Temporal lobe malacia as a rare cause of gelastic seizure:A case report

BACKGROUND Gelastic seizure(GS)is a rare type of epilepsy that most commonly appears in patients with hypothalamic hamartoma.It is rarely associated with other types of brain damage.This particular type of epilepsy is relatively rare and has few links to other brain lesions.Temporal lobe malacia is mostly caused by cerebral infarction or cerebral hemorrhage,which can lead to seizures.We report a case of GS in a woman with temporal lobe malacia which was reported for the first time in the literature.CASE SUMMARY A 73-year-old female,diagnosed case of GS,presented with repetitive stereotyped laughter a month prior to presentation,happening multiple times daily and with each time lasting for 5-15s.Electroencephalogram displayed a focal seizure seen in the right temporal region.Magnetic resonance imaging head with contrast showed a right temporal lobe malacia.The patient was started on levetiracetam daily.The patient indicated that they had fully recovered and were not experiencing any recurrent or stereotyped laughter during their daily routines.These results remained consistent even after a one-year follow-up period.CONCLUSION GS can be caused by temporal lobe malacia,which is an uncommon but potentially grave condition.The outcome of this present case exhibited the importance of the temporal lobe in the genesis of GS.

Glutamic acid decarboxylase 65-positive autoimmune encephalitis presenting with gelastic seizure, responsive to steroid: A case report

BACKGROUND Anti-glutamic acid decarboxylase(GAD)antibody is known to cause several autoimmune-related situations.The most known relationship is that it may cause type I diabetes.In addition,it was also reported to result in several neurologic syndromes including stiff person syndrome,cerebellar ataxia,and autoimmune encephalitis.Decades ago,isolated epilepsy associated with anti-GAD antibody was first reported.Recently,the association between temporal lobe epilepsy and anti-GAD antibody has been discussed.Currently,with improvements in examination technique,many more autoimmune-related disorders can be diagnosed and treated easier than in the past.CASE SUMMARY A 44-year-old female Asian with a history of end-stage renal disease(without diabetes mellitus)under hemodialysis presented with diffuse abdominal pain.The initial diagnosis was peritonitis complicated with sepsis and paralytic ileus.Her peritonitis was treated and she recovered well,but seizure attack was noticed during hospitalization.The clinical impression was gelastic seizure with the presentation of frequent smiling,head turned to the right side,and eyes staring without focus;the duration was about 5–10 s.Temporal lobe epilepsy was recorded through electroencephalogram,and she was later diagnosed with anti-GAD65 antibody positive autoimmune encephalitis.Her seizure was treated initially with several anticonvulsants but with poor response.However,she showed excellent response to intravenous methylprednisolone pulse therapy.Her consciousness returned to normal,and no more seizures were recorded after 5 d of intravenous methylprednisolone treatment.CONCLUSION In any case presenting with new-onset epilepsy,in addition to performing routine brain imaging to exclude structural lesion and cerebrospinal fluid studies to exclude common etiologies of infection and inflammation,checking the autoimmune profile has to be considered.In the practice of modern medicine,autoimmune-related disorders are relatively treatable and should not be missed.

Neural network mapping of gelastic behavior in children with hypothalamus hamartoma

Background Hypothalamus hamartomas(HHs)are rare,congenital,tumor-like,and nonprogressive malformations resulting in drug-resistant epilepsy,mainly affecting children.Gelastic seizures(GS)are an early hallmark of epilepsy with HH.The aim of this study was to explore the disease progression and the underlying physiopathological mechanisms of pathological laughter in HH.Methods We obtained clinical information and metabolic images of 56 HH patients and utilized ictal semiology evaluation to stratify the specimens into GS-only,GS-plus,and no-GS subgroups and then applied contrasted trajectories inference(cTI)to calculate the pseudotime value and evaluate GS progression.Ordinal logistic regression was performed to identify neuroimaging-clinical predictors of GS,and then voxelwise lesion network-symptom mapping(LNSM)was applied to explore GS-associated brain regions.Results cTI inferred the specific metabolism trajectories of GS progression and revealed increased complexity from GS to other seizure types.This was further validated via actual disease duration(Pearson R=0.532,P=0.028).Male sex[odds ratio(OR)=2.611,P=0.013],low age at seizure onset(OR=0.361,P=0.005),high normalized HH metabolism(OR=−1.971,P=0.037)and severe seizure burden(OR=−0.006,P=0.032)were significant neuroimaging clinical predictors.LNSM revealed that the dysfunctional cortico-subcortico-cerebellar network of GS and the somatosensory cortex(S1)represented a negative correlation.Conclusions This study sheds light on the clinical characteristics and progression of GS in children with HH.We identified distinct subtypes of GS and demonstrated the involvement of specific brain regions at the cortical–subcortical–cerebellar level.These valuable results contribute to our understanding of the neural correlates of GS.

