biopsy revealed the diagnosis of muscle infarctions. She did no respond to the conservative therapy. Pain and swelling in her thigh worsened progressively. She underwent surgical debridment and then, her clinical status improved.
International Urology and Nephrology (2005) 37:629–632

12. Diabetic lumbosacral radiculoplexopathy (amyotrophy)

13. overview of diabetic neuropathy
involvement of peripheral and autonomic nervous system
 probably the most common complication of diabetes
occurs in ~50% of individuals with long-standing type 1 or type 2 DM
Prevalence is a function of disease duration and of glycemic control.
BMI and smoking are known to be another risk factors.

14. Risk factors

15. Classification

16. DLRPN subtype of diabetic polyradiculopathy
aka Bruns-Garland syndrome, diabetic myelopathy, diabetic amyotrophy, diabetic mononeuritis multiplex, diabetic polyradiculopathy, femoral or femoral–sciatic neuropathy of diabetes, diabetic motor or paralytic neuropathy, proximal diabetic neuropathy
Involvement : nerve root, lumbosacral plexus, peripheral nerve
most common type: high lumbar radiculopathy involving L2, L3 and L4 roots

17. Comparison DLRPN Polyneuropathy Onset Abrupt Insidual Location Focal
Diffuse
Initial symptome
Unilateral
Symmetrical
Complication
Rare
Often
Glycermic control
Well controlled
Not well controlled
Weight loss
Frequent
Not frequent
DM duration
Short
Long

18. Clinical features
initially asymmetrical/unilateral involvement in hip and thigh
 quickly spreading to unaffected/contralateral side
Although pain is the most prominent early symptom, weakness soon becomes the major symptom.
The average of bilateral disease: 3 months
Early wasting of Quadriceps muscleatrophy
Autonomic impairment such as orthostatic intolerance and sphincter control
Muscle Nerve 25: , 2002 , Neurol India. 2008;56(4):420-5

19. Prognosis
Although substantial improvement could be expected, recovery is usually delayed and incomplete.
Persistent pain and weakness tend to remain.
m/c long-term problem : foot drop
Jeniffer et al. 33 patients with DLRPN,
only one did not develop bilateral involvement
one-half require the use of a wheelchair
16 require ongoing assistive aids at long-term F/U (2 years)
Pascoe et al. 21 patients
three years from the onset of symptoms
12 had resumed normal walking, seven ambulated with an aid and two were wheelchair-bound
Phys Med Rehabil Clin N Am Mayo Clin Proc 1997;72:1123-3

20. pathophysiology an immune attack and probably microvasculitis of nerve
some degree of inflammation, mostly surrounding epineurial microvessels
inflammatory cells disrupted vessel walls, possibly causing microvasculitis
evidence of bleeding due to vessel
 damage: hemosiderin-laden macrophage
Muscle Nerve 25: , 2002

21. pathophysiology A B C D E F Muscle Nerve 25:477-491, 2002 normal
microvasculitis D E F
Muscle Nerve 25: , 2002

22. Diagnosis
mainly based upon the presence of suggestive clinical features
MR-r/o compression, hematoma, tumor
electrodiagnositic studies
nerve conduction study
reduced amplitude of the muscle/sensory nerve action potential
only mild slowing of conduction velocity
needle electromyelography (EMG)
Acutefibrillation potential, positive shock wave, decreased motor unit recruitment
Chronichigh amplitude, long duration motor unit action potential
Mayo Clin Proc 56: , 1981

23. treatment Immune suppression Symptomatic treatment
narcotic medications (TCA or anti-seizure agents)
anti-depressants may be helpful.
exercise, gait training, equipment/orthotics

24. Immunotherapy A retrospective study by Pascoe et al (1997)
Treated or untreated with immunosuppressive agents (either steroids, intravenous immune globulin or plasma exchange).
higher rate of improvement (9 of 12 patients) in the treated group compared to the untreated group (17 of the 29 patients )
Mayo Clin Proc 1997;72(12):1123–32.

25. Immunotherapy
A randomized double-blinded placebo-controlled prospective treatment trial Seventy-five patients
placebo or IV methylprednisolone
at baseline, and at weeks 1, 6, 12, 24, 36, 52, and 104.
The primary outcome -time to improvement in NIS(LL) by four points.
No significant difference between the two groups in time to improvement in the NIS(LL) by four points
the decision to initiate immunosuppression
individualized considering the clinical presentation, relative acuity and time between symptom onset (less than three months), extent of disability and severity of symptoms, as well as the risks and benefits in a given patient
Neurology 2006;662):A191