Could Madelung Disease be a Cause of Stroke? A Rare Case Report

Multiple symmetric lipomatosis (MLS), also known as Launois-Bensaude syndrome or Madelung disease, is a rare metabolic condition characterized by generally symmetrical, non-encapsulated fat accumulations in the patient’s body. Is there a link between neurological disorders and multiple symmetric lipomatosis, as we demonstrate with our 75-year-old male patient who had an ischemic stroke and was also diagnosed with MLS? Our goal here is to provide an answer to this question.


CASE REPORT
A 75-year-old male patient was admitted to the emergency department an hour after the sudden onset of speech disorder and loss of strength in his left arm and leg.He had no known previous disease or medication.He had a history of smoking and alcohol use.There was no history of febrile illness or trauma.On physical examination, the patient's general condition was good to moderate, body temperature was normal, and vital signs were stable.On neurological examination, his speech was dysarthric, muscle strength in the left arm and leg was 2/5 hemiparetic according to the Medical Research Council (MRC) scale, his plantar reflex was absent on the left, and the patient's gait was left hemiparetic.The patient was suitable for intravenous thrombolytic (IV tPA) treatment because his National Health Stroke Scale (NIHSS) score was 10, no hemorrhage was detected on brain computed tomography, and his current complaints occurred within the first hour of their onset.Sixty-three mg of IV tPA was administered to the patient.No major artery occlusion was detected on brain-neck CT angiography.The patient was admitted to the neurology intensive care unit.In the patient's repeated ex-aminations, at the end of the 24th hour, the left upper and lower extremities were 5/5 according to the MRC scale, but dysarthria continued.
In the patient's brain magnetic resonance imaging (MRI), there was a diffusion-restricting area in the cortical-subcortical area of the right frontal lobe, extending to the lateral precentral gyrus (Fig. 1).
The patient's treatment was arranged as acetylsalicylic acid 300 mg daily, clopidogrel 75 mg and lansoprazole 40 mg daily.
During the physical examination of the patient, rhinophyma and numerous symmetrical swellings were detected in his arms and legs.The same swelling was also present in his right breast and resembled gynecomastia (Fig. 2).

Electrocardiography and echocardiogram were normal.
No significant pathology was detected in brain-neck CT angiography.
In the breast and superficial tissue ultrasound (USG), the lesions on his body were evaluated as non-encapsulated lipoma.A biopsy of the lesions was requested, but the patient refused.
The patient was discharged on the eighth day of hospitalization, with minimal dysarthria on neurological examination and a recommendation to continue his current treatment.

DISCUSSION
Multiple symmetric lipomatosis (MSL), also known as Madelung or Launois-Bensaude syndrome, is a rare syndrome with multiple lipomatous lesions, frequently seen in men aged 30-60, and whose etiology has not been fully elucidated.Alcohol use has an important place in the etiopathogenesis of the disease.When we look at the literature, there are cases with genetic transmission.3][4][5] It has been said that alcohol can affect physiological enzymatic process disorders in the mitochondria, and excess alcohol can disrupt adrenergic lipolysis and cause the appearance of uncontrolled fat deposits in different parts of the body.[3][4][5] In addition, our patient had a long history of alcohol consumption.
3][4][5] We looked at the literature, it can be seen that MSL is classified by Enzi and Donhauser.According to the classification of the Enzi, in type 1, the distribution is symmetrical and is frequently located in the neck, shoulder girdle, back, supraclavicular region and proximal upper extremities, while in type 2, accumulations occur in the abdomen and thigh regions without affecting the upper region.
4][5][6] Considering these classifications, accumulations in our patient were present both in the proximal and distal parts of the upper extremity, in the proximal and distal parts of the lower extremity, and in the right breast.Our patient did not have lipomatous lesions in the neck or back area.
It is well known that mitochondria play an important role in fatty acid production and breakdown in the process related to energy production.The sources in literature had highlighted that brown adipose tissue abnormalities could result from enzymatic mitochondrial defects that could alter the storage and utilization of fatty acids.This can trigger cellular energy deficiency and eventually neuron death.It should also be noted that mitochondrial disorders may be associated with multiple different phenotypes, thus including the development of stroke-like attacks and paralytic ileus, as previously reported. [1,7,8]Although a relationship between mitochondrial disorder and MSL could not be established because biopsy and genetic testing could not be performed in our patient, the hypothesis that it may be a stroke risk factor is also seen in the literature and is intended to be kept in mind in current patients.

Folia
Medica I 2024 I Vol.66 I No. 4

Figure 1 .
Figure 1.Acute diffusion restriction extending to the lateral side of the precentral gyrus of the right frontal lobe.

Figure 2 .
Figure 2. Multiple lipomatous lesions on the patient's right chest, arms and legs.It is not found in the head and neck area.