Diabetic Neuropathic Cachexia and Mauriac Syndrome: A Rare Complication of Poorly Controlled Diabetes Mellitus

Adel Djermane; Abdennader Fafa; Yasmina Ouarezki; Asma Ghedjati; Lynda Kermi; Laziz Atek; Radjaa Arous; Yasmine Semar; Samira Aggoune; Hachemi Maouche

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ESPE Abstracts (2024) 98 P3-53

ESPE2024 Poster Category 3 Diabetes and Insulin (36 abstracts)

Diabetic Neuropathic Cachexia and Mauriac Syndrome: A Rare Complication of Poorly Controlled Diabetes Mellitus

Adel Djermane ^1,2 , Abdennader Fafa ^1,2 , Yasmina Ouarezki ^1,2 , Asma Ghedjati ² , Lynda Kermi ^1,2 , Laziz Atek ^1,2 , Radjaa Arous ^1,2 , Yasmine Semar ^1,2 , Samira Aggoune ^1,2 & Hachemi Maouche ^1,2

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¹Algiers University 1, Algiers, Algeria. ²Public Hospital, El Harrach, Algeria

Background: Diabetic neuropathic cachexia is a rare syndrome of acute neuropathic pain and profound weight loss. Mauriac syndrome has rarely been reported in children and adolescents with a poorly controlled diabetes mellitus type 1. Common presenting features include short stature, growth retardation, moon facies, protuberant abdomen, and proximal muscle wasting. We report the first case of the association of Mauriac Syndrome and Diabetic neuropathic cachexia in 13-year-old female.

Case Report: A 13-year-old female, referred to us for evaluation of short stature, delayed puberty and leg pain. Patient also had decreased vision and tingling and pain sensation in the lower limbs since one month. She was diagnosed to have type 1 diabetes mellitus at 8 years, and had been severely under insulinized. However, compliance as well as sugar monitoring was very poor. On examination, patient was short statured (132 cm, -3.62SDS), her BMI was 15.36 less then 3rd percentile, she muscles wasting and delayed puberty (stage 1). Investigations showed normal hemogram, normal liver and renal function test. Ultrasound abdomen revealed hepatomegaly and nephrocalcinosis. the ophthalmological examination showed poor visual acuity and glaucoma. Her premeal blood sugar was 2,40g/l, HBA₁C was 12.5%, microalbuminuria =21 µg/mL (normal < 18 µg/mL). Patient also found to have gastroparesis on fundus examination. Based on history, examination and investigation findings, final diagnosis of Mauriac syndrome was made. Patient was shifted to strict dietary management high dose insulin, metformin and vitamin D3. The neuropathic pain was managed by amitriptyline. After 2 months of therapy, patient improved symptomatically, premeal sugar came down, there was reduction in hepatomegaly and spectacular resolution of neurological pain.

Conclusion: In this case, poor compliance, monitoring un low dose of insulin was the main cause behind the poorly controlled diabetes and the occurrence of a Mauriac Syndrome, Glaucoma and neurological pain who was succefuly managed by antidepressant drug, metformin and high dose of insulin.