|Year : 2023 | Volume
| Issue : 1 | Page : 163-165
Bilateral perisylvian syndrome management and drug-related problems
Nirupama Kulkarni, Sarwamangala S Nanjayyanamath, DS Usha, Sanatkumar B Nyamagoud
Department of Pharmacy Practice, KLE College of Pharmacy, Vidyanagar, Hubballi, A Constitute Unit of K.L.E Academy of Higher Eduction and Research, Belagavi, Karnataka, India
|Date of Submission||30-Jul-2022|
|Date of Acceptance||02-Sep-2022|
|Date of Web Publication||21-Jan-2023|
Dr. D S Usha
Department of Pharmacy Practice, KLE College of Pharmacy, Hubballi, A Constitute Unit of K.L.E Academy of Higher Eduction and Research, Belagavi, Karnataka
Source of Support: None, Conflict of Interest: None
Perisylvian syndrome is a noncurable condition characterized by polymicrogyria in the perisylvian or perirolandic region. Perisylvian means the region or area of the brain that is responsible for language. The bilateral perisylvian syndrome is a congenital neurological condition marked by abnormal neuron distribution in the cortex. We report the case of a 21-year-old female patient with the complaints of recurrent generalized clonic–tonic seizures with intellectual development disorder, loss of consciousness (5–10 min), blurring of vision, poor academic performance, slurring of speech, and history of clubfoot. An intelligence quotient assessment was conducted, and the result showed borderline intelligence. She was diagnosed with a case of bilateral perisylvian syndrome with recurrent seizure disorder with intellectual development disorder and she was treated with anticonvulsant medications.
Keywords: Anticonvulsants toxicity, bilateral perisylvian syndrome, corpus callosotomy, intellectual development disorder, seizures
|How to cite this article:|
Kulkarni N, Nanjayyanamath SS, Usha D S, Nyamagoud SB. Bilateral perisylvian syndrome management and drug-related problems. Indian J Health Sci Biomed Res 2023;16:163-5
|How to cite this URL:|
Kulkarni N, Nanjayyanamath SS, Usha D S, Nyamagoud SB. Bilateral perisylvian syndrome management and drug-related problems. Indian J Health Sci Biomed Res [serial online] 2023 [cited 2023 Jan 28];16:163-5. Available from: https://www.ijournalhs.org/text.asp?2023/16/1/163/368330
| Introduction|| |
Perisylvian means the region or area of the brain that is responsible for language. Perisylvian syndrome is a noncurable condition characterized by polymicrogyria in the perisylvian or perirolandic region. The perirolandic region is the surrounding region of the Rolandic fissure (a region that separates the frontal and parietal lobes). Polymicrogyria is a disorder characterized by faulty brain development before birth. Gyri are stiff or folds seen on the surface of the brain. The brain develops too many folds and is extremely tiny in patients with microgyria.
| Case Report|| |
Here, we report a 21-year-old female presented with a medical history of recurrent seizures (clonic–tonic type) with an intellectual development disorder. She had a seizure episode of 5 min 1 day before admission to the hospital. She has been taking levipil 500 mg 1-0-1 for seizure disorder for 8 years. On clinical examination by the psychiatry unit, she had a history of loss of consciousness (5–10 min), blurring of vision, poor academic performance, and slurring of speech. She had a history of motor delay (clubfoot). A clinical psychologist reference was taken and an intelligence quotient assessment was conducted; the result showed borderline intelligence. Psychiatry history was taken with the patient and informant (mother). The patient's mother gives a history of abnormal behavior for 8 years in the form of dropping things which she would be holding to the ground and decreased muscle tone. She also reports that her daughter experienced these symptoms whenever she was distressed and worried. The patient is able to respond to commands at that time. Psychomotor agitation was normal, and speech was coherent and relevant. CT scan of brain was carried out and in the [Figure 1] it was revealed that bilateral focal cortical thickening extending to the level of the vertex along the Sylvian fissures. Treatment interventions included valproate four tablets in two divided doses (2-0-2), levipil 500 mg twice daily (levetiracetam), clobazam 10 mg at night, and lamotrigine 50 mg twice daily. Electroencephalography findings were within normal limits and had no episode of seizure after hospitalization.
|Figure 1: (a and b) Cranial computed tomography demonstrating bilateral focal cortical thickening extending to the level of the vertex along the Sylvian fissures. Brain MRI scan demonstrating perisylvian polymicrogyria in (c) coronal and (d) sagittal views|
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| Discussion|| |
Causes of congenital bilateral perisylvian syndrome
The exact etiology is unknown; according to sources, it is said that there is an abnormal growth of the outer surface of the brain (cerebral cortex) during embryonic growth (neuronal dysmigration).
