A pox tale with a twist

October 8, 2019 0 By FM

While most children are vaccinated against chickenpox during infancy, the disease often attacks us at some point or the other. In the majority of cases, children develop rash-like lesions along with cough/cold and fever which resolves with minimal treatment. The only memories that might remain are perhaps the odd scar left behind after the skin lesions heal.

However, this was not the case for 11-year-old Nathan (name changed). He was initially admitted to a local hospital over muscle weakness, paralysis of all four limbs and breathing difficulty. His parents mentioned that he had started showing chicken pox lesions 5 days earlier and had not been vaccinated against chickenpox during infancy.

Over the next 3 days, his condition continued to worsen – his weakness increased to the point where he was unable to sit or stand and his head started to drop. His breathing difficulty also kept increasing. The doctors recommended that Nathan be transferred to another hospital for advanced treatment, and he was taken to Surya Hospitals, Mumbai. At the time of his arrival at Surya Hospitals, Nathan was practically in a terminal phase with complete limb paralysis and very poor breathing and swallowing power. He was immediately inserted with an endotracheal pipe for assisted breathing. In the next couple of days, Nathan started convulsing and slipped into a coma. He was started on anti-epileptic drugs for his non-stop convulsions, a condition called super refractory status epilepticus.

An EEG was done to help titrate the dose of the anti-epileptics. The number of drugs, as well as their dosages, had to be increased to bring his convulsions under control. Nathan was administered with 9 different anti-epileptic medicines at high doses over the next 5 days before doctors were able to bring the convulsions under control and bring him out of coma.

Meanwhile, the search for what was causing the convulsions continued. A CT scan and MRI, as well as the cerebrospinal fluid viral load test were carried out. The cerebrospinal fluid sample came back positive for the varicella zoster virus and the MRI showed an infection in the brain, indicating that Nathan had encephalitis.

However, the viral load in the brain does not cause muscle weakness and breathing issues. Thus, something else must be going on in Nathan’s case and parallel investigations were carried out. Nerve conduction studies showed a weakness in the nerves in all four limbs as well as in respiratory muscles. Such symptoms are typical of a condition called Guillain-Barré syndrome. Guillain-Barré syndrome (GBS) is an autoimmune disorder that often occurs in response to other infections or medical conditions. The nerve damage causes rapid progressive muscle weakness affecting all four limbs. It normally takes 1-2 months to completely recover from GBS and Nathan was given intravenous immunoglobulin for weakness and maintained on ventilation support for close to one and a half months. By this time, Nathan’s convulsions had completely stabilized and his muscle weakness and breathing issues resolved. He could now be taken off the ventilator and was eventually discharged from the hospital after a 2-month stay on a single anti-epileptic drug. On follow-up, Nathan has shown no neurological issues and is back to normal life.

“Chickenpox complications are rare. Usually, children may get complications affecting the brain or muscles, but very rarely both. Nathan’s case is therefore extremely rare where he suffered from peripheral neuropathy as well as encephalitis,” says Dr. Neil Castellino, Consultant Paediatric Intensivist, Surya Hospitals, Mumbai. “Nathan’s chickenpox complications were challenging and complex. Not only is it rare to have complications, but to have multiple complications is extremely rare”.

Such chickenpox complications occur only in infants when the virus infects other organs, or in children with congenital immune disorders. Congenital immune disorders typically result in frequent infections at earlier stages of life. While Nathan had never been admitted to the hospital previously, he was further investigated to determine if he had any underlying immunity problems. However, all the tests came back negative.

“Nathan was just unlucky to have suffered from multiple complications. Had he been vaccinated against chickenpox during infancy, the chances of any complication would have been close to zero,” says Dr. Castellino, stressing the importance of vaccinations.