"A New Coin Of The Same Value"

I would like to propose for a new term to describe a clinical syndrome to be known as ACUTE IDIOPATHIC ENCEPHALOPHATHIC DISEASE and that this disease causes secondary Respiratory Failure and/or Cardiac Failure.

A two months plus old male child had presented to Miri Hospital yesterday from Bintulu with four days history of fever and treated by a GP symptomatically with antipyretics ( ? type of antipyretics suppository) initially. Developed decreasing alertness and activity on the fourth day with irritability and tendencies for jerking movements ("terkejut") with loud noise and handling. The child also tended to deviate the eyes upwards from time to time. The child then vomited twice the fourth day and was not keen on feeds. Prior to that the child had been keen with feeds, active, alert and quite playful inspite of the fever over the previous days. The child had no running nose or cough; no respiratory difficulty; no diarrhoea; no body rigidity; no tonic clonic convulsions. On the second and third day of the fever the mother had noted some minor rash/ulcers? in the child's mouth which had soon dissappeard. But there never had been any rash on the palms, soles or body. There was no untoward traditional medicines given to the child apart from "Air tawar" given by the father.( "Air tawar" described as pure, cooked water from the kettle and whispered with some traditional magical words, only verbal doings and denies any additional formula into it )

The child's condition had deteriorated quickly on the fourth day with decrease in sensorium, became very lethargic, muscle weakness and respiratory depression. The child subsequently needed intubation to support impending respiraory failure upon arrival at Miri Hospital and hooked on to mechanical ventilation.

Going back to the child's history, as narrated by his mother, the husband works in an office environtment in Bintulu; mom a house wife; non consanguinous marriage; both are Melanau Malays. They live in clean barrack/Flat provided by the employee, Bintulu Development Authority. Youngest of four boys in the family; older siblings are Seven, Five and Three years old. Ironically, the mother had been very well aware about the current " coxsackie viral myocarditis" scare in the news papers and had been taking extra precautions at home with personal hygiene, washing of kitchen utensils and had kept the children indoors all this while. She had only allowed his eldest son to go to back to school after two weeks the school opened. The eldest boy developed a severe mouth disease with ulcers associated with fever and causing much difficulty in feeding followed by the third child and than the second child all with the same similar illness. All were treated by local GP who prescribed oral antibiotics, antipyretics, bongela gel and oral Aid. Their disease lasted about four to five days. None of them had hand or foot lesions. The parents had been well during this time with no flu illness, fevers, diarrheas etc.

Three days after the three older siblings recovered, this patient developed fever which alarmed the parents who than took him to see a private GP.

The child had been a very well child prior to this illness and this happens to be the first time he got sick. He has on mixed breast feeding and Dumex formula.

On examination in the ICU.

CNS: The child's central nervous system was obviously depressed and the child was unconscious and only localised pain by withdrawal. There was loss of motor function with hypotonic muscles. The knee reflexes were rather hyper reflexic and the plantars Babinsky sign were down going. The pupils were pinpoint (2mm) bilaterally (without any sedation) and reacting sluggishly to light. The fontanelles were normotensive.

Respiratory: While on ventilatory support, there was no problem with gas exchange. One very important feature noted is the occasional gasping repiratory efforts made by the child ( no sedation or pavlon used). The lungs were clinically clear and the CXR showed normal lung fields.

CVS: The child's cardiovascular system had appeared markedly compromised initially with tachycardia and poor peripheral perfusions. But improved remarkably after inotropic supports with dobutamine and Amrinone and volume expanders. The tachycardia reduced from over 200 down to 160 per minute and the pulses and tissue perfusion returned to normal.

GIT: Coffee ground material was aspirated from the Ryles tube and was started on i.v zantac and given FFP. No bleeding was noted in buccal mucosae, nostril or urine which looked grossly clear. The liver edge was only palpable under the rib cage and normal. The spleen and kidneys were not palpable.

Skin/Buccal mucosae : The mouth cavity I must say looked very normal and healthy looking without a trace of an ulcer or the slightest inflammation. The skin, palms and soles were free of any rashes.

On Ultrasound the cardiac contractility was within normal limits with no pericardial effusion and a structurally normal heart. The ventricular systems in the brain were normal and no indication of major parenchymal haemmorhages in the brain. The Liver was not enlarged and the kidneys were normal.

Lumbar puncture was non traumatic (courtesy of my luck) at first attempt and the flow was dripping normally. The CSF was clear and colourless. The CSF picture showed increased inflammatory cells( 80) with predominance of lymphocytes (53) while PMN (47). RBC nil; protein 75 and globulin negative.

FBC, platlets, BUSE are nothing exciting.

The child's treatment included Immunoglobulin, Dobutamine, Amrinone, Claforan, Acyclovir ( in view of history of severe oral lesions in the three older siblings) , Zantac, FFP and kiv Dexamethasone and mannitol if signs of cerebral oedema develops.

On the second day in ICU as of today the child condition remains stable. I will continue to extend my assistance to Dr. Ho, MGH Paediatrician who had been working seflessly and on many occasions I must say and sad to say had gone far beyond an ordinary human to extend his energy exceeding the boudaries of the nights and certainly what is due to him.......we left the hospital early this morning.

IN SUMMARY : His clinical deterioration is not a cardivascular one such as the absence of symptoms of pulmonary oedema, enlarged liver, enlarged heart or poor contractility on ultrasound.ECG is normal sinus tachycardia. There is no good evidence in this case for cardiac failure from acute myocarditis.

The prominent clinical picture here is the acute progression of the CNS deterioration and thus the basis for calling it as ACUTE IDIOPATHIC ENCEPHELOPATHIC DISEASE. This is an attempt to describe it just as it is from a purely clinical point of view.

Dr Philip Raja MD,MRCP,DCH
Paediatrician, Miri, Sarawak.