Stiff person syndrome: An unusual paraneoplastic neurological phenomenon in Carcinoid tumour

@#Stiff person syndrome is a rare neurologic disorder characterised by rigidity of the truncal and proximal limb muscles with intermittent superimposed spasms. It’s unique because it lacks similarity to any other neurologic disorder. Possibly tetanus is the closest related condition with both inhibiting central gamma-aminobutyric (GABA) systems. Stiff person syndrome is extremely rare with less than 20 cases reported from South Asia which has a population of nearly 2 billion. In its classic form, it is associated with the presence of high titres of glutamic acid decarboxylase (GAD) antibodies. Paraneoplastic stiff person syndrome comprising of around 5% of the patients has been reported with malignancies of the breast, colon, lung, thymus and Hodgkin’s lymphoma. Antibodies against amphiphysin and gephyrin are detected in paraneoplastic stiff person syndrome. We report a 58 year old Sri Lankan male with stiff person syndrome with a high GAD antibody titre and classical electromyographic changes, who was found to have an underlying carcinoid tumour. We postulate that stiff person syndrome was a paraneoplastic phenomenon secondary to the carcinoid in this case. Although neurological syndromes such as sensory neuropathy, limbic encephalitis and, myelopathy have been described as paraneoplastic features in carcinoid, we believe this is the first report of stiff person syndrome associated with carcinoid tumour.

Extra and Intramuscular Distribution of the Thoracodorsal Nerve with Regard to Nerve Reconstruction Surgeries.

Background The lateral branch of the thoracodorsal nerve (LBTN) is used for nerve transfer in facial, musculocutaneous, axillary nerve injuries and for irreparable C5, C6 spinal nerve lesions and accessory nerve defects. For a successful surgical outcome, the nerve to be used in nerve transfer should be of adequate length and thickness for nerve coaptation. Aim Our objective was to evaluate the length of the LBTN that could be obtained as a donor nerve, externally and within the muscle. Method Eight (8) cadavers with intact upper limbs and thorax which could be positioned in the anatomical position were selected for the study. Cadavers with dissected axillae, brachial plexus or upper limbs were excluded. The thoracodorsal neurovascular bundle was dissected and the number of branches of the thoracodorsal nerve was identified along with its lateral branch. The lateral branch was dissected up to the latissimus dorsi muscle and further intramuscularly. All lengths were measured using a vernier caliper. Results The mean length of the LBTN, up to its first intramuscular branch, is 8.14 cm (range 5.99-12.29 cm). Beyond this, the intramuscular nerve branched further and was of very minute diameter. The mean unbranched intramuscular length of the nerve is 3.36 cm (range 1.3-7.71 cm) which is 41.28% of the total length of the LBTN. Conclusion A significant proportion of the LBTN is found within the latissimus dorsi muscle. This length could potentially be used for direct nerve coaptation by intrafascicular dissection.