Adult‑onset hypothalamic hamartoma:origin of epilepsy?

Background Hypothalamic hamartoma(HH)is a congenital non-progressive lesion of hypothalamus during fetal development.Mass-like lesions in different anatomical locations often develop a variously disabling course presenting with cognitive decline,psychiatric symptoms,as well as multiple seizure types.As a rare disease,HH is relatively common in infants and children,but it is extremely rare in adults.Case presentation We reported a case of adult-onset hypothalamic hamartoma,and summarized and analyzed relevant reports and studies of HH worldwide.The patient had clinical manifestations characterized by multiple seizure forms.After stereotactic radiofrequency thermocoagulation and drug treatment,the condition was effectively controlled.The patient was followed up till October 2022,with no recurrence of seizures.Conclusions Epilepsy caused by HH can resemble that of temporal lobe seizures,as HH forms a complex epileptogenic network with other regions of the brain through anatomical and functional connections.Early treatment of HH can provide better control of the symptoms of epilepsy,and patients with longer disease courses may have more complications.

The diagnosis and management of hypothalamic hamartomas in children

Hypothalamic hamartoma (HH) is a rare developmental malformation often characterized by gelastic seizures.Recent advances in treating HH have led to dramatic improvements.However,clinical protocol of HH is poorly understood.Since 2002,department Pediatric Neurosurgery of Xinhua Hospital has maintained a multidisciplinary clinical program to evaluate and treat children with HH.This program has provided the opportunity to investigate the management of HH.In this review,we summarize the clinical progress and propose a clear management principle for different HH patients.

^(18)F-FDG-PET glucose hypometabolism pattern in patients with epileptogenic hypothalamic hamartoma

Epileptogenic hypothalamic hamartoma is characterized by intractable gelastic seizures.A systematic analysis of the overall brain metabolic pattern in patients with hypothalamic hamartoma(HH)could facilitate the understanding of the epileptic brain network and the associated brain damage effects of HH.In this study,we retrospectively evaluated 27 patients with epileptogenic HH(8 female patients;age,2–33 years)by using ^(18)F-fluorodeoxyglucose-positron emission tomography.The correlations among tomography result,seizure type,sex,and structural magnetic resonance imaging were assessed.Whole metabolic patterns and voxel-based morphometry findings were assessed by group analysis with healthy controls.Assessment of the whole metabolic pattern in patients with HH revealed several regional metabolic reductions in the cerebrum and an overall metabolic reduction in the cerebellum.In addition,areas showing hypometabolism in the neocortex were more widely distributed ipsilaterally than contralaterally to the HH.Reductions in glucose metabolism and gray matter volume in the neocortex were predominant ipsilateral to the HH.In conclusion,the glucose hypometabolism pattern in patients with epileptogenic HH involved the neocortex,subcortical regions,and cerebellum.The characteristics of glucose hypometabolism differed across seizure type and sex.Reductions in glucose metabolism and structural changes may be based on different mechanisms,but both are likely to occur ipsilateral to the HH in the neocortex.We hypothesized that the dentato-rubro-thalamic tract and cerebro-ponto-cerebellar tract,which are responsible for intercommunication between the cerebral cortex,subcortical regions,and cerebellar regions,may be involved in a pathway related to seizure propagation,particularly gelastic seizures,in patients with HH.