Overview of congenital bilateral perisylvian syndrome symptoms
Apnea, aspiration, cerebellar dysplasia (presence of abnormal cells within a tissue that are noncancerous), delayed speech and language development, drooling, intellectual disability, language impairment, and seizures are the symptoms observed in Bilateral Perisylvian Syndrome. Seizures in congenital bilateral perisylvian syndrome (CBPS) typically occur between the ages of 4 and 12 years old and are poorly managed in around 60% of patients. In the series described by Gowda et al., epilepsy was identified in over 90% of the patients. Perisylvian syndrome has a wide range of epileptic manifestations in infants such as general tonic–clonic seizures and typical and atypical absences.
Clinical management of congenital bilateral perisylvian syndrome
The primary therapy for CBPS is anticonvulsants, which prevent and manage seizures triggered by CBPS. If pharmacotherapy that is medications used to control seizures is ineffective then, the physician along with a team of neurologists and surgeons refer and plan for surgical intervention. The surgical procedure is known as corpus callosotomy, and it includes the removal of tissue in specific parts of the brain, which may result in the prevention of recurrent seizures.
Confusion, rapid involuntary eye movements, ataxia lethargy, coma, respiratory depression, and death are the symptoms observed in anticonvulsant toxicity. One of the important anticonvulsant features is to cause depression due to excitatory and inhibitory mechanisms of the central nervous system (CNS).
Dysrhythmia is the most common complication of epilepsy. Few anticonvulsants are known to cause seizures. Medications that block sodium channels, such as lamotrigine, carbamazepine, valproic acid, and phenytoin, can cause cardiac conduction abnormalities as well as abrupt blood pressure and heart rate alterations such as sinus bradycardia, sinus tachycardia, and hypotension.
In this particular case, treatment includes the use of four anticonvulsants. If the patient is stabilized and had no episodes of seizure during hospitalization, withdrawing two or three anticonvulsants is recommended. Lamotrigine has an effect of CYP2C9 enzyme inhibition. Since valproate uses the CYP2C9 enzyme for metabolism, there are chances of an increase in the levels of lamotrigine. Therefore, close monitoring is required since there is suspected lamotrigine toxicity that might result in cardiac complications. The patient might experience a short-term elevation of blood pressure and heart rate changes. Thus, based on the symptoms of toxicity, the patients must be thoroughly evaluated to prevent complications.
| Conclusion|| |
Management of perisylvian syndrome is symptomatic. Since this condition is noncurable, regular counseling from your psychiatrist is important for both physical and mental well-being. Anticonvulsant toxicity was suspected in this case therefore, clinical pharmacists play an important role in identifying drug-related problems and providing suitable dosage requirements, information on suspected toxicity, and adverse drug reaction. Hence, this can improve individualized care and fewer medication errors.
Declaration of patient consent
The authors confirm that they procured the patient consent form. The patient has provided agreement in the form of photos and other patient findings to be published in the journal. We ensure that the patient's names and initials will not be published and made sure that the patient and bystander both are aware of this policy. There were no queries from the patient and bystander.
The author would like to thank KLE College of Pharmacy, department of pharmacy practice take it as a privilege to acknowledge the physicians, nursing staff, and co-interns for guiding us in this case study and extend heartfelt thanks to KIMS Hospital, Hubballi for providing all the facilities.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Narra RK, Krishna AM, Manjeera B, Anusha P. Bilateral Perisylvian syndrome with typical imaging features – A case report. Int J Anat Radiol Surg 2019;8:11-3.
Gowda AK, Mane RS, Kumar A. Congenital bilateral Perislyvian syndrome: Case report and review of literature. J Clin Neonatol 2013;2:196-8.
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