Shadowed by a tumor: Astonishing link to a rare nerve disorder

New Delhi: Mumbai’s Jaslok Hospital and Research Centre recently treated a unique and rare neurological condition in a 58-year-old individual. The patient was suffering with multiple symptoms which made it difficult to diagnose. This case showcases the importance of early diagnosis and intervention for better disease management and improved quality of life for patients.

In this case, the patient started to feel burning sensation in both his feet which slowly spread to his legs and arms over the past two years. This alarming development was accompanied by imbalance while walking with falls. He also experienced a tingling sensation in the extremities, making simple tasks like walking on uneven surfaces a challenge. In the last six months, this was accompanied with tremors in his upper extremities. He also was noted to have swelling in his legs.

A thorough examination revealed weak ankle dorsiflexion in both legs, and a peculiar inability to walk on heels. Reflexes were notably absent throughout. Additionally, a tremor had surfaced in the upper limb, raising concerns about the underlying cause. Extensive tests hinted at potential immune system involvement as there was elevated IgG levels along with elevated light chains. Further investigations revealed elevated VEGF (vascular endothelial growth factor levels), thrombocytosis, erythrocytosis and a borderline creatinine level. Nerve conduction studies painted a picture of diffuse primary demyelinating polyneuropathy, indicating a nerve-related complication.

When the doctors did a special scan called PET Scan on the patient, they found an unexpected growth in the hip area. A biopsy was performed by Dr Shrinivas Desai, Director Imaging & Interventional Radiology which showed it was a type of tumor made of a specific kind of blood cells. This tumor (solitary plasmacytoma) was causing an abnormal increase in a particular protein in the blood, which, in turn, was affecting the nerves and causing problems. The overall diagnosis is a rare condition called POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes) a type of a paraproteinemic neuropathy, where nerve issues are connected to this specific tumor. It’s worth noting that this type of tumor is not very common, with fewer than 450 cases reported each year. Managing this condition involves dealing with the tumor, controlling the levels of the problematic protein, and addressing the nerve-related symptoms. The medical team will work together to provide the best care for the patient.

The patient was referred to Dr. Reetu Jain, Director Bone Marrow Transplantation and Dr. Sharmila Agarwal, Director Radiation Oncology to decide regarding tumor management. The patient is currently receiving radiation therapy as part of their treatment plan. This intervention aims to target and shrink the tumor, ultimately improving the patient’s quality of life.

Speaking on the importance of this case, Dr Vinaya Bhandari, Consultant Neurology & Neuromuscular Specialist, Jaslok Hospital Mumbai said, “This case underscores the importance of timely medical intervention and highlights the need for increased awareness regarding rare neurological conditions. Early diagnosis can be a game-changer, potentially altering the course of treatment and improving the quality of life for individuals facing similar challenges. We encourage everyone to stay informed and seek medical advice for any unusual or persistent symptoms. Together, we can make a difference in the lives of those battling rare neurological conditions”.

Speaking on this incredible diagnosis Dr Joy Desai mentioned- “Any neurological disorder requires diligent history taking, comprehensive examination and a judicious analysis of data deciphered. At Jaslok Hospital this process is deeply ingrained resulting in successful outcomes”.

Mamta Kadam, the daughter of Manohar said “I was trying my best to get right treatment for my father, I visited multiple hospitals but it’s only after meeting Dr Vinaya we understood the correct disease of my father and now I am happy he is on the correct treatment plan. I thank Dr Vinaya and Dr Desai for all that they have done for my father.