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|Year : 2020 | Volume
| Issue : 1 | Page : 179-181
Spontaneous Conception, Pericardial Effusion, and Pseudohypertrophic Myopathy Mimicking Muscular Dystrophy in Delayed Presentation of Sheehan Syndrome
Hiya Boro, Alpesh Goyal, Nikhil Tandon
Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||28-Feb-2020|
Dr. Alpesh Goyal
Room No. 309, Third Floor Biotechnology Block, Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Sheehan syndrome, characterized by postpartum pituitary necrosis, is an important cause of hypopituitarism in developing countries. We report the case of a 50-year-old female with Sheehan syndrome, who had two spontaneous conceptions following severe postpartum hemorrhage in her first delivery and presented 27 years later with moderate pericardial effusion and features of pseudohypertrophic myopathy mimicking muscular dystrophy.
Keywords: Hoffman syndrome, hypopituitarism, myopathy, Sheehan syndrome, spontaneous conception
Key Messages: Spontaneous pregnancy following postpartum hemorrhage does not exclude the possibility of Sheehan syndrome. Metabolic causes like hypothyroidism, which are often reversible and treatable, should always be excluded in patients with myopathy. Longstanding severe secondary hypothyroidism may behave like primary hypothyroidism in terms of clinical manifestations and the classical distinction described between the two conditions may not hold true.
|How to cite this article:|
Boro H, Goyal A, Tandon N. Spontaneous Conception, Pericardial Effusion, and Pseudohypertrophic Myopathy Mimicking Muscular Dystrophy in Delayed Presentation of Sheehan Syndrome. Neurol India 2020;68:179-81
|How to cite this URL:|
Boro H, Goyal A, Tandon N. Spontaneous Conception, Pericardial Effusion, and Pseudohypertrophic Myopathy Mimicking Muscular Dystrophy in Delayed Presentation of Sheehan Syndrome. Neurol India [serial online] 2020 [cited 2020 Mar 29];68:179-81. Available from: http://www.neurologyindia.com/text.asp?2020/68/1/179/279696
Sheehan syndrome occurs due to ischemic necrosis of the pituitary gland, usually following severe postpartum hemorrhage. It is characterized by varying degrees of hypopituitarism. Despite the improvement in obstetric care worldwide, it continues to remain a significant cause of morbidity and mortality in developing countries., Most patients present with agalactia and failure to resume menses after delivery; spontaneous conception has however been reported very rarely. Pericardial effusion, once a commonly reported finding in patients with primary hypothyroidism is less common these days owing to early detection of thyroid dysfunction and is hardly ever reported in secondary hypothyroidism. Hoffman syndrome, characterized by pseudohypertrophy and proximal muscle weakness is a delayed neurological manifestation of primary hypothyroidism in adults. It has rarely been described with secondary hypothyroidism resulting from Sheehan syndrome. Our case report highlights a rare case of Sheehan syndrome with multiple atypical manifestations.
| » Case Report|| |
A 50-year-old female presented with complaints of progressive proximal muscle weakness involving both lower limbs and upper limbs for the past 3 years. This was associated with self-noticed undue prominence of bilateral calves for the same duration. She also complained of generalized body swelling, predominantly involving bilateral lower limbs along with facial puffiness for the past 3 years. At the same time, she also noticed dryness of her skin with decreased sweating, hoarseness of voice, and loss of hair from scalp, eyebrows, axilla, and pubes. She was easily fatigable, had a poor appetite, and complained of long-standing constipation requiring laxatives regularly.
Her first pregnancy, a spontaneous conception which occurred 27 years ago, had resulted successfully in a vaginal delivery followed by severe postpartum hemorrhage. Subsequently, she developed hypotension, shock and remained unconscious for 24 hours. She received resuscitation in the form of three units of blood products and multiple pints of intravenous fluids. However, she could not lactate her baby from that, and all subsequent pregnancies. She resumed her menses postpartum, however, with an increased intermenstrual interval of 1.5–2 months. The patient conceived spontaneously again, four and seven years after her first pregnancy, resulting in normal vaginal deliveries of live babies without any complication. However, she continued to have oligomenorrheic menses with increasing intermenstrual duration and decreasing flow following these conceptions and eventually, 3 years after the last delivery, at the age of 32 years, she became amenorrhoeic.
On examination, the patient had a pulse of 60 beats/min, regular in rhythm and blood pressure of 86/60 mmHg without a significant postural drop. She had characteristic coarsened myxedematous facies with fine wrinkles around the corners of the eyes and mouth and hoarse voice [Figure 1]. There was sparse, dry and brittle scalp hair; axillary, pubic hair and hair over her eyebrows were also scanty. She had cold extremities with dry skin, mild pallor, and non-pitting pedal edema bilaterally.
|Figure 1:Clinical photograph showing coarsened facial features with periorbital edema, fine wrinkles around corners of eyes and mouth, and scanty scalp hair|
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Neurological examination revealed proximal weakness with 4/5 power in proximal muscles of her bilateral upper and lower limbs. She also had pseudohypertrophy of bilateral calves [Figure 2] and delayed tendon jerks involving bilateral knee, supinator, and biceps. There was no percussion myoedema nor was there any clinical evidence of carpal tunnel syndrome (CTS).
|Figure 2: Clinical photograph showing pseudohypertrophy of bilateral calves|
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Blood investigations revealed normocytic normochromic anemia, hyponatremia, and dyslipidemia [Table 1]. Basal hormonal investigations were suggestive of panhypopituitarism [Table 2]. Electrocardiography (ECG) showed normal sinus rhythm with sinus bradycardia and low voltage complexes. The chest radiograph was normal. 2D echocardiography revealed moderate circumferential pericardial effusion. Electrophysiologic study (EPS) revealed small, short polyphasic motor unit action potentials (MUAP) with early and full recruitment (suggestive of myopathy) along with evidence of right grade V and left grade III CTS. Magnetic resonance imaging (MRI) of the sellar region showed the presence of very thin pituitary parenchymal rim with cerebrospinal fluid (CSF) filling the sellar cavity, suggestive of partial empty sella [Figure 3].
|Figure 3: Magnetic resonance imaging (MRI) of the sellar region showing the thin rim of pituitary parenchyma with cerebrospinal fluid (CSF) filling the sellar cavity, suggestive of partial empty sella|
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She was started on prednisolone 5 mg daily and 5 days after initiating the steroid therapy, levothyroxine replacement was introduced, and she was closely watched for any symptomatic deterioration.
| » Discussion|| |
Our patient had a delayed presentation, reporting with atypical manifestations approximately 27 years after the postpartum hemorrhage. She went on to have two spontaneous conceptions after the index delivery. However, she had lactational failure after the first delivery, which persisted with the two subsequent pregnancies. This would suggest that the onset of hypopituitarism occurred after the first delivery itself. Moreover, the patient resumed menstruation after the first delivery, though she had oligomenorrhea, which gradually progressed, with increasing intermenstrual interval, to amenorrhea. This would indicate preservation of gonadotrophe reserve following the initial event, but with subsequent depletion. Pregnancy in patients with Sheehan syndrome has been reported commonly following attempts at ovulation induction, however, spontaneous conception is very rare.,,
Increased capillary permeability and slow lymphatic drainage contribute to the pericardial effusion in hypothyroidism. Classically, patients with secondary hypothyroidism have a small-sized cardiac silhouette and pericardial effusion is rare. This is because these patients have relatively mild hypothyroidism and most patients seek attention early in the disease course due to other hormone deficiencies. However, in developing countries, where the diagnosis is delayed, even patients with secondary hypothyroidism may develop gross features of myxedema and present with pericardial effusion, as illustrated in our case. Neurological manifestations of hypothyroidism occur late in the disease course and it is very rare for these to be the initial presentation of the disease. Hoffman syndrome has mainly been described in patients with primary hypothyroidism., As per a detailed literature review, there has been only one other case describing this entity in secondary hypothyroidism due to Sheehan syndrome.
Our case had three atypical manifestations of Sheehan syndrome: first, the patient had spontaneous conception following the onset of Sheehan syndrome, second, she had pericardial effusion associated with secondary hypothyroidism, and third, she had a presentation with proximal myopathy mimicking muscular dystrophy. To conclude, in developing countries like India, where Sheehan syndrome is still a common cause of hypopituitarism, and delayed presentation is not uncommon, the clinician should be wary of atypical presentations, where severe secondary hypothyroidism can mimic features of primary hypothyroidism.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]