Makindo Medical Notes

Related Subjects: Type 1 DM |Type 2 DM |Diabetes in Pregnancy |HbA1c |Diabetic Ketoacidosis (DKA) Adults |Hyperglycaemic Hyperosmolar State (HHS) |Diabetic Nephropathy |Diabetic Retinopathy |Diabetic Neuropathy |Diabetic Amyotrophy |Maturity Onset Diabetes of the Young (MODY)

⚡ Cardinal symptoms: abrupt onset of severe proximal leg pain (thigh, hip, or back), followed within weeks by progressive weakness and muscle wasting.

📖 About

• Also called Bruns Garland syndrome.
• A rare neurological complication of diabetes, usually in Type 2 diabetes and older adults.
• Mainstay of management: improved glycaemic control and physiotherapy.

📊 Epidemiology

• ~1.1% of Type 2 diabetes patients.
• ~0.3% of Type 1 diabetes patients.

🧬 Aetiology / Pathophysiology

• Multifactorial: autoimmunity, metabolic derangements, microvascular insufficiency, oxidative stress, and growth factor deficiency.
• Endoneurial microvasculitis and impaired perfusion → axonal damage.
• Immune-mediated nerve injury in a metabolically stressed, genetically predisposed host.

🩺 Clinical Features

• Typical age: >50 years.
• 💥 Severe unilateral thigh/hip pain → spreads bilaterally over weeks.
• Asymmetrical proximal weakness (quadriceps, hip adductors, iliopsoas) + wasting.
• 🔥 Pain precedes weakness, often disabling.
• May have mild sensory loss (coexisting diabetic peripheral neuropathy).
• Absent knee-jerk reflexes; ankle jerks preserved unless distal polyneuropathy coexists.
• Recovery: slow, often incomplete; course lasts up to 3 years.

🧾 Differential Diagnosis

• Cauda equina syndrome.
• Guillain-Barré syndrome (AIDP).
• Spinal canal stenosis.
• Neoplastic lumbosacral plexopathy.
• Chronic inflammatory demyelinating polyneuropathy (CIDP).

🔎 Investigations

• 🧪 Blood glucose (may be presenting feature of diabetes).
• 📉 Haematinics: rule out B12/folate deficiency.
• 💉 Lumbar puncture if AIDP/CIDP suspected → CSF protein may be raised.
• ⚡ EMG & nerve conduction studies → axonal damage (or demyelination if CIDP overlap).
• 🖥️ MRI lumbosacral spine → exclude structural causes (disc, stenosis, tumour).

🛠️ Management

• 🔍 Recognise: may be first sign of undiagnosed diabetes.
• 🧩 Classification: – Axonal type → supportive care. – Demyelinating type (CIDP overlap) → may respond to IVIG, plasmapheresis, steroids, immunosuppressants.
• 💉 Strict glycaemic control (often with insulin initially).
• 🏋️ Aggressive physiotherapy & mobility support.
• 💊 Pain management: TCAs, gabapentinoids, analgesics.
• 👩‍⚕️ Neurology referral for diagnostic clarification and advanced therapies.

📚 References

• Diabetic Amyotrophy: From the Basics to the Bedside