encephalitis Sir, Rabies is a common zoonotic illness seen across the world and has the highest case fatality rate known for any infectious disease. The disease leads to about 60,000 deaths annually and there are only twelve survivors reported till date.[1]
In July 2014, a 16‑year‑old boy was referred to us in a a b c
severely cachectic state. History revealed that the boy had Figure 1: MRI image (a) showing the hyperintense pituitary along with diffuse cerebral atrophy and body composition analysis before (b) and suffered an unprovoked bite from a street dog in March after (c) therapy 2014 and had presented with a febrile encephalopathic illness after three weeks. He was initially managed with complete absence of subcutaneous fat [Figure 1b]. He was broad spectrum antibiotics, ventilatory support, steroids diagnosed as a case of pituitary cachexia secondary to rabies and other supportive therapy. Cerebro‑spinal fluid (CSF) encephalitis and was treated with levothyroxine, growth showed neutrophilic pleocytosis, hypoglycorrhachia and hormone, testosterone and vitamin supplements. During elevated proteins. The paired samples of serum and CSF the last follow up, 3 months after the therapy, the body showed high levels (>1:1,64,000) of rabies virus neutralizing weight had improved by 4 kg and there is an appearance antibodies. The nuchal skin biopsy stained positive for of subcutaneous fat at a few places as shown in the repeat the presence of viral antigen confirming the diagnosis of BCA [Figure 1C]. rabies. MRI revealed lesions in bilateral thalami and in the cerebellum, sella and brainstem, including the upper Pituitary cachexia or Simmonds’ disease denotes the complete cervical cord [Figure 1a]. He was further managed with destruction of the anterior pituitary gland leading to a rabies immunoglobulin, physiotherapy, nasogastric feeds cachectic state. The disease is characterized by cutaneous and other supportive therapy. His neurological status, changes like loss of secondary sexual characteristics, especially his sensorium, improved gradually over the next hypotension, muscle weakness, and premature senility. Our six weeks. However, he developed significant cachexia patient had developed most of these features fairly rapidly in despite an intensive physiotherapy and a high caloric a few weeks after the onset of rabies meningoencephalitis. intake, prompting an endocrinological consultation. His Survival after rabies infection is reported in only a dozen anthropometric measures like body weight (27 kg) and patients till date and to the best of our knowledge, none body mass index (9.3 kg/m2) suggested severe cachexia. of them have reported the features of pituitary cachexia. His systemic examination revealed a dry skin, loss of Simmonds’ disease could be caused by infarction, bleeding, pubic hair with no evidence of dehydration. His hormonal tumor, inflammation and occasionally infection.[2] Previous profile showed free triiodothyronine‑ 1.1 pmol/L (normal reports suggest hypersexuality and behavioral abnormalities 3 – 6.2), free thyroxine ‑ 0.4 ng/dL (normal 0.8 – 1.2), during the acute phase of rabies infection. thyrotropin ‑ 2.2 mIU/L (normal 0.3 – 4.6), prolactin‑ 4 ng/ml (normal 0 – 15), leutinising hormone (LH) ‑ 0.6 IU/L (normal Cachexia is also associated with the presence of a number 0 – 8), follicle stimulating hormone (FSH) ‑ 0.5 IU/L (normal of disease states like malignancy, HIV infection, chronic 1 – 10), testosterone 126 ng/dL (normal 250 – 820) and IGF‑1 obstructive pulmonary disease and congestive cardiac of 86 IU/L (normal 145‑540). His peak cortisol and growth failure. The loss of muscle and fat tissue is due to an hormone (GH) after hypoglycemia were 22.4 mg/dL and altered endocrinal milieu coupled with increase in the 1.16 ng/mL, respectively. His body composition analysis (BCA) proinflammatory cytokines. [3] Testosterone and insulin by the dual energy X‑ray absorptiometry (DEXA) showed like growth factor‑1 (IGF‑1) play an important role in the
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Letters to Editor
maintenance of the muscle and fat tissue and our patient had with the inferior trunk of the brachial plexus, posterior to the deficiency of both these hormones leading to severe cachexia. pectoralis major muscle and medial to the lateral cord. The To conclude, we report the case of a young boy, who survived lesion showed a homogeneous enhancement [Figure 1]. A right the deadly rabies infection but had persistence of a cachectic axillary approach was adopted to excise the lesion. A 3 × 3 cm, state resulting from the loss of anterior pituitary hormones. firm, encapsulated, gray‑white mass arising from the medial cord was found [Figure 2]. The tumour was enucleated microsurgically avoiding any damage to the parent nerve. No K. V. S. Hari Kumar, F. M. H. Ahmad, Vijay Dutta nerve grafting was required. In the early post‑operative period, Departments of Endocrinology, Neurology and Respiratory the patient complained of an increased loss of sensations over Medicine, Command Hospital, Chandimandir, Haryana, India the ring and little finger. The pathological diagnosis of the E‑mail: hariendo@rediffmail.com excised axillary lesion was a schwannoma. At her last follow‑up References appointment 10 weeks after her surgery, she was free of pain and had an advancing Tinel’s sign. The muscle weakness in her 1. de Souza A, Madhusudana SN. Survival from rabies encephalitis. J Neurol Sci 2014 15;339:8‑14. fingers had shown a steady improvement. 2. Escamilla RF, Lisser H. Simmonds’ Disease (Hypophyseal Cachexia): Clinical report of several cases with discussion of diagnosis and Brachial plexus tumours are rare and comprise of only 5% of all treatment. Cal West Med 1938;48:343‑8. tumours of the upper limb.[2] Even though schwannomas may 3. Morley JE, Thomas DR, Wilson MM. Cachexia: Pathophysiology and clinical relevance. Am J Clin Nutr 2006;83:735‑43. be located in any part of the body, cutaneous nerves of the head and neck region and the flexor parts of the extremities Access this article online are the most commonly involved.[3] Website: Quick Response Code
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DOI: 10.4103/0028-3886.156295
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Schwannoma of medial cord
of the brachial plexus: An uncommon localisation a b Figure 1: MRI of the patient. T1-weighted, (a) axial, (b) coronal images show 3x3 cm oval shaped, homogenous contrast enhancementing lesion Sir, Schwannomas are rare, slow growing, encapsulated, benign tumours with regular margins that originate from the schwann cells of the nerve sheath.[1] They are the commonest tumours of peripheral nerves, but less than 5% of all schwannomas arise from the brachial plexus.[2] Due of their rarity and complex anatomical location, they may pose a formidable challenge to surgeons.
A 38‑year old female patient presented with a painful swelling
in her right axilla of 4 months duration. She also complained of numbness on the medial aspect of the right forearm and hand. On examination, she had an oval and firm swelling in the right axilla. In addition, muscle weakness with a constant hypoesthesia was observed in the right index and ring fingers. She had a positive Tinel’s sign. Her breast examination and mammography were normal. A magnetic resonance image (MRI) Figure 2: Per-operative photograph of the tumor and the cords of the revealed a 3 × 3 cm solitary mass in the right axilla, in continuity brachial plexus. Medial cord (white star), lateral cord (black star)
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