Letters to Editor
Pituitary cachexia after rabies
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
severely cachectic state. History revealed that the boy had
suffered an unprovoked bite from a street dog in March
2014 and had presented with a febrile encephalopathic
illness after three weeks. He was initially managed with
broad spectrum antibiotics, ventilatory support, steroids
and other supportive therapy. Cerebro‑spinal fluid (CSF)
showed neutrophilic pleocytosis, hypoglycorrhachia and
elevated proteins. The paired samples of serum and CSF
showed high levels (>1:1,64,000) of rabies virus neutralizing
antibodies. The nuchal skin biopsy stained positive for
the presence of viral antigen confirming the diagnosis of
rabies. MRI revealed lesions in bilateral thalami and in
the cerebellum, sella and brainstem, including the upper
cervical cord [Figure 1a]. He was further managed with
rabies immunoglobulin, physiotherapy, nasogastric feeds
and other supportive therapy. His neurological status,
especially his sensorium, improved gradually over the next
six weeks. However, he developed significant cachexia
despite an intensive physiotherapy and a high caloric
intake, prompting an endocrinological consultation. His
anthropometric measures like body weight (27 kg) and
body mass index (9.3 kg/m2) suggested severe cachexia.
His systemic examination revealed a dry skin, loss of
pubic hair with no evidence of dehydration. His hormonal
profile showed free triiodothyronine‑ 1.1 pmol/L (normal
3 – 6.2), free thyroxine ‑ 0.4 ng/dL (normal 0.8 – 1.2),
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
0 – 8), follicle stimulating hormone (FSH) ‑ 0.5 IU/L (normal
1 – 10), testosterone 126 ng/dL (normal 250 – 820) and IGF‑1
of 86 IU/L (normal 145‑540). His peak cortisol and growth
hormone (GH) after hypoglycemia were 22.4 mg/dL and
1.16 ng/mL, respectively. His body composition analysis (BCA)
by the dual energy X‑ray absorptiometry (DEXA) showed
Neurology India / March 2015 / Volume 63 / Issue 2
a b c
Figure 1: MRI image (a) showing the hyperintense pituitary along with
diffuse cerebral atrophy and body composition analysis before (b) and
after (c) therapy
complete absence of subcutaneous fat [Figure 1b]. He was
diagnosed as a case of pituitary cachexia secondary to rabies
encephalitis and was treated with levothyroxine, growth
hormone, testosterone and vitamin supplements. During
the last follow up, 3 months after the therapy, the body
weight had improved by 4 kg and there is an appearance
of subcutaneous fat at a few places as shown in the repeat
BCA [Figure 1C].
Pituitary cachexia or Simmonds’ disease denotes the complete
destruction of the anterior pituitary gland leading to a
cachectic state. The disease is characterized by cutaneous
changes like loss of secondary sexual characteristics,
hypotension, muscle weakness, and premature senility. Our
patient had developed most of these features fairly rapidly in
a few weeks after the onset of rabies meningoencephalitis.
Survival after rabies infection is reported in only a dozen
patients till date and to the best of our knowledge, none
of them have reported the features of pituitary cachexia.
Simmonds’ disease could be caused by infarction, bleeding,
tumor, inflammation and occasionally infection.[2] Previous
reports suggest hypersexuality and behavioral abnormalities
during the acute phase of rabies infection.
Cachexia is also associated with the presence of a number
of disease states like malignancy, HIV infection, chronic
obstructive pulmonary disease and congestive cardiac
failure. The loss of muscle and fat tissue is due to an
altered endocrinal milieu coupled with increase in the
proinflammatory cytokines. [3] Testosterone and insulin
like growth factor‑1 (IGF‑1) play an important role in the
255
 Letters to Editor

maintenance of the muscle and fat tissue and our patient had
deficiency of both these hormones leading to severe cachexia.
To conclude, we report the case of a young boy, who survived
the deadly rabies infection but had persistence of a cachectic
state resulting from the loss of anterior pituitary hormones.
K. V. S. Hari Kumar, F. M. H. Ahmad, Vijay Dutta
Departments of Endocrinology, Neurology and Respiratory
Medicine, Command Hospital, Chandimandir, Haryana, India
E‑mail: hariendo@rediffmail.com
References
1. de Souza A, Madhusudana SN. Survival from rabies encephalitis.
J Neurol Sci 2014 15;339:8‑14.
2. Escamilla RF, Lisser H. Simmonds’ Disease (Hypophyseal Cachexia):
Clinical report of several cases with discussion of diagnosis and
treatment. Cal West Med 1938;48:343‑8.
3. Morley JE, Thomas DR, Wilson MM. Cachexia: Pathophysiology and
clinical relevance. Am J Clin Nutr 2006;83:735‑43.
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with the inferior trunk of the brachial plexus, posterior to the
pectoralis major muscle and medial to the lateral cord. The
lesion showed a homogeneous enhancement [Figure 1]. A right
axillary approach was adopted to excise the lesion. A 3 × 3 cm,
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
nerve grafting was required. In the early post‑operative period,
the patient complained of an increased loss of sensations over
the ring and little finger. The pathological diagnosis of the
excised axillary lesion was a schwannoma. At her last follow‑up
appointment 10 weeks after her surgery, she was free of pain
and had an advancing Tinel’s sign. The muscle weakness in her
fingers had shown a steady improvement.
Brachial plexus tumours are rare and comprise of only 5% of all
tumours of the upper limb.[2] Even though schwannomas may
be located in any part of the body, cutaneous nerves of the
head and neck region and the flexor parts of the extremities
are the most commonly involved.[3]

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DOI:
10.4103/0028-3886.156295

PMID:
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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)

256 Neurology India / March 2015 / Volume 63 / Issue 2

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