Rev. Med. Chir. Soc. Med. Nat., Iaşi – 2013 – vol. 117, no. 2

INTERNAL MEDICINE - PEDIATRICS CASE REPORTS

404

MARKERS OF INSULIN RESISTANCE IN A CASE OF LAUNOIS-

BENSAUDE SYNDROME

Doina-Clementina Cojocaru1,2

, Corina Dima Cozma1,3

, Paraschiva Postolache1,2

University of Medicine and Pharmacy “Grigore T. Popa” – Iasi

Faculty of Medicine

1. Ist Medical Department

Rehabilitation Clinic Hospital Iasi

2. Pulmonary Rehabilitation Clinic

3. Cardiovascular Rehabilitation Clinic

MARKERS OF INSULIN RESISTANCE IN A CASE OF LAUNOIS-BENSAUDE SYN-

DROME (Abstract): Launois-Bensaude syndrome (benign symmetric lipoma-tosis) is a rare

disease characterized by symmetric fat deposits localized in the cervical region, shoulders

and proximal parts of upper and lower limbs. We present the case of a 63-year-old male who

presented the typical location of fatty masses and a history of chronic alcoholism associated

with elements defining the metabolic syndrome. The biological profile indicated high -

atherogenic mixed dyslipidemia, high basal insulinemia (30 μU/ml), and multiple markers of

insulin resistance (Reaven index, lipid accumulation product, homeostatic model, insulin

sensitivity index, and modified glycemic curve following oral glucose load). The particular i-

ty of the presented case is the discordance between the severity of metabolic disturbances

and their clinical expression, raising the question whether this patient's cardiometabolic risk

is increased or rather lowered by the association of benign symmetric lipomatosis. Kewords:

LAUNOIS-BENSAUDE SYNDROME, INSULIN RESISTANCE.

Launois-Bensaude syndrome (benign

symmetric lipomatosis, Madelung’s disease)

is a rare disease of unknown prevalence,

characterized by symmetric fat deposits

prevalently localized around the cervical

region, in the shoulders and proximal part of

the arms and legs. The name of this disease

is chronologically disputed by Sir Benjamin

Brodie (1846), the first who described this

deformity, followed by Otto W. Madelung

(1888), a German surgeon, his name being

particularly related to the disease epony-

mous with prevalently cervical location

("Madelung’s collar or neck”); in 1898,

Raoul Bensaude and Pierre-Emile Launois

published the first series of 75 cases and

provided a full description of the character-

istic features of the disease, adopting the

term symmetric lipomatosis (1,2).

Middle-aged (30-60 years) males are

most commonly affected, developing the

characteristic distribution of body fat after

at least 10 years of abusive ethanol con-

sumption. The etiopathogenesis of benign

symmetric lipomatosis is not fully known,

the hypertrophy of malfunctioning brown

fat and the decreased activity of mitochon-

drial respiratory enzymes that may result in

lipolytic pathway depression being incrim-

inated. In all these processes, alcohol is a

Markers of insulin resistance in a case of Launois-Bensaude syndrome

405

possible co-factor. The only effective

treatment is surgery, through lipectomy

and/or liposuction of fat masses (2, 3).

CASE PRESENTATION

A 63-year-ols male from a rural area

presented to the Pulmonary Rehabilitation

Clinic of the Iasi Rehabilitation Clinic

Hospital complaining of dyspnea, mMRC

(modified Medical Research Council)

breathlessness scale grade 2, intermittent

productive cough and mild fatigability.

The patient presented to our clinic for

entering a respiratory rehabilitation pro-

gram having a previous diagnosis of mod-

erate chronic obstructive pulmonary di-

sease (COPD), the symptoms of this chron-

ic lung disease being well controlled with

inhaled tiotropium18 µg/day. He also pre-

sented grade 2 essential hypertension,

blood pressure being optimally controlled

with amlodipine 10 mg daily.

What drew our attention during physical

examination were the pseudotumoral sub-

cutaneous masses: relatively symmetrical,

located mainly in the proximal parts of the

upper and lower limbs, with a pseudohy-

pertrophic appearance, and under the chin,

behind the ear, and in the nuchal region,

resembling a Madelung’s collar (fig. 1).

Fig. 1. Relatively symmetric fat deposits in the upper half of the trunk and upper limbs

On palpation, multiple mobile and pain-

less small abdominal lipomas, like the

masses in the upper body, were detected

(fig. 2). Family history revealed that two

patient’s brothers present a similar appear-

ance; from personal history we found out

that the fat masses with a typical location

for Launois-Bensaude syndrome developed

progressively over the last 20 years. Surgi-

cal removal of one mass located behind the

right ear that became painful confirmed its

benign nature, lipoma, but resulted in local

recurrence.

The patient was a smoker (35 pack-

years) and had a history of chronic ethanol

consumption (70 international units/week,

abstinent for about 6 months); clinically,

stigmata of chronic alcoholism persisted,

represented by retraction of palmar apo-

neurosis (Dupuytren’s contracture), bilat-

Doina-Clementina Cojocaru et al.

406

eral parotid gland hypertrophy, telangiec-

tasias on the nose and cheekbone, but

laboratory tests confirmed abstinence,

serum transaminases, gamma-glutamyltrans-

peptidase and mean corpuscular volume

being normal.

Fig. 2. Abdominal lipomas (left) and under the chin (right)

Patient’s anthropometric data showed a

body mass index of 30.14 kg/m2, corre-

sponding to grade I obesity, and a patholog-

ical waist circumference of 115 cm, meas-

ured in standing position halfway between

the costal margin and iliac crest line.

The metabolic profile revealed mixed

dyslipidemia at high atherogenic risk (total

cholesterol = 6 mmol/l, HDL-cholesterol = 0.9

mmol/l, triglycerides = 1.64 mmol/l, LDL-

cholesterol = 4.32 mmol/l), normal glucose

levels (4.93 mmol/l, and high basal insuline-

mia (30 μU/ml), indicator of insulin resistance.

Given the presence of basal hyper-

insulinemia, we calculated the markers of

insulin resistance derived from previous

biological and anthropometric measure-

ments (tab. I).

Also a 3-hour 75-gram oral glucose tol-

erance test was performed (fig. 3).

TABLE I

Markers of insulin resistance

MARKERS OF INSULIN

RESISTANCE

RESULTS COMMENTS

TG/HDL ratio (Reaven index) 4.17 Reaven index ≥ 3,5 indicates insulin re-

sistance (4)

LAP (lipid accumulation product) 82 cm. mmol/l

Lipid accumulation product correlates well

with insulin resistance and metabolic syn-

drome (5)

HOMA-IR (homeostatic model

assessment of insulin resistance) 5.33

Values ≥ 2.6 indicate insulin resistance sta-

tus; ≥ 4 it is considered prediabetic status (6)

QUICKI( quantitative insulin

sensitivity check index) 0.29

Low values (< 0.45) suggest insulin re-

sistance ; < 0.3 in diabetics (7)

Markers of insulin resistance in a case of Launois-Bensaude syndrome

407

Fig. 3. Evolution of glycemia over the 3-hour 75-g oral glucose load

DISCUSSION

The patient presented a rare disease,

benign symmetric lipomatosis, associated

with metabolic syndrome, defined in this

case by three factors: hypertension, ab-

dominal obesity and low HDL-cholesterol,

serum triglycerides being at the limit of

diagnostic significance and basal glycemia

levels within normal range (8). Simultane-

ous determination of basal insulinemia and

calculation of some markers of insulin

resistance (tab. I) revealed frankly patho-

logical values, allowing the assertion of a

hyperinsulinemic status associated with

insulin resistance.

Remarkable is the discordance between

the severity of metabolic disturbances and

their clinical expression: the associated

pathological conditions (hypertension,

COPD) were easily controlled therapeuti-

cally and glucose metabolism disturbances

were not very obvious. Thus, basal glyce-

mia and the values of oral glucose toler-

ance test (fig. 3) were normal at fasting or

at 2 hours, but prolonged monitoring for

three hours revealed a late hypoglycemic

dip, result of an abnormal overproduction

of insulin in response to glucose load.

The particularity of this case is related

to whether the patient's cardiometabolic

risk, consequence of the accumulation of

risk factors - male, smoking status, hyper-

tension, chronic alcohol consumption, obe-

sity - is increased or rather lowered by the

association of Launois-Bensaude syn-

drome, having in view the mild clinical

course despite the severity of underlying

metabolic changes?

Review of literature data on Launois-

Bensaude syndrome reveals a quite wide

phenotypic and clinical variability in this

category of patients, ranging from a type of

"metabolically innocent" obesity with in-

creased insulin sensitivity by decreased

visceral adiposity ascertained by computer

tomography (9), to a metabolic picture

dominated by insulin resistance, particular-

ly in those patients associating cervical

lipomatose deposits and an obstructive

sleep apnea syndrome, in its turn generat-

ing insulin resistance (10).

The presented case seems to be halfway

between these two extremes, but the lack of

large studies and validation of any of the

models does not allow drawing an indis-

putable conclusion.

Doina-Clementina Cojocaru et al.

408

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PELTOPHORUM AFRICANUM: ANTIMICROBIAL POTENTIAL AND HEPATOTOXICITY

The antimicrobial activity and hepatotoxicity of Peltophorum africanum were evaluated in a

study by Okeleye et al. Agar well and macrodilution methods were used for antimicrobial

activity testing, as well as CellTiter-Blue cell viability assay to assess the toxicity on human

liver cells. The results showed both bactericidal and bacteriostatic or fungistatic activity of

the extract. The researchers observed good activity against Plesiomonas shigelloides. De-

spite its antimicrobial activity, the extract was toxic to human liver cell lines. Lethal dose at

50 revealed 82.64 ± 1.40 degree of toxicity at 24 hrs, while 95 percentile of cell death dose

activity varied between log 3.12 and 4.59. (Okeleye BI, Mkwetshana NT, Ndip RN. Evalua-

tion of the Antibacterial and Antifungal Potential of Peltophorum africanum: Toxicological

Effect on Human Chang Liver Cell Line. ScientificWorldJournal 2013; 2013: 878735. doi:

10.1155/2013/878735).

Teodora Vremera

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