1/4. Interictal spike EEG source analysis in hypothalamic hamartoma epilepsy.OBJECTIVE: The epilepsy associated with the hypothalamic hamartomas constitutes a syndrome with peculiar seizures, usually refractory to medical therapy, mild cognitive delay, behavioural problems and multifocal spike activity in the scalp electroencephalogram (EEG). The cortical origin of spikes has been widely assumed but not specifically demonstrated. methods: We present results of a source analysis of interictal spikes from 4 patients (age 2-25 years) with epilepsy and hypothalamic hamartoma, using EEG scalp recordings (32 electrodes) and realistic boundary element models constructed from volumetric magnetic resonance imaging (MRIs). Multifocal spike activity was the most common finding, distributed mainly over the frontal and temporal lobes. A spike classification based on scalp topography was done and averaging within each class performed to improve the signal to noise ratio. Single moving dipole models were used, as well as the Rap-music algorithm. RESULTS: All spikes with good signal to noise ratio were best explained by initial deep sources in the neighbourhood of the hamartoma, with late sources located in the cortex. Not a single patient could have his spike activity explained by a combination of cortical sources. CONCLUSIONS: overall, the results demonstrate a consistent origin of spike activity in the subcortical region in the neighbourhood of the hamartoma, with late spread to cortical areas.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/4. Continuous giggling and autistic disorder associated with hypothalamic hamartoma.We present the case of a child affected since early infancy from recurring episodes of giggling mixed with stereotypical behaviours, mingled with head drops, and eventually with falls, in the context of an autistic disorder. scalp video-EEG recordings revealed an epileptic encephalopathy with generalized slow spike-and-wave complexes alternating with electrodecremental periods, which generally corresponded to the onset of the aforementioned clinical sequences. A resection of a hypothalamic hamartoma was achieved at the age of two. Since then, after 22 months of follow-up, the child is totally free from the giggling and the drops, and the autistic behaviour significantly improved during the second year of follow-up. This case illustrates the difficulties to recognize some subtle ictal manifestations during infancy and childhood, and encourages the early surgical treatment of hypothalamic hamartomas when associated with epileptic encephalopathy and when technically possible.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
3/4. Psychiatric disturbances in children with hamartomas: a neglected somatopsychic issue. A case report.To date, in publications on hamartomas, precocious puberty and laughing seizures have been discussed, but behavioural and cognitive abnormalities have been neglected. Therefore, we report a 14-year-old girl with a proven hamartoma, in which abnormalities of behaviour and cognition played an important role within the somatopsychic complex. In our patient, urinary incontinence during the seizures and psychiatric symptoms, such as eating disorder with obesity, school phobia, antisocial behaviour, withdrawal and cognitive problems (e.g. general slowness, deficiency of cognitive flexibility) came to the fore. The girl had not attended school regularly for almost 2 years, had stayed at home and was overtaxed psychosocially. The seizures and the urinary incontinence improved with drug treatment, but psychiatric difficulties increased and remained untreated until the girl came to a child psychiatric inpatient clinic where drug treatment and behavioural therapy were combined. During well-coordinated neurological and psychiatric treatment the laughing seizures (spontaneous, event-related, psychogenic) decreased and a considerable improvement in psychiatric and psychosocial problems was attained. Consequently, we recommend a close and timely integration of the psychiatric aspects in the treatment of children with hamartomas.- - - - - - - - - - ranking = 4keywords = behaviour (Clic here for more details about this article) |
4/4. Hypothalamic hamartoma in adults.epilepsy in adult patients with hypothalamic hamartoma has not been well studied. It is uncommon but merits recognition. In this paper, 14 adult patients with hypothalamic hamartoma and epilepsy, of whom three developed epilepsy only in adult life, are presented. The later onset of epilepsy appears to be associated with a milder epilepsy syndrome, less severe learning difficulties and behaviour problems, and better occupational and social status. Gelastic seizures are less prominent in this age group. Of particular interest, one patient had prominent sleep disturbances characterized by a reduction in total slow wave and REM sleep without daytime sleepiness. The milder epilepsy and preserved cognitive and social functioning have implications for management. A number of patients were controlled by anticonvulsant medication, and were functioning satisfactorily. For such patients minimally invasive surgical procedures, or medical therapy should be considered.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |