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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 3 | Page : 564-566
Chronic Sheehan syndrome presenting as pseudo-hypertrophic myopathy (Hoffman syndrome): A very rare combination
Harish Kumar, Veer Bahadur Singh, Jatin Agrawal, Babu Lal Meena
Department of Medicine, S P Medical College and Associated Group of PBM Hospital, Bikaner, Rajasthan, India
|Date of Web Publication||3-May-2016|
Department of Medicine, S P Medical College and Associated Group of PBM Hospital, Bikaner, Rajasthan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kumar H, Singh VB, Agrawal J, Meena BL. Chronic Sheehan syndrome presenting as pseudo-hypertrophic myopathy (Hoffman syndrome): A very rare combination. Neurol India 2016;64:564-6
|How to cite this URL:|
Kumar H, Singh VB, Agrawal J, Meena BL. Chronic Sheehan syndrome presenting as pseudo-hypertrophic myopathy (Hoffman syndrome): A very rare combination. Neurol India [serial online] 2016 [cited 2019 Dec 15];64:564-6. Available from: http://www.neurologyindia.com/text.asp?2016/64/3/564/181568
Sheehan syndrome is a rare, delayed diagnosis of pituitary necrosis after postpartum hemorrhage and shock. It is still a common cause of hypopituitarism in India. It usually presents with anterior pituitary hormonal deficiency. In this study, we present a very rare case of Sheehan's syndrome, who manifested with pseudohypertrophic-myopathy (Hoffman syndrome). Hoffman syndrome itself is very rare as only a few cases (<10%) have been reported that are found to be having the primary aetiology. In this case, the cause of Hoffman syndrome was another syndrome, making it an even rarer entity in our case.
Sheehan' s syndrome presents with anterior pituitary hormone deficiency due to ischemic necrosis as a result of postpartum hemorrhage and shock. The syndrome manifests as lactation failure, amenorrhea, involution of the breasts, loss of axiliary and pubic hair, and features typical of other anterior pituitary hormonal deficiencies.
In this syndrome, the blood profiles of hormones secreted by the anterior pituitary hormones are low as a result of panhypopituitarism. The thyroid hormone level is low in Sheehan' s syndrome as a result of thyroid-stimulating hormone (TSH) deficiency. The thyroid hormone is very important for growth and mental development. The classical clinical features of hypothyroidism are weight gain, lethargy, cold intolerance, depression, drying and coarsening of skin and hair, and delayed tendon reflexes. Full blown myopathies are a rare manifestation of the deficiency. Various types of myopathies are associated with hypothyroidism; pseudohypertrophic myopathy (Hoffman syndrome) is one of them. Hoffman syndrome was described by Hoffman in 1987. Hoffman syndrome presents with muscle cramps with weakness, pain, stiffness and pseudohypertrophy of muscle mass. Very few cases of Hoffman syndrome have been reported.,,,, In previously reported cases of Hoffman syndrome, the cause of hypothyroidism was primarily related to thyroid gland function such as autoimmune hypothyroiditis. This is the first case in which the cause was secondary and was a delayed manifestation of hypothyroidism secondary to Sheehan's syndrome. Myopathy in hypothyroidism depends on the duration and severity of the thyroid deficiency. Our patient was a known case of Sheehan's syndrome for 5 years with poor compliance to treatment.
A 27-year-old female patient, a known case of Sheehan's syndrome, was admitted with a 6-month history of gradually increasing swelling below the knee in both legs [Figure 1]. The patient had also been complaining of pain and stiffness for the past 2 months, which was associated with proximal weakness in both legs. The patient had difficulty in conducting her daily routine activities in the form of climbing stairs and standing from a squatting position. Weakness involved the proximal muscles more than the distal muscles. Her sensations were intact. No bladder and bowel involvement was reported. The patient had a history of tiredness, slowness, hair fall, menstruation irregularities and drying and coarsening skin and hair, with hoarseness of voice. Clinical examination revealed hypertrophy of calf muscles in both legs with stiffness and tenderness [Figure 1]. Power was 3/5 at hip and 4/5 at knee and ankle joint. Her reflexes were normal with delayed relaxation present in both upper and lower limbs at all joints, particularly at the ankles and the biceps. The overlying skin of the calf muscles was smooth and thick and no inflammatory signs were present. Her hair were dry and thin and she had asymmetrical and coarse facial features. There was a loss of lateral half of the eyebrows and the hair growth on rest of the body was also reduced (including the pubic and axiliary regions). The thyroid gland was normal in appearance. No lymphadenopathy or tremors were noted.
The laboratory tests showed a hemoglobin level of 11.5 gram% with a normal peripheral blood film. Other cell lines were within normal limits. Liver function and renal function tests were also normal. Serum creatinine phosphokinase was extremely high at 2301 U/L (normal <170 U/L). Serum cholesterol was 230 mg/dl. Lactate dehydrogenase was 2160.40U/L (normal 240-480 U/L). TSH was 0.34 mIU/ml (normal: 0.25-5.0 mIU/ml). The free T3 and T4 values were <0.70pmol/L and <1.0pmol/L, respectively (normal free T3 and T4 values: 4-8.3 and 9-20 pmol/L, respectively) by fully automated enzyme-linked fluorescent essay. Serum anti-thyroid peroxidase (TPO) antibody levels were normal [ level was <0.8IU/ML (normal: <8 IU/ML)]. Serum electrolytes, bleeding time, clotting time and prothrombin time/international normalized ratio were within normal limits. The hormones secreted by anterior pituitary gland were low such as the follicle stimulating hormone (0.01mIU/ml) and luteinizing hormone (0.16 mIU/ml). Her chest roengenography was normal. She had a low-voltage sinus bradycardia on electrocardiogram. The color Doppler study of the venous system in both legs was normal. Two-dimensional echocardiogram did not reveal any abnormality; the ejection fraction was 60%. Histopathological features of calf muscle included myopathy associated with the endocrine disorder with nonspecific changes. Nerve conduction velocity (NCV) and electromyography (EMG) revealed no specific abnormality. Ultrasonography and magnetic resonance imaging (MRI) of the calf muscles showed edematous changes in bilateral leg (more in bilateral tibialis anterior muscles) with subcutaneous edema [Figure 2]. The anterior lobe of pituitary was not visualized on MRI with the sella being filled with cerebrospinal fluid [Figure 3]. The bright spot of the posterior pituitary gland was normal in position. The patient was kept on thyroid hormone therapy (thyroxine 100µg OD), and after 10 days, experienced improvement in pain and stiffness.
|Figure 3: Magnetic resonance imaging of pituitary fossa showing empty sella filled by cerebrospinal fluid|
Click here to view
Hoffman syndrome is a very uncommon form of hypothyroid myopathy occurring due to long- standing hypothyroidism., Hence, pseudohypertrophic myopathy may be thought of as a differential diagnosis in a patient with long-standing untreated hypothyroidism and having muscle hypertrophy and weakness. The cause in 95% of the cases of thyroid insufficiency is primary, commonly related to an autoimmune etiology. Hashimoto thyroiditis is the most common cause of primary hypothyroidism. It usually presents with high levels of serum anti -TPO and anti thyroglobulin antibodies but the same was not documented in our case. Anti-thyroglobulins are present in 80-90% of the cases and anti-TPO antibodies may be present in 90 -100% of the cases  The case at hand is the first case in which the cause of hypothyroidism was secondary, leading to a pseudohypertrophic myopathy. In the cases which have been reported earlier, the cause of Hoffman syndrome was primary.,
Hoffman syndrome is a pseudohypertrophic myopathy described by Hoffman in 1897 in adult patients. Our case fits in this syndrome and presented with a hypertrophic muscle mass, stiffness, pain and proximal muscle weakness. It is a non-inflammatory myopathy with infiltration of the muscle by myxedema associated with an autoimmune reaction and membrane dysfunction of muscles. Variations in the size of the muscle fibres with focal necrosis, type 1 and type 2 fibre atrophy and hypertrophy, and granulomatous accumulation without inflammatory infiltrate, differentiates it from other inflammatory myopathies such as polymyositis., Creatine phosphokinase enzyme levels are elevated in 60% of the cases and this increase does not correlate with the severity of myopathy. The low cost of estimating creatine phosphokinase, its sensitivity and the ease in estimating its levels, make it the best biochemical marker for assessment of myopathies. The mechanism of pseudohypertrophic myopathy in Hoffman syndrome is not fully understood and is a matter of discussion. It may be due to an increase in the size and number of muscle fibres with accumulation of connective tissue. Ono et al., demonstrated that all these findings may be absent in Hoffman syndrome. There are many causes of calf muscle hypertrophy such as neurogenic-myositis and genetic dystrophy. The clinical profile; the low TSH, free T3 and T4; and the NCV, EMG and biopsy findings, differentiate the case at hand from the aforementioned causes. Muscle enzymes are highly elevated in Hoffman myopathy as compared to their normal levels in other causes of hypothyroid myopathy.
Hoffman syndrome has a good prognosis if diagnosed earlier. Our patient had relief with thyroid hormone replacement. Hoffman syndrome is a very rare entity with a good prognosis. The etiology in our case was not autoimmune, as has been seen in previously reported cases.
We would like to thank Rajesh Kumar for his valuable contribution in the writing of this manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]