|Year : 2015 | Volume
| Issue : 1 | Page : 32-34
Recurrent hypoglycemia in a patient of neurofibromatosis type 1 and type 1 diabetes mellitus: Munchausen's syndrome mimicking Insulinoma
Mubaraq Naqash1, Muzafar Naik1, Tariq Bhat1, Irfan Yusuf1, Abdul Wahid Khan2, Ajaz Suhaff2
1 Department of General Medicine, Sher-I-Kashmir Institute of Medical Sciences, Medical College and Hospital, Srinagar, Jammu and Kashmir, India
2 Department of Psychiatry, Sher-I-Kashmir Institute of Medical Sciences, Medical College and Hospital, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||8-Sep-2015|
Department of General Medicine, Sher-I-Kashmir Institute of Medical Sciences, Medical College and Hospital, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
A 35-year-old widow having neurofibromatosis type 1 (NF 1) and type 1 diabetes mellitus (DM) was admitted for abdominal pain, vomiting and recent onset low blood sugars. During hospitalization, the patient developed recurrent episodes of hypoglycemia despite stopping insulin. A possibility of insulinoma was made due to the presence of abdominal pain, vomiting, hypoglycemia, and association of insulinoma with NF 1. However, surprisingly c-peptide was very low and plasma insulin levels were high. A detailed psychiatric evaluation of the patient revealed Munchausen's syndrome as a cause of recurrent hypoglycemia. Unrecognized psychiatric illnesses in type 1 DM may lead to overzealous investigation. In conclusion, the clinical presentations of persons with factitious disorder are varied and impact every area of medicine, factitious causes should always be considered in unclarified recurrent hypoglycemia, especially in cases presented with dramatic but inconsistent medical history, and an unusual personal behavior.
Keywords: C-peptide, hypoglycemia, insulin, neurofibromatosis type 1, type 1 diabetes mellitus
|How to cite this article:|
Naqash M, Naik M, Bhat T, Yusuf I, Khan AW, Suhaff A. Recurrent hypoglycemia in a patient of neurofibromatosis type 1 and type 1 diabetes mellitus: Munchausen's syndrome mimicking Insulinoma. J Mental Health Hum Behav 2015;20:32-4
|How to cite this URL:|
Naqash M, Naik M, Bhat T, Yusuf I, Khan AW, Suhaff A. Recurrent hypoglycemia in a patient of neurofibromatosis type 1 and type 1 diabetes mellitus: Munchausen's syndrome mimicking Insulinoma. J Mental Health Hum Behav [serial online] 2015 [cited 2020 Oct 26];20:32-4. Available from: https://www.jmhhb.org/text.asp?2015/20/1/32/164820
| Introduction|| |
Neurofibromatosis type 1 (NF 1) was first described by von Recklinghausen in 1882. It is an autosomal dominant disorder affecting 1 in 3500 people. Clinical diagnosis requires the presence of at least 2 of 7 criteria to confirm the presence of NF 1.  Unlike other endocrinal abnormalities, type 1 diabetes mellitus (DM) is also seen in association with NF I which may be attributed to occurrence of somatostatinomas in pancreas and duodenum.  Interestingly insulinoma is also seen in association with NF 1 and can present as recurrent hypoglycemia usually in the fasting state. , Here we report a case of recurrent hypoglycemia in a patient of NF 1 with type 1 DM due to Munchausen's syndrome mimicking insulinoma.
| Case Report|| |
A 35-year-old female having NF 1 and type 1 DM on insulin presented to our out-patient department with perspiration, palpitations, tremor and restlessness. The patient also complained of abdominal pain and vomiting for last 10 days. She had been using the recommended dose of insulin on which she used to have controlled sugars. On examination, the patient was conscious, oriented, sweating and had anxious look. The pulse 98/min, blood pressure of 140/80 mm Hg and respiratory rate 22 breaths/min. There were multiple neurofibromas all over the body including scalp and face and more than 10 café au lait macules over 15 mm in diameter. She also had freckling in the axillary region. Fundus examination revealed nonproliferative retinopathy. Systemic examination revealed distal sensorimotor neuropathy. Rest of the systemic examination was normal.
Investigations revealed mild anemia, normal kidney, liver function tests and amylase. Blood sugar [Table 1] the arterial blood gas was normal. The urinary ketones were negative. Electrocardiography and X-ray chest was normal, 24-h urinary protein was normal. Ultrasound abdomen was normal.
The patient was managed by stopping insulin and administering dextrose 25% followed by dextrose 10% at 100 ml/h. However, the random and postprandial blood sugar remained <160 mg/dl and patient continued to get hypoglycemias after stopping the dextrose 10% infusion. The patient also developed an episode of seizure and blood sugar random was 25 mg/dl. A possibility of insulinoma was made as insulinomas are associated with NF 1. The c-peptide was < 0.30 ng/mL (1.10-5.00) and concomitant insulin was 400.20 uIU/mL (2.00-25.00) indicating exogenous insulin administration. The hospital room, patients surrounding and patient belongings were examined for any hidden insulin vials. On insistence, the insulin vials and injections were found in her pillow. The patient was advised psychiatry consultation which she refused persistently. However, after discovering that she was to be discharged home that afternoon as her hypoglycemia had resolved and her condition started improving, patient changed her mind and consented for psychiatric consultation.
The patient was confronted with the possibility of insulin abuse, but she persistently denied it. The detailed psychiatric interview was held, the patient appeared anxious but disinterested in the consultation. Initially, the patient tried to conceal her mental symptoms, denied any problems and declared feeling well. Thereafter rapport was established, and then she shared her problems in a more detailed inquiry. After which she could easily recite her prescribed insulin regimen, and she readily admitted to having taking of her insulin in the washroom during the night between 2.00 AM to 3.00 AM. Yet, she was unable to provide any rationale for doing so and promptly changed the subject. From a psychological standpoint, the patient admitted she had depressed mood, and emotional incontinence, she firmly denied suicidal thoughts but agreed on more insistent questioning. She claimed to have no friends and that she avoided social contacts.
The evidence concerning sleep disorder was also positive-difficulty in falling asleep, a disorder of the continuity of sleep and early awakening. She had started these symptoms after the death of her father, and she was also distressed due to the disfigurement of her face due to NF. In terms of psychiatric anxiety, the patient reported experiencing feelings of anxiety, tension or irritability. No hypochondriac concerns were found. On questioning about the additional use of insulin, she took clearly a defensive position. There was an obvious discrepancy between the allegations that she is giving her best not to bother her relatives and their statements. Her mother was interviewed and according to her patient was second in the birth order among three children family. She also confessed that the patient has an overwhelming desire to be noticed and often behave dramatically or inappropriately to get attention. Furthermore, her mother confirmed that she had seen her symptomatic only after she was left alone or whenever she returned from the washroom.
The analysis of the observations on the patient and the conversation with her relatives lead to the conclusion that she attempted to focus attention on herself again rather than to consider a real suicidal attempt. She showed hysterical attitude, like personal characteristics of the dramatic cluster such as distortion of the facts, increased the desire to be the center of attention and manipulative behavior in the serve of her own needs.
| Discussion|| |
Hypoglycemia in patients of DM who are on insulin can be of diverse etiology which includes an overdose of insulin, missing a meal, liver, and renal failure. The hypoglycemia in such situation is usually transient and a good history and baseline investigations clearly points toward the etiology and such hypoglycemia invariably responds to the correction of underlying etiology. However, rarely the hypoglycemia may be recurrent and protracted, and a different etiology lies behind it.
One such situation is insulinoma, due to the excessive continuous production of insulin by the beta cells. The clinical suspicion of insulinoma's becomes stronger if the patient is having NF 1. The other condition in addition to NF 1 which is associated with insulinoma is multiple endocrine syndrome. 
Interestingly NF 1 has been associated with somatostatinoma's that secretes somatostatins which inhibits the release of insulin thereby giving rise to type 1 DM as is the case in our patient. The increased incidence of other endocrinoma's which occur with NF 1 beside insulinomas are pheochromocytoma, gastrointestinal neuroendocrine tumors, medullary thyroid cancer and hyperparathyroidism. ,
Insulinoma's are usually known to cause fasting hypoglycemia due to suppression of glucose production rather than due to the acceleration of glucose utilization, as is widely thought. On the contrary, our patient had a persistent hypoglycemic state during fasting as well as on regular diet and intravenous fluids. Also the patient was not interested in further investigations like computed tomography abdomen and relevant investigations that made the case more difficult. Her lack of interest in further investigations made us suspicious, and concealed investigations revealed the surreptitious use of insulin as the cause of recurrent hypoglycemia.
Patients with diabetes (type 1 and type 2 combined) have nearly twice the risk of depression as the general population.  Exogenous insulin administration as a mode of suicide is more common in medical and paramedical personnel.  The risk of depression is higher in type 1 DM in the presence of NF 1 due to pigmentation and neurofibromas on the face giving rise to cosmetic disfigurement. The exogenous insulin administration in such setting is usually a single attempt to commit suicide and diagnosis is not difficult. However, other psychiatric disorders can also present with symptoms and signs of intentional insulin overdosage of lesser severity, one of which is Munchausen's syndrome. 
Munchausen's syndrome is a factitious disorder wherein those affected feign illness, disease, aggravation of symptoms of some disease or psychological trauma to draw attention or sympathy to themselves.
The clinical spectra of hypoglycemia in our patient favored insulinoma due to abdominal symptoms and association of NF with insulinoma. However, persistent hypoglycemia in the nonfasting state opposed the diagnosis of insulinoma. The presence of raised insulin levels and low c-peptide levels in the presence of hypoglycemia favored exogenous insulin administration. Management of such a case is difficult as it requires a multidisciplinary approach which includes an active role of the psychiatrist also to the support of family and friends. The treating physician or endocrinologist should be well versed with the subtle manifestations of psychiatric illness in DM and should have a low threshold for asking patients to go for a psychiatric consultation.
| Conclusion|| |
Munchausen's syndrome should not be under looked in recurrent and persistent hypoglycemia, especially if the patient has risk factors for psychiatric illnesses and psychiatric evaluation in such patients is of paramount significance.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, et al.
The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA 1997;278:51-7.
Zaka-ur-Rab Z, Chopra K. Diabetes melliitus in neurofibromatosis I: An unusual presentation. Indian Pediatr 2005;42:185-6.
Perren A, Wiesli P, Schmid S, Montani M, Schmitt A, Schmid C, et al.
Pancreatic endocrine tumors are a rare manifestation of the neurofibromatosis type 1 phenotype: Molecular analysis of a malignant insulinoma in a NF-1 patient. Am J Surg Pathol 2006;30:1047-51.
Fung JW, Lam KS. Neurofibromatosis and insulinoma. Postgrad Med J 1995;71:485-6.
Boukhman MP, Karam JH, Shaver J, Siperstein AE, Duh QY, Clark OH. Insulinoma - Experience from 1950 to 1995. West J Med 1998;169:98-104.
Zografos GN, Vasiliadis GK, Zagouri F, Aggeli C, Korkolis D, Vogiaki S, et al.
Pheochromocytoma associated with neurofibromatosis type 1: Concepts and current trends. World J Surg Oncol 2010;8:14.
Takazawa Y, Sakurai S, Sakuma Y, Ikeda T, Yamaguchi J, Hashizume Y, et al.
Gastrointestinal stromal tumors of neurofibromatosis type I (von Recklinghausen's disease). Am J Surg Pathol 2005;29:755-63.
Rush WA, Whitebird RR, Rush MR, Solberg LI, O'Connor PJ. Depression in patients with diabetes: Does it impact clinical goals? J Am Board Fam Med 2008;21:392-7.
Hawton K, Clements A, Simkin S, Malmberg A. Doctors who kill themselves: A study of the methods used for suicide. QJM 2000;93:351-7.
Kamenov Z, Zaharieva E, Stoyanova V, Nikolova P, Bataklieva S, Sheinkova G. Clinical case of Munchausen syndrome in a patient with type 1 diabetes. J Diabetol 2011;2:5.