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Porencephaly: Treatment, Cost and Side Effects

Last Updated: May 15, 2024

What is the Porencephaly?

Porencephaly is an extremely rare type of disorder that affects the central nerous system. This neurological cephalic disorder involves encephalomalacia. This rare disorder is characterized by the formation of cysts or cavities on the surface of the brain and within the cerebral hemisphere. There are two types of porencephaly noted. They are, developmental porencephaly and congenital encephaloclastic porencephaly. The cysts and cavities (cystic brain lesions) formed due to porencephaly are thought usually to be the result of destructive (encephaloclastic) cause but can also be formed from abnormal development (malformative), direct damage, inflammation, or hemorrhage.

The cysts and cavities are mostly found within infants and can cause a wide range of physiological, physical, and neurological symptoms. These symptoms affect each patient differently, some may only suffer a deterioration in intelligence or memory while acute attacks cause patients be fatally disabled and might even cause death. The most commonly noted signs and symptoms are delayed growth and development, diminished muscle tone (hypotonia), seizures, and microcephaly or macrocephaly. The additional symptoms are hypotonia or reduced muscle strength, hemiplegia or paralysis of appendages, poor speech development or absent speech, cerebral palsy or a motor condition which causes movement disabilities in the patients.

Up till now, the limited resources and knowledge about this neurological disorder has rendered no cure for porencephaly. The treatment procedure is carried out on the basis of the symptoms noted. The several treatment options available for porencephaly include physical therapy, medication for seizures or epilepsy, rehabilitation, shunt (medical), or neurosurgery that is removal of the cyst. Based on several factors like the location, size of cavities, extent of the lesion, and severity of the disorder, one or combinations of treatment methods are imposed on the patient. Porencephaly patients are provided with drugs and medicines for controlling the seizure. This drug therapy includes valproate, carbamazepine, and clobazam. Also, anti-epileptic drugs serve as another positive method of treatment in the patients.

How is the Porencephaly treatment done?

Diagnosis of sporadic porencephaly can be done prior to or after birth through a variety of methods. There are specialized imaging tests such as an ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI) which can help detect the presence of cysts and cavities in the brain and its surroundings. During an ultrasound test before birth, the reflected sound waves are used to make an image of the developing fetus. CT scan uses a computer and x-rays to create a film showing cross-sectional images of the necessary tissue structures. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. Other assessments for the related defects include memory, speech, or intellect testing. These help further to determine the exact diagnose and severity of and damaged caused by the disorder.

Up till now, the limited resources and knowledge about this neurological disorder has rendered no cure for porencephaly. The treatment procedure is carried out on the basis of the symptoms noted. The several treatment options available for porencephaly include physical therapy, medication for seizures or epilepsy, rehabilitation, shunt (medical), or neurosurgery that is removal of the cyst. Based on several factors like the location, size of cavities, extent of the lesion, and severity of the disorder, one or combinations of treatment methods are imposed on the patient. Porencephaly patients are provided with drugs and medicines for controlling the seizure. This drug therapy includes valproate, carbamazepine, and clobazam. Also, anti-epileptic drugs serve as another positive method of treatment in the patients.

Who is eligible for the treatment? (When is the treatment done?)

Porencephaly signs and symptoms vary from being mild to acute. Some patients affected with porencephaly show no harmful symptoms like seizure but only face slight deterioration of central nervous system. People who show severe and acute symptoms of porencephaly are advised for treatment. These are patients who develop disabling complications and can also face death very soon are eligible for the treatment. Patients with severe and fatal signs and symptoms should consult a doctor and be under medical care and attention.

Who is not eligible for the treatment?

People with extremely mild symptoms of porencephaly do not need to undergo treatment. In these cases, the cysts or cavities do not develop that much and the slight problem faced by these infants are degraded memory and intelligence. They do not need to undergo medical treatment for porencephaly. Also, needless to mention, people with no signs and symptoms of any mental disorder do not need to consult a doctor for treatment.

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Are there any side effects?

There are no noted side effects associated with the treatment methods of porencephaly.

What are the post-treatment guidelines?

Treatment for patients with this rare disorder usually lasts for a lifetime. Their symptoms need to be continuously monitored, specially for those with severe symptoms of disability. Medication, rehabilitation, communication therapies and other physical work outs and speech defects must be worked on efficiently. Only then can the patient live a healthy life without much disabilities or fatal accidents.

How long does it take to recover?

Recovery time needed after the treatment depends on the severity and damage caused by porenephaly. Some people with porencephaly show no major neurological problems. They can live a normal life with self care, thus recovery time is very less. But for patients with acute symptoms and major neurological defect and disabilities, the treatment method is different. Their treatment usually lasts lifelong and with proper care, medicinal treatment and skills, they can recover to lead a normal life.

What is the price of the treatment in India?

Physical therapy, speech therapy and other medications for epilepsy are more or less required by the patient for a lifelong time. Thus, the overall expenditure behind this treatment can be quite high. Besides, neurosurgery or radiation therapy to curb or destroy the cyst or cavity is very high as it involves a great deal of effort. On an approximation, the treatment cost may range between Rs 50,000- Rs2,00,000/-

Are the results of the treatment permanent?

Treatment for the patients of porencephaly will depend on a number of factors which include the location, size of the cyst, the extent of damage caused and the degree of symptoms noted. Treatment for patients with severely effecting porencephaly disorder will last for a life time. Some conditions are so critical that patients die early. For those with severe disability, early diagnosis, medication, participation in rehabilitation to control skills, and communication therapies can significantly improve their conditions and help them live a normal life.

What are the alternatives to the treatment?

So far, there is no such other alternative means of treatment for porencephaly apart from the standardized method. However, homeopathic medicines have proved to be effective in controlling and easing the symptoms of the patients when the treatment is done for long time (around 9-10 months at a stretch). Some homeopathic medicines are effective in the treatment of porencephaly when taken alongside the conventional treatment. Patients show an improved condition in their speech and mental defects.

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Written ByDr. Arun Sharma MBBS,MS - General Surgery,MCh - Neuro SurgeryNeurology
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