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Hypothalamic hamartomas (HHs) are a known cause of refractory focal epilepsy. Advancement in microsurgical techniques and introduction of stereotactic ablative methods have led to improved complication rates, but the effect on seizure control is still to be determined. In this systematic review, we present a thorough analysis of published literature on the outcomes of various surgical treatments of HHs for refractory epilepsy.
Methods:
A literature search using the MedLine, SCOPUS and Cochrane databases was conducted. All English language studies describing surgical treatment of HH with refractory epilepsy, with a minimum of three patients and a follow-up of at least one year, were identified.
Results:
An initial selection of 55 studies was reduced to 41 after combining studies from the same groups; 14 open, 4 endoscopic, 8 Gamma Knife radiosurgery (GKRS), 9 laser interstitial thermal therapy (LITT) and 6 radiofrequency thermocoagulation (RF-TC) studies were included. From a total of 832 patients, 209 underwent open (25.1%), 80 endoscopic (9.6%), 124 GKRS (14.9%), 229 LITT (27.5%) and 190 RF-TC (22.8%). Engel I or ILAE 1 or 2 was achieved in: open 115 (55.0%), endoscopic 38 (47.5%), GKRS 49 (39.5%), LITT 176 (76.9%) and RF-TC 128 (67.4%). Invasive surgeries (open and endoscopic) had a higher incidence of neurological complications (27.0%) than ablative surgeries (GKRS, LITT, RF-TC) (7.2%). Reoperation rates were higher for ablative surgeries (23.8%) than invasive surgeries (9.0%).
Conclusion:
Surgical treatment of HH causing refractory epilepsy is effective. RF-TC and LITT surgery types have the highest Engel class I outcomes, and ablative surgeries have a lower neurological complication profile compared to open and endoscopic approaches.
Psychiatric symptoms are a common comorbid feature of hypothalamic hamartoma(HH) with epilepsy. They are a significant challenge for patient and their families. Most common psychiatric symptoms are externalizing behaviors such as aggression and defiance.
Objectives
To outline an atypical presentation of HH in form of development of ADHD post-surgery.
Methods
A 6-year old child born out of non-consanguinous marriage, with history of hyperemesis gravidarum and depression in mother in ante-natal period, delivered by NVD at term(did not cry at birth and was hospitalized for 3 days) with birth weight of 2.25 kg, currently presented to Neurology with global developemental delay and history of gelastic seizures since 3 years of age. Patient was diagnosed with pituitary hamartoma(through MRI) and precocious puberty that time and was operated for it after which he started having behavioural issues like irritability, aggression, hyperactivity and lack of appropriate social behaviour with peers along with defiance towards parents. Child was then refered to Psychiatry. On MSE patient did not interact with interviewer and was noticed to shout loudly when confronted for using mobile phone. MRI brain(2 months back) showed post-op changes with cystic lesion in suprasellar region. IQ assessment showed borderline intelligence.
Results
Patient was started on Risperidone(upto 1.5 mg) which lead to some improvement. However antiepileptics are being rationalized to prevent behavioural issues secondary to epilepsy
Conclusions
Patients of HH with epilepsy, present with varied psychiatric symptoms which usually improve after surgery. However we came across a child with worsening of psychiatric symptoms after he was operated for above lesion.
Hypothalamic hamartomas (HH) are an uncommon human pathology resulting in a distinctive and often severe epilepsy syndrome, usually including gelastic (laughing) seizures. This chapter focus on HH associated with epilepsy. The gross anatomy of HH lesions determines the nature of their clinical symptoms. Epilepsy associated with HH is a progressive disease for the majority of patients, with development of multiple seizure types, and coincident deterioration in cognition and behavior. Antiepilepsy drugs (AEDs) are unsuccessful in managing seizures associated with HH, and eradication or complete disconnection of the HH is successful for controlling seizures, and may help ameliorate the comorbid problems with cognition and behavior. Treatment choice is guided by the individual circumstances of the case, including the clinical course of the disease and an assessment of the size and attachment of the HH.
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