LETTERSTable 1. Questions asked of spouse and distribution of responses

1. What do you think when your partnerdoes not show up at an appointed time?

Sure that s/he is suffering from hypoglycaemia 17.3 %Concerned that something might have 60.3 %happened, e.g. accidentOther thoughts 22.4 %

2. What is your emotional reaction to severehypoglycaemia?

Consternation 67.3 %Keep relatively calm 31.7 %

3. Is the potential of a severe hypoglycaemia a familyburden?

Always 9.1 %Sometimes 47.3 %Never 41.8 %

the possibility of severe hypoglycaemiawas ‘always’ a burden.

Further research is needed to determinewhether some spouses and marriages aremore vulnerable to psychosocial problemssecondary to recurrent severe hypoglycae-mia. Clinical experience suggests that, forsome couples, such hypoglycaemia stresscan perpetuate problems with severehypoglycaemia. For example, spousalconcern about future severe hypoglyca-emic episodes can trigger attempts to takemore control over diabetes management,with more resistance to treatment assist-ance in patients.8 Couples who demon-strate such power struggles should bereferred for marital therapy to addressthese issues and improve their ability tocope with hypoglycaemia.

We would expect that spouses, likepatients,3 develop more fear of hypoglyca-emia the more traumatic its past conse-quences. Severe hypoglycaemia may havesimilar psychosocial ramifications for chil-dren who have discovered their diabeticparents stuporous or unconscious and inneed of emergency treatment. We believethat the psychosocial impact of hypoglyca-emia on family members deservesincreased clinical and empirical attention.

M. Stahl,1 W. Berger,1 H. Schaechinger,1D.J. Cox2

1Division of Endocrinology, Diabetologyand Clinical Nutrition, University ofBasel, Switzerland2Department of Psychiatric Medicine,University of Virginia Health SciencesCenter, Charlottesville, Virginia, USA

References

1. The Diabetes Control and Compli-cations Trial Research Group. Influ-

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ence of intensive diabetes treatmenton quality-of-life outcomes in TheDiabetes Control and ComplicationTrial. Diabetes Care 1996; 19: 195–203.

2. Gold AE, MacLeod KM, Frier BM.Frequency of severe hypoglycaemiain patients with Type 1 diabetes withimpaired awareness of hypoglycae-mia. Diabetes Care 1994; 17: 697–703.

3. Irvine A, Cox DJ, Gonder-FrederickLA. The fear of hypoglycaemia scale.In. Bradley C, ed., Handbook ofPsychology and Diabetes. Nether-lands: Harwood Academic Pub-lishers, 1994: 133–155.

4. Lorenz R, Siebert C, Cleary P, SantiagoJ, Heyse S, for the DCCT ResearchGroup. Epidemiology of severe hypo-glycaemia in the DCCT. Diabetes1988; 37: 3A.

5. Clarke WL, Gonder-Frederick LA,Miller S, Richardson T, Snyder A.Maternal fear of hypoglycaemia intheir children with insulin-dependentdiabetes mellitus. J Pediatr Encodcri-nol Metab 1998; 4: 189–194.

6. Gonder-Frederick LA, Cox DJ, Kovat-chev B, Julian D, Clarke W. Theimpact of severe hypoglycaemic epi-sodes in patients with IDDM onspouses’ psychosocial status andmarital relationships. Diabetes Care1997; 20: 1543–1546.

7. Cox DJ, Gonder-Frederick LA, Polon-sky W, Schlundt D, Julian D, ClarkeW. A multi-center evaluation of bloodglucose awareness training-II. Dia-betes Care 1995; 18: 523–528.

8. Polonsky WH. Besieged by the dia-betes police. Diabetes Forecast 1995;12: 21–26.

Panhypopituitarism, Neurosensory Deaf-ness and Noonan’s Syndrome in a Childof a Diabetic Mother: Role of MaternalHypoglycaemia during Pregnancy inInduction of Congenital Lesions

We describe a Type 1 diabetic patientwith severe and recurrent hypoglycaemicepisodes during her pregnancy who gavebirth to a child with severe lesions. Thepatient is a 28-year-old woman with Type1 diabetes mellitus diagnosed at the ageof 3 years. Her knowledge of diabeteswas good and, during the course of herdisease, glycated haemoglobin measure-ments have usually been within or closeto the reference levels. She never smoked.Her first two pregnancies and deliverieswere without complications.

