Monday, December 13, 2021

Is there a Link between Depression and Morgagni-Stewart-Morel Syndrome? About a Clinical Case- Juniper Publishers

Juniper Publishers-Open Access Journal of Case Studies


Is there a Link between Depression and Morgagni-Stewart-Morel Syndrome? About a Clinical Case

Authored by Smaoui Najeh

Abstract

Morgagni Stewart Morel (MSM) syndrome is an association of clinical features with radiological findings. It is defined as the presence of hyperostosis frontalis interna, variably associated with metabolic, endocrine, and neuropsychiatric disorders. In the literature, the majority of reported cases presented with depression, without any studies focusing on researching the nature of the link between the two types of disorders. In fact, depression raises the question of whether it is related to the Disease or to the elderly since the prevalence of the MSM syndrome increases with age. There is considerable debate in the scientific community as whether to the depression is a comorbidity or a clinical manifestation of this syndrome. In this work, we report a clinical case to discuss the relationship between MSM syndrome and depression in a 67-year-old woman who presented with depression and Imaging consistent with MSM syndrome.

Keywords: Morgagni-stewart-morel syndrome; Depression; Comorbidity; Co-occurrence; Relationship

Introduction

Morgagni Stewart Morel (MSM) syndrome was first described by Morgagni, during autopsy, in an obese female patient who had hirsutism and thickening of inner table of her skull. Stewart, in 1928, and Morel, in 1930, added neuropsychiatric symptoms [1]. Due to the frequency of endocrinal dysfunctions, it was also called metabolic craniopathy. The symptoms are generally non specific and benign, but they may cluster together in some cases, giving rise to various syndromes. This disease is usually misdiagnosed.

In the literature [2], the majority of reported cases presented with psychiatric disorders, in particular depression, without any studies focusing on researching the nature of the link between the two types of disorders. In fact, depression raises the question of whether it is related to the disease or to the elderly since the prevalence of the MSM syndrome increases with age. We report a clinical case to discuss the relationship between MSM syndrome and depression.

Case Report

A 67-year-old woman was referred to our outpatient psychiatry department for persistent affective symptoms during three years, which had gradually worsened in the latest month. She also was suffering from diabetes and high blood pressure, well-balanced under treatment. There was no family history of psychiatric or neurological disorders.

In fact, she suffered from depressed mood, loss of initiative, decreased appetite, anxiety, difficulty staying asleep, and lack of energy and motivation. Over the years, she had gradually withdrawn from social activities.

She was overweighted (body mass index 31kg/m2) with blood pressure: 140/80mm Hg. She looked tired with under-eye dark circles and reduced facial expression. She reported being sad, with anhedonia and panic attacks. There was neither suicidal ideations nor psychotic symptoms. She was vigilant and cooperative. There were no focal neurological symptoms. Her Mini Mental State Exam (MMSE) and Geriatric Depression Scale (GDS) scores were 26/30 and 13/15 respectively.

Routine blood chemistry showed hyperglycemia at 1.46g/l. A brain computed tomography showed bilateral and symmetrical frontal bone hyperostosis centered on the inner table (Figure 1). No parenchymatous or vascular lesions were objectivized. Possible secondary causes were excluded by investigating acromegaly, malignity, Paget’s disease, neuroendocrine tumours, and hyperparathyroidism. The diagnosis of MSM syndrome was retained.

We prescribed selective serotonin reuptake inhibitor (SSRI) antidepressant (Sertraline 50mg/day) for depressive symptoms. A therapeutic adjustment with hygiene and dietetic rules was made. A neurosurgical opinion was sought and concluded that no immediate surgical intervention was indicated.

After three months, there was a noticeable improvement in mood and sleep disorders with resumption of certain activities. Her GDS score became 8/15.

Discussion

MSM syndrome is an association of clinical features with radiological findings. It is defined as the presence of hyperostosis frontalis interna (HFI), variably associated with metabolic, endocrine, neurological and psychiatric disorders [3,4]. The common clinical features are virilism, diabetes, abdominal adiposity, high blood pressure, pituitary disorders (polyphagia, polydypsia, visual disturbances, asthenia) and neurological disorders (headache, migraine, comitial seizures, cognitive impairments, parkinsonism) [5,6]. In the literature [2], psychiatric symptoms have had various manifestations; behavioral disturbances, psychotic symptoms, character change, aggression, suicide attempts, and mainly depression. Our patient presented with a partial expression of MSM symptoms, with depressive disorder in the foreground, metabolic and endocrine disorders (high blood pressure, obesity, diabetes) and HFI on imaging.

Its prevalence in autopsy series is 12%, affecting mainly women, and increasing with age [4], which is the case of our patient. Also like in our clinical case, its discovery is often incidental, either in isolation or associated with disease states. The diagnosis relying on imagery. The characteristic X-ray finding is thickening of inner table of skull [1]. The exact etiology of HFI and MSM syndrome remains unclear. The most interesting theories relate to sex hormones dysregulation, obesity and leptin dysfunction that promoted osteoformation [7,8]. Genetic basis was hypothesized in a case report of monozygotic twins both suffering from MSM syndrome [8]. However, the symptoms were non uniform between them, suggesting phenotypic variability probably due to environmental factors. In practice, doctors are confronted primarily with the “fragments” of the disease, because this syndrome develops its symptoms for many years and at different speeds [9]. But in some cases, they may cluster together, giving rise to delay in diagnosis and treatment.

There is considerable debate in the scientific community as whether to the psychiatric syndrome is a comorbidity or a clinical manifestation of this syndrome. Some authors classify the psychiatric symptoms as ancillary signs because they are heterogeneous, inconstant and almost always present in elderly due to senility [4]. Other authors [9] think there is a clear association between HFI and psychiatric disorders. Future studies aiming to clarify their relationship are necessary to determine if the psychiatric symptom is a primary or secondary manifestation of MSM syndrome. Mental disorders, especially depression may be secondary to the endostosis and its mechanical effects on the one hand. On the other hand, the prevalence of depression in the general population is high, and HFI is quite a common finding nowadays [10]. Therefore, the combination of these two conditions is not unusual. Nevertheless, studies revealed an increased incidence of HFI in mental hospital patients regardless of age [11]. Indeed, the infundibulo-pituitary region is the center of endocrine regulation. It is also involved in psychiatric disorders. Moreover, among the etiopathogenic hypotheses of depression, the endocrine disturbance play a key role, notably sex hormone and leptin dysfunctions which involved in MSM syndrome [12]. All of this could justify depression as a primary manifestation in MSM syndrome.

So, we presume that there was a relationship between our patient’s depressive disorder and her HFI. However, the cause-effect relationship of these association remains unclear. Pathological changes in the brain and cranial box, as well as endocrine imbalance, can contribute to neuropsychiatric disorders, especially depression [2,9]. Anyway, as well as in our case-report, the psychiatric disorder may be improved by specific treatment.

Conclusion

MSM syndrome is one of the less understood syndromes. The patient may present with varied symptoms and thus lead to difficulty in diagnosis. Whatever, depression should be interpreted not only as component of MSM syndrome, but also as a comorbidity of this syndrome. The psychiatric comorbidities, including depression, whether or not related to the disease, are likely to be improved by appropriate treatment.

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Wednesday, December 8, 2021

An Uncommon Localization of a Hydatid Cyst Presenting with Pulmonary Embolism - Juniper Publishers

 Juniper Publishers-Open Access Journal of Case Studies


An Uncommon Localization of a Hydatid Cyst Presenting with Pulmonary Embolism

Authored by Francesco Finizola

Abstract

Human echinococcosis is an important infection in undeveloped and developing countries, caused by larval forms of the genus Echinococcus. In western countries this disease is sporadic. The organs mostly involved by the cysts are the liver (70%) and the lungs (20%) [1]. Cardiac hydatid cyst is a rare condition, and the location of a hydatid cyst in the interventricular septum is exceptional. Cardiac echinococcosis is not frequent, only 0.01%-2% of all hydatid infestations [2]. The left ventricle is the heart chamber most frequently involved (55-60%) and the involvement of the interventricular septum is reported in 4% of cardiac cases. Chest pain, palpitations, and dyspnea are the most frequent symptoms associated with cardiac echinococcosis. We describe a case of an unusual presentation of this disease.

Keywords: Echocardiography; Interventricular septum; Hydatid cyst; Echinococcosis

Case Report

A 39-year-old Caucasian female was admitted to our department for the evaluation of syncope and dyspnea. Two years before, the patient presented with an abdominal pain. An abdominal echography revealed the presence of two liver hydatic cyst. Treatment with Albendazole was undertaken for one year with improvement of symptoms. Physical and neurological examination revealed nothing unusual and routine laboratory tests were normal. Chest CT scan showed pulmonary embolism involving principal arteries and a mass in the right ventricle, initially interpreted as a tumor or blood clot. A transthoracic echocardiography was done and revealed the presence of a 40x25mm cystic mass in the apical part of interventricular septum with protrusion to the right ventricle cavity. ELISA test was positive for echinococcosis antibodies. Albendazole was started for 6 months with an improvement of pulmonary symptoms. Then,cystectomy was performed and the remaining cyst contents and germinative membrane were removed. Histopathological exam of cystic material confirmed the diagnosis of hydatid cyst. The postoperative period was uneventful, and the patient was discharged from the hospital.

Discussion

Echinococcosis or hydatid disease is caused by larval stage of Echinococcus granulosus. Humans are only intermediate and accidental hosts. ELISA is the most specific serologic tests that can be used and a positive result for echinococcus antibodies confirms the diagnosis. Cardiac hydatidosis is a rare condition potentially fatal or leading to severe complications without treatment being undertaken. The most common cardiac locations are left ventricular wall (60%), right ventricle (10%), pericardium (7%), left atrium (6-8%), right atrium (4%), and the interventricular septum (4%). Right ventricle is rarely involved and cysts are more prone to rupture, as compared to cysts on the left ventricle. This condition may lead to pulmonary embolization, a lethal complication. Such a condition should be taken into consideration as other complications (arrhythmia, angina, dysfunction of valvular and ventricular functions) may initially give the same symptoms. Echocardiography is the most efficient method in the diagnosis of cardiac hydatid cyst [3]. CT scan and MRI can be used to detect the involvement of other organs, but they have a low sensitivity and a low sensitivity in the diagnosis of intra-ventricular hydatidosis. Transesophageal ecography can provide a more accurate definition of the mass. Surgical excision with removing germinal layer and albendazole therapy showed successful results in treating cardiac hydatid cysts [4] (Figure A-C).

 

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For more articles in  Open Access Journal of Case Studies please click on: https://juniperpublishers.com/jojcs/index.php

 

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