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Case 14
Published in Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta, Clinical Cases, 2021
Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta
InsulinomaFasting hypoglycaemiaSymptoms and signs of hypoglycaemia such as dizziness, headache, sweating, visual disturbance, confusion, amnesia, tachycardia, palpitations, anxiety, weakness, seizureWhipple's triad = symptomatic hypoglycaemia + plasma glucose ≤2.2 mmol/L + resolution of symptoms with glucose
Psychiatric Diagnosis is Not a Diagnosis of Exclusion
Published in Paul Ian Steinberg, Psychoanalysis in Medicine, 2020
Insulin-secreting tumors may, with their hypoglycemic effect, produce symptoms of central nervous system dysfunction, including dizziness, headache, blunted mental acuity, confusion, abnormal behavior, convulsions, and loss of consciousness, as well as clouding of vision (Clarke et al., 1972; Comi and Gorden, 1987; Foster et al., 1987; Scholz et al., 1960; Schein, 1973). The symptoms of insulinoma often lead to misdiagnosis of a neurologic or psychiatric disorder. Twelve of 60 patients were initially diagnosed to have a neurologic or psychiatric disorder (Service et al., 1976). Temporal lobe seizures were most commonly diagnosed. One patient was admitted to a psychiatric unit for “hysterical” behavior. Other misdiagnoses included cerebrovascular insufficiency, multiple sclerosis, narcolepsy, and hysteria. Service et al. (1976) note that hypoglycemic episodes can also mimic psychosis and brain tumor. A patient experiencing a number of psychiatric symptoms was referred to psychiatry by a neurologist for treatment of his “psychiatric” condition.
HPB Surgery
Published in Tjun Tang, Elizabeth O'Riordan, Stewart Walsh, Cracking the Intercollegiate General Surgery FRCS Viva, 2020
London Lucien Ooi Peng Jin, Teo Jin Yao
What is the treatment of a localised insulinoma?Ideal tumour to consider enucleation IF: clearly localised on preoperative imaging, in a favourable location (exophytic/superficial) and relation to the pancreatic duct is clearly delineated.Formal resection (i.e. pancreaticoduodenectomy or distal pancreatectomy depending on location) otherwise.If frankly malignant or suspicious i.e large (>4 cm), lymph node metastases, vascular or other local invasion, then a formal resection as well.
Preoperative treatment of benign insulinoma: diazoxide or somatostatin analogues?
Published in Acta Chirurgica Belgica, 2022
Quentin Gilliaux, Claude Bertrand, François Hanon, Julian E. Donckier
Insulinoma is a rare neuroendocrine tumour of the pancreas with an incidence of four cases per one million persons per year [1,2]. Its resection is the only curative treatment. However, medical therapy may be needed to prevent severe preoperative hypoglycaemia, when surgery is contraindicated, delayed or refused and in case of unresectable metastatic disease. Diazoxide, a non-diuretic benzothiadiazine derivative diminishing insulin secretion from beta-cells by opening ATP-dependent potassium channels has been used for controlling hypoglycaemia [3,4]. Somatostatin analogues (octreotide or lanreotide) can also suppress insulin secretion by activating somatostatin receptors (SSTR), in particular SSTR2 [4,5]. They have been regarded as second line in benign insulinoma or as first line in malignant insulinoma because of their antiproliferative effects and a higher expression of SSTR2 in malignant tumours [4,5]. To our knowledge, the effects of these two medications have never been compared in a same patient with insulinoma.
Current challenges in the pathophysiology, diagnosis, and treatment of paroxysmal movement disorders
Published in Expert Review of Neurotherapeutics, 2021
Cécile Delorme, Camille Giron, David Bendetowicz, Aurélie Méneret, Louise-Laure Mariani, Emmanuel Roze
Diagnosis of PMD secondary to an insulinoma is often challenging with a median delay reported to be as long as 2 to 3 years [130,131]. The wide phenotypic expression of neuroglycopenia and its potential variability in a single subject (as also observed in GLUT1-deficiency syndrome) may largely account for this delay. Characteristics of the paroxysmal episodes may provide good clues to the diagnosis i) episodes sometimes occur during the second part of the night or early morning or are triggered by prolonged exercise ii) paroxysmal symptoms tend to increase and decrease within an episode in a gradual manner iii) mixed hyperkinetic movements could vary from one day to another, lasting minutes to hours iv) motor manifestations are combined with behavioral disturbances, such as aggressiveness/disinhibition or psychomotor slowing v) drowsiness/dreaminess are frequently observed vi) neurovegetative features can also be present, such as sweating. One diagnosis pitfall is that hyperkinetic abnormal movements may sometimes combine genuine movement disorders and awkward pseudo-voluntary movements accompanying the behavioral problems [132–134]. The key diagnostic investigation is the measurement of ictal glycemia, which is very low (0.2 to 0.4 g/L in reported cases) [132–134]. Frequent misdiagnosis occurs for psychiatric conditions, epilepsy, or functional movement disorder [132,133]. Ictal glycemia should thus be largely assessed in patients with PMD without a definite diagnosis. Treatment mostly consists in the resection of the insulinoma.
An unusual presentation of insulinoma and the serious consequences of delayed diagnosis
Published in Journal of Endocrinology, Metabolism and Diabetes of South Africa, 2020
Insulinoma is one of the functional pancreatic tumours derived from the pancreatic ductal/acinar system that is rare but often missed.1 Patients typically present with symptoms of unprovoked hypoglycaemia but as these symptoms are non-specific diagnosis is often delayed due to late presentation of patients or failure of healthcare workers to recognise the disease early. The main clinical features include adrenergic symptoms such as sweating and palpitations and neuroglycopenic symptoms including confusion, anxiety, stupor, convulsions and coma. The neuroglycopenic symptoms are often misdiagnosed as primary neurological disorders, which often causes a delay in diagnosis and treatment that can result in permanent neurological damage. Insulinomas are usually small in size and benign in nature. The tumours are malignant in less than 10% of cases.2 Insulinomas are commonly sporadic in nature with only a few cases described as being associated with genetic mutations.2 Localisation of these tumours presents a challenge to most clinicians as the standard techniques often used for localisation have poor sensitivity and specificity. Here we present a case of a patient who presented with recurrent hypoglycaemia complicated by neurological sequelae.