Eliane Roulet

Benign partial epilepsy of childhood: a longitudinal neuropsychological and EEG study of cognitive function

The study combined prospective neuropsychological and EEG results of 22 children presenting with typical benign partial epilepsy with rolandic spikes (n=19) and occipital spikes (n=3). The aims were to assess the types of cognitive problems which may be encountered in this population, to evaluate the course of cognitive and learning capacities during the active phase of epilepsy, and to see if there was a correlation with paroxysmal activity on the EEG. Average age at entry in the study was 8.4 years and each child was seen two to four times over a period of 1 to 3 years. EEGs showed persistent spike foci in most cases that worsened in three cases, but there were no continuous spike‐waves during sleep. No child had persistent stagnation, marked fluctuations, or a regression in cognitive abilities. Of 22 children, 21 had average IQ (>80). Eight children had school difficulties requiring special adjustment. No single cognitive profile was identified. Four children had delayed language development and eight children had transient weak scores in one isolated domain (verbal, visuospatial, memory) which improved or normalized during the course of the study with concomitant EEG improvement or normalization. In two of the three children with aggravation of the paroxysmal EEG activity, clinical changes were documented. A proportion of children with typical benign partial epilepsy with rolandic spikes showed mild, varied, and transient cognitive difficulties during the course of their epilepsy, and in most cases this probably had a direct relation with the paroxysmal EEG activity.

Autistic Spectrum Disorder: Evaluating a Possible Contributing or Causal Role of Epilepsy

Summary:  The onset of epilepsy in brain systems involved in social communication and/or recognition of emotions can occasionally be the cause of autistic symptoms or may aggravate preexisting autistic symptoms. Knowing that cognitive and/or behavioral abnormalities can be the presenting and sometimes the only symptom of an epileptic disorder or can even be caused by paroxysmal EEG abnormalities without recognized seizures, the possibility that this may apply to autism has given rise to much debate. Epilepsy and/or epileptic EEG abnormalities are frequently associated with autistic disorders in children but this does not necessarily imply that they are the cause; great caution needs to be exercised before drawing any such conclusions. So far, there is no evidence that typical autism can be attributed to an epileptic disorder, even in those children with a history of regression after normal early development. Nevertheless, there are several early epilepsies (late infantile spasms, partial complex epilepsies, epilepsies with CSWS, early forms of Landau‐Kleffner syndrome) and with different etiologies (tuberous sclerosis is an important model of these situations) in which a direct relationship between epilepsy and some features of autism may be suspected. In young children who primarily have language regression (and who may have autistic features) without evident cause, and in whom paroxysmal focal EEG abnormalities are also found, the possible direct role of epilepsy can only be evaluated in longitudinal studies.

Home and hospital treatment of acute seizures in children with nasal midazolam

Rectal diazepam is widely used in the treatment of acute seizures in children but has some disadvantages. Nasal/sublingual midazolam administration has been recently investigated for this purpose but never at home or in a general paediatric hospital. The aim of this open study was to determine the efficacy, the tolerance and the applicability of nasal midazolam during acute seizures in children both in hospital and at home. We included known epileptic children for treatment at home and all children with acute seizures in the hospital. In all, 26 children were enrolled, 11 at home and 17 in the hospital (including two treated in both locations); only one had simple febrile seizure. They had a total of 125 seizures; 122 seizures (98%) stopped within 10 minutes (average 3.6 minutes). Two patients in the hospital did not respond and in three, seizures recurred within 3 hours. None had serious adverse effects. Parents had no difficulties administering the drug at home. Most of those who were using rectal diazepam found that nasal midazolam was easier to use and that postictal recovery was faster. Among 15 children who received the drug under electroencephalogram monitoring (six without clinical seizures), the paroxysmal activity disappeared in ten and decreased in three. Nasal midazolam is efficient in the treatment of acute seizures. It appears to be safe and most useful outside the hospital in severe epilepsies, particularly in older children because it is easy for parents to use. These data should be confirmed in a larger sample of children. Its usefulness in febrile convulsions also remains to be evaluated.

Benign paroxysmal torticollis in infancy.

References 1 . Kinsbourne, M . (1962) ‘Myoclonic encephalopathy of infants.’ Journal of Neurology, Neurosurgery and Psychiatry, 25, 27 1-276. 2. Boltshauser, E. , Deonna, Th., Hirt, H. R. (11979) ‘Myoclonic encephalopathy of infants or “dancing eyes syndrome” .’ Helvetica Paedialrica Actu, 34, 119-133. 3 . Allen, J . P . , Kandall, J . W . , McCilvra, R . , Vancoura, C. (1974) ‘Immunoreactive ACTH in cerebrospinal fluid.’ Journal of Clinical Endocrinology and Metabolism, 38, 586-593. 4. Lagenstein, I . , Willig, R. P., Kuhue, D. (1979) ‘Cranial computer tomography in children treated with ACTH and dexamethasone: first results.’ Neuropediutrics, 10, 370-384. 5 . Doria, L. , Pisaturo, C. , de Negri, M. (1983) ‘Modalitees et effects secondaires du traitement par I’ACTH dans les epilepsies diffuses chez I’enfant.’ Neuropsychiatrie de I’Enfance et de I’Adolescence, 31, 531-536.

Post-traumatic mutism in children: clinical characteristics, pattern of recovery and clinicopathological correlations

Among the numerous clinical syndromes observed after severe traumatic head injury, post-traumatic mutism is a disorder rarely reported in adults and not studied in any detail in children. We report seven children between the ages of 3 1/2 and 14 years who sustained severe head injury and developed post-traumatic mutism. We aim to give a precise clinical characterization of this disorder, discuss differential diagnosis and correlations with brain imaging and suggest its probable neurological substrate. After a coma lasting from 5 to 25 days, the seven patients who suffered from post-traumatic mutism went through a period of total absence of verbal production lasting from 5 to 94 days, associated with the recovery of non-verbal communication skills and emotional vocalization. During the first days after the recovery of speech, all patients were able to produce correct small sentences with a hypophonic and monotonous voice, moderate dysarthria, word finding difficulties but no signs of aphasia, and preserved oral comprehension. The neurological signs in the acute phase (III nerve paresis in three of seven patients, signs of autonomic dysfunctions in five of seven patients), the results of the brain imaging and the experimental animal data all suggest the involvement of mesencephalic structures as playing a key role in the aetiology of post-traumatic mutism.

Treatment of acquired aphasia and epilepsy.

SIR-In his letter to this journal (DMCN, 32, 930), Pascual-Castroviejo claims that acquired aphasia and epilepsy (AAE) (Landau-Kleffner syndrome) is ‘caused by isolated cerebral arteritis’ and that ‘calcium antagonists, and nicardipine in particular, are very effective as adjuvant drugs for this disorder’. In support of the first claim, the author mentions six personal cases who had vasculitis on cerebral angiography and increased protein in the cerebrospinal fluid in all cases. In the last 30 years, several children with AAE have had angiography and CSF studies, which have always been negative. One must conclude that Pascual-Castroviejo has seen angiographic abnormalities that have eluded others, or that he is studying a special condition. Cerebral vasculitis at times may well involve cortical language areas and lead to seizures and aphasia’. No wever, with vasculitis which was either ‘disseminated’ or ‘localized in branches of the right or left middle cerebral arteries’, one wonders how aphasia can be the sole and the same neurological abnormality.

Deonna and Roulet reply

ity can be fairly well compensated for with head movements. Eyes: normal fundi. Neuroimaging: moderate periventricular leukomalacia documented with MRI. Functional diagnosis: severe cerebral visual impairment. Child 2 was 7 years old, with grating acuity 38 c/d, linear optotype acuity RE (right eye) = LE (left eye) 1.0. Fields: normal Goldmann12 fields. WISC: Verbal IQ 140, Performance IQ 52, Full Scale IQ 98. Eye motility: normal. Eyes: normal fundi. Neuroimaging was not performed. Functional diagnosis: Asperger syndrome, complicated by a severe visual perceptualcognitive disorder. Child 3 was 9 years old, with grating acuity 38 c/d, linear optotype acuity RE 0.63, LE 0.2. Fields: left homonymous hemianopia, increased threshold, and mild restriction of the right hemifield measured with Goldmann12 perimetry. WISC: Verbal IQ 70, Performance IQ 52, Full Scale IQ 57. Eye motility: esotropia and dysmetric saccades. Eyes: normal fundi. Neuroimaging: CT performed in the neonatal period verified a unilateral brain haemorrhage, resulting in a later documented atrophy of the right occipital lobe. Functional diagnosis: moderate cerebral visual impairment in combination with mild cerebral palsy (hemiplegia) and mild learning disability. Child 4 was 5 years old, with grating acuity 1.2 c/d, linear optotype acuity RE 0, LE 0.07. Fields: RE blind LE restricted confrontational field. WISC: Verbal IQ 62, Performance IQ 32, Full Scale IQ 42. Eye motility: esotropia, manifest nystagmus, poor fixation. Eyes: cicatricial retinopathy of prematurity (ROP), myopia. Neuroimaging: mild periventricular leukomalacia documented with