At the beginning of her third pregnancyat the age of 27 years she had no signsof nephropathy, normal blood pressurelevels, and fundal photographs demon-strated only solitary dot haemorrhages.During the pregnancy she mesaured herblood glucose concentration 4–6 timesdaily. From the start of gestational week6 and to the end of week 11, 41 of 120registered blood glucose concentrationswere #4 mmol l−1 and 19 #2 mmol l−1.HbA1C levels from week 6 to week 16decreased from 6.3 % to 4.2 %, the lowerreference level in the non-diabetic rangein our laboratory. The levels were lowerthan in the previous two pregnancies. Shesuffered five hypoglycaemic comas, hadmarked tremor in connection with onehypoglycaemic episode and experiencedpronounced tiredness during 13 furtherhypoglycaemic episodes. After 38 weeksof pregnancy she gave birth to a femaleinfant with a weight of 3.250 kg, a lengthof 47 cm and head circumference of38 cm. The infant cried at once, and theApgar scores were 8, 8, 9, respectively.Her initial blood glucose concentrationwas low (1.4, 0.9, and 1.6 mmol−1) andthe hypoglycaemia lasted for 40 hours. Inaddition obstipation and gastric retentionwere found. Non-immunological icterusalso developed. Further investigationsrevealed panhypopituitarism in the infant.Computer tomography of the brain andpituitary demonstrated no pathologicalmorphology. l-Thyroxine, cortisol, andgrowth hormone replacement was startedat day 10 postnatally. Later she requiredhearing aids for congenital neurosensorydefects and spectacles because of myopia.At the age of 5 years, she has moderatepsycho-motor retardation and has beendiagnosed with Noonan’s syndrome.1 Thediagnosis is based on the findings of shortneck stature, hypertelorism, characteristicfacial changes, mild mental retardation,and peripheral pulmonary stenosis.

Noonan’s syndrome can occur spon-taneously, occasionally in associationwith pituitary hormonal deficiencies, and

LETTERS

Figure 1. Transverse T1-weighted SE image obtained after intravenous injection ofcontrast material shows a central dark, non-enhanced area inside the right enlargedquadriceps muscle with surrounding enhancement. Linear enhanced signal infiltrates(arrows) are present through the dark core and tissue planes are preserved. R=right

could arise coincidentally in the baby ofa mother with diabetes.

In this particular case, the mother hadexperienced recurrent severe hypoglycae-mia during early pregnancy which couldhave been of importance.

The increase in perinatal morbidity andmortality in diabetic pregnancy is wellknown and has been shown to be associa-ted with maternal hyperglycaemia.2 Evi-dence that maternal hypoglycaemia maycause human fetal lesions is scarce. In areview of eight pregnant women whowere subjected to insulin-induced hypog-lycaemic comas for treatment of psychi-atric diseases it was found that twodelivered normal children, four maceratedfetuses, and two mentally retarded chil-dren of whom one had skeletal skullanomalies and atrophy of the optic nerve.3In animal experimentation, pharmacologi-cal doses of insulin administered to preg-nant animals were associated with fetalstructural damage and increased fetalresorption rate.4,5 Preventing the hypogly-caemia in the insulin-treated rats nor-malized embryonic development and invitro addition of pharmacological dosesof insulin to normoglycaemic serum didnot cause malformations in the culturedembryos.6

These data do raise the possibility thatsevere recurrent hypoglycaemia may betoxic to fetal development.

C.-J. Tornhage1, F. Lithner2,U. J. Eriksson3

1Department of Pediatrics and 2Depart-ment of Medicine, University Hospital,Umeå, and 3Department of Medical CellBiology, University of Uppsala, Sweden

621LETTERS

1998 by John Wiley & Sons, Ltd. Diabet. Med. 15: 619–622 (1998)

References

1. Noonan JA, Ehmke DA. Associatednoncardiac malformations in childrenwith congenital heart disease. J Pediatr1963; 63: 468–470.

2. Hansson U, Persson B, Thunell S.Relationship between haemoglobinA1C in early Type 1 (insulin-dependent)diabetic pregnancy and the occurrenceof spontaneous abortion and fetal mal-formation in Sweden. Diabetologia1990; 33: 100–104.

3. Wickes JG. Fetal defects followinginsulin coma therapy in early preg-nancy. Br Med J 1954; II: 1029–1030.

4. Smithberg M, Runner MN. Teratogeniceffects of hypoglycemic treatments ininbred strains of mice. Am J Anat1963; 113: 479–489.

5. Hannah RS, Moore KL. Effects of fastingand insulin on skeletal developmentin rats. Teratology 1971; 4: 135–140.

6. Sadler TW, Horton WE Jr. Effects ofmaternal diabetes on early embryogen-esis: the role of insulin and insulintherapy. Diabetes 1983; 32: 1070–1074.

Diabetic Muscle Infarction: a DifficultDiagnosis Suggested by Magnetic Reson-ance Imaging

We report the case of a 22-year-oldwoman with Type 1 diabetes mellitus of6 years’ duration and anorexia nervosawho was admitted to our unit because ofhypoglycaemic coma. She had a bedsoreof the right buttock, infected by entero-coccus faecalis. Her diabetes was poorly

controlled (HbA1c 10 %) and was compli-cated by cardiac and gastrointestinal auto-nomic neuropathy, cardiac failure, andproliferative retinopathy. One morning,the patient awoke with pain in the rightthigh which was swollen (maximal cir-cumference of 41 cm, versus 38 cm onthe left side). The calves were painlessand Homans’s sign was absent. Thelaboratory tests showed a C-reactive pro-tein of 33 mg dl−1 and a white-cell countof 12 000 mm−3. Creatine kinase levelswere normal. The Doppler study of theright leg was normal. The pain worsenedand a first MRI study of the right thighsuggested soft tissue infection on the basisof the hypersignal of the quadricepsmuscle on T2-weighted images and diffuseenhancement on post-contrast T1weighted images with central and focaldark areas. The evolution worsened anda second MRI scan revealed significantenlargement of the muscle with the samecharacteristics on T2-weighted images.On T1-weighted images after gadoliniuminjection (Figure 1), the unhanced centralcore within the muscle appeared largerwith a surrounding enhanced rim, mim-icking a muscular abscess. However,linear enhanced signal infiltrates werepresent through the dark core. On clinical,biological and radiological basis, apyomyositis was suspected and surgicalintervention occurred. The surgeon didnot find an abscess but necrotic tissue. Thehistologic examination showed necroticmyocytes without nuclei. The perimysiumcontained red cells. There was no evi-dence of vasculitis or microvessel angi-opathy, and bacteriologic samples werenegative.

Skeletal muscle infarction, firstlydescribed in diabetes in 19651 is a rarecomplication of diabetes and may bemisdiagnosed as a neoplasm, an abscessor myositis, as recently reported.2 It occursin patients with poorly controlled diabetesand with diabetic complications, as inour patient. The infarction has beensuggested to result from ischaemia causedby arteriosclerosis and microangiopathy,although we did not find histologicalevidence of either in the present case.Creatine kinase levels may be normal orslightly elevated.2 Our case illustrates thatthis difficult diagnosis must be consideredin the face of a painful and tender swellingof a leg occurring abruptly in a diabeticpatient. We also suggest that post-contrastT1-weighted MRI revealing the presenceof enhanced linear signal infiltratesthrough an abscess-like lesion preservingthe tissue planes may suggest diabeticinfarction.

F. Heureux, J.-F. Nisolle, E. Delgrange,J. DonckierDepartment of Internal Medicine andEndocrinology, University Hospital UCLof Mont-Godinne, 5530 Yvoir, Belgium