Despite Low Alzheimer’s Disease Rates, CJD Surging In India

CJD Cases Diagnosed Every Month In Mumbai

Murlidhar Ahire lies lifeless in the bedroom of his Vikhroli apartment, while his younger son, Tushar tells us he was an active 69-year-old until two months ago. The retired Air India employee has been diagnosed with Creutzfeldt Jakob disease (CJD), a supposedly rare and deadly neurological brain disorder.

Until 10 days ago, Ahire was at Jaslok Hospital’s isolation ward. But with specialists able to do little except offer supportive treatment, now, one room of the flat has been turned into a nursing unit. Although global incidence is one in a million per year, doctors in Mumbai have noticed a rise in numbers, with one patient diagnosed with CJD every month.

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Research has been unsuccessful at isolating an organism in patients (called a prion actually). What they have learned is that it’s difficult to kill and lacking in genetic information in the form of nucleic acids (DNA or RNA). It is also characterized by an elongated incubation period, sometimes stretching several decades, before symptoms appear.

In keeping with the leading scientific theory, Dr. Annu Aggarwal, consultant neurologist and specialist in cognitive and behavioral neurology with Kokilaben Dhirubhai Ambani Hospital, says prion proteins are to blame. Prion proteins can be normal or infectious. Both contain the same sequence of amino acids, but the infectious form tends to take a folded shape. “Just like a plank of wood must be carved into furniture, proteins have to be folded to form a structure that’s useful to the human body.

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Misfolded proteins known as prions cause many forms of prion disease, including mad cow, Alzheimer’s, chronic wasting, Hutchinson’s and possibly Parkinson’s disease. Prions accumulate in the nervous system and then throughout the body as they convert healthy proteins into deadly ones.” The chain reaction is what leads to brain damage and neuron loss. The disease is indistinguishable from Alzheimer’s disease and the diagnosis between one or the other is often just a coin flip. And the deterioration is often swift. Tushar says Ahire was unrecognizable in a matter of a week.

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It started with a simple feeling of imbalance, which he addressed by using a walking stick. Soon after, during a visit to the bank in September, he found himself clutching onto walls for support before he could be seated. A battery of tests revealed nothing. Doctors advised little other than rest. Bed wetting was followed by dizziness, nausea and weakness.

When Tushar returned home one night, he found his father staring at a wall. “All he said was, ‘Something is happening to me but I don’t know what’,” remembers the self employed mechanical engineer, who is on a sabbatical to care for Ahire.

While subsequent tests continued to reveal little about his condition, he was developing symptoms that would raise the suspicion of any neurologist. “Once, my mother called me at work to say my father had been in the shower for two hours. When he walked out, he said he had spent the usual 10 minutes in there,” Tushar remembers.

He researched online for memory loss and decided to admit him to a hospital. Within the next week, Ahire’s left leg and arm were paralysed. His tonsils had swollen, leaving him unable to swallow or speak. “He spoke slowly in a measured way, as if he was taking time to churn out his thoughts,” says Tushar.

The bigger challenge for family and doctors battling CJD is that in about 85 percent cases, it occurs as a sporadic disease with no recognizable pattern of transmission. Diagnosis is equally baffling.

Ahire’s diagnosis involved an MRI scan to note characteristic patterns of brain degeneration, a CSF test (Cerebro Spinal Fluid) that measures chemicals in the fluid that surrounds the brain and spinal chord and an EEG (Electroencephalogram) to record the brain’s electrical pattern. “Generally, an MRI reflects classic signs that point to CJD, but sometimes, it doesn’t,” says Dr Om Srivastava, director of infectious diseases at Jaslok. Hopeful families insist on running multiple tests even after a diagnosis has been made, which Srivastava calls nothing but an academic exercise. Only a brain biopsy – removal of a piece of tissue from the patient’s brain for examination by a neuropathologist – can confirm the condition. But it’s often discouraged because it’s a complicated and dangerous procedure, and costs a fortune.

It’s often then that doctors use the rule of elimination. They weed out all other possible conditions or types of dementia before arriving at a CJD diagnosis.

The symptoms, often occurring in combination, are a patient’s best signal. While the initial neurological and psychological symptoms include difficulty walking, talking, despair, dizziness, vision problems, anxiety and trouble sleeping, they gradually advance into loss of bladder-bowel control, voluntary movement and memory.

Tushar agrees Ahire had trouble sleeping, and would often complain of being left alone when lights were switched off at bedtime. Unable to speak, chew or swallow, he is now fed every morning with vegetable and lentil puree, protein powder and medicines crushed in water through an abdominal tube.

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Researchers across the world are trying to identify factors that affect prion activity and conditions that make a patient susceptible to the disease. But in the absence of any real cure, doctors say they are reduced to attending to symptoms. “If a patient suffers from anxiety, we prescribe anti-anxiety pills. If he has problem walking, we provide gait therapy,” says Aggarwal.

Another essential part of supportive care is to ensure that hydration and nourishment is accurate so that secondary infections from broken skin otherwise don’t arise.

“When you’re lying in bed, you are predisposed to clots and aspiration in the lungs (which can cause pneumonia) due to secretion deposits,” says Srivastava. Blood thinners are prescribed, and physiotherapy through air mattresses to provide minute vibration and pressure to the lungs is what’s offered.

Families often shoulder the additional burden of misinformation and misdiagnosis. Hospitals, including a suburban one that Ahire was initially admitted to, don’t have isolation rooms.

Considering CJD as a severely contagious disease, they shun patients. It was only when he was moved to an ACU (Ambulatory Care Unit) at Jaslok that his family learned that the condition cannot be transmitted through casual contact (this is not true). It isn’t airborne either, which means isolation isn’t mandatory. It’s only exposure to spinal cord fluid and brain tissue of an infected individual that can lead to an infection. (Untrue. Prions are in all bodily fluids, including saliva. Sneezing and coughing can send prions into the air and onto other surfaces, where sterilization is impossible.)

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