Progressive Supranuclear Palsy: small brain damage, massive life impact

Progressive supranuclear palsy PSP causes profound and irreversible damage to the brain often before a diagnosis is even made Unlike ALS or Parkinsonrsquos PSP gradually affects balance eyesight and cognition due to the accumulation of tau protein in vital areas of the brain The damage is small but the impact on patients and their families is devastating Yet amid the loss lies an opportunity for learningBrain donation is an empowering and deeply meaningful act one that could pave the way for future PSP treatments But this potential hinges on awareness and early planning In India brain donation for research is a littleknown option Families often face confusion legal ambiguity and difficult decisions during an already painful timeHowever with timely counselling and support from institutions like the National Institute of Mental Health and Neuro Sciences NIMHANS Brain Bank such donations are not only possible but invaluableldquoA single donation can illuminate the path to future curesrdquo says Dr Anita Mahadevan coordinator of the Human Brain Bank and Professor of Neuropathology at NIMHANS Bengaluru in an exclusive interview with middayShe explains the science the systems and the urgent need for public awarenessHow does PSP affect the brain differently from Parkinsonrsquos or ALS Can individual cases help advance early detection or understandingNeurodegenerative diseases like Alzheimerrsquos Parkinsonrsquos and ALS are caused by abnormal accumulation of proteins Tau alphasynuclein TDP43 etc in different regions of the brain The location of these proteins determines the symptomsPSP is a tauopathy marked by tau buildup in the midbrain basal ganglia and brainstem This causes early falls impaired eye movement stiffness and cognitive declineParkinsonrsquos involves alphasynuclein in the substantia nigra leading to tremors slowed movement and rigidityALS affects motor neurons not the basal ganglia leading to muscle weakness and respiratory failureBrain donations offer a ldquoGift of Hoperdquo by allowing scientists to study actual human tissue something animal models cannot replicate Such donations have already led to advances in Parkinsonrsquos and could do the same for currently incurable diseases like PSP and MSAIf someone with PSP dies what should their family do immediately to enable brain donationTime is crucial Ideally the brain should be removed within 1224 hours of death up to 48 hours if the body is refrigeratedStepsgtgt Immediately contact the brain bank coordinator eg NIMHANSgtgt Ensure the body is kept cool in a body freezergtgt Coordinate with a mortuary and pathologist for brain removal this cannot be done at home like eye donationsWhat paperwork is required Can families in Maharashtra still donate to NIMHANS in BengaluruYes Although Maharashtra currently lacks a dedicated brain bank NIMHANS can help coordinate with local hospitals for the retrieval and transport of the brainTo preparegtgt Sign a Donor Declaration amp NOC formgtgt Arrange medical release documentationgtgt Identify a hospitalpathologist in advancegtgt NIMHANS can guide families and help coordinate every step even from other statesWhat are the biggest hurdles families face when trying to donate a brain in Indiaa Lack of awareness Many families and even healthcare professionals donrsquot know that brain donation for research not transplant is possibleb Hospital cooperation Not all hospitals have mortuary services or are willing to assist with postmortem donationsc Legal red tape Organ transplant laws donrsquot clearly cover brain donation for research creating procedural confusion If a death occurs at home some hospitals insist on registering it as a police case before the postmortem causing distress and delaysHowever there are exceptions For example some colleges accept fullbody donations from home deaths These pathways can be used if families are informed and supported earlySecondly the hospitals need to be willing to take on this task as it is beyond the framework of their routine activities It requires a great deal of commitment from the administration as there are no direct beneficiaries nor any revenue generation but only an additional investment for a distant future intended for the betterment of human lives In one notable case a family in Mumbai donated the brain of a loved one with Alzheimerrsquos with help from MGM Hospital and NIMHANSCan the new legal right to a Living Will make brain donation easierYes itrsquos a gamechanger Earlier families could override the deceasedrsquos wishes Now a legal Living Will stating ldquoI wish to donate my brain for medical researchrdquo can strengthen the donorrsquos decision reduce confusion guide families through grief and encourage hospitals to act promptly and respectfullyIs there a simple guide or helpline for families who want to donate a brainYes The NIMHANS Brain Bank provides downloadable pledge forms and a clear stepbystep guidegtgt httpsthenimhansbrainbankindonateQuick checklistgtgt Preregister with NIMHANSgtgt Sign donor declaration amp NOCgtgt Keep forms and hospital contacts readygtgt At time of death refrigerate the body and inform NIMHANS immediatelyHelpline no for brain donation080 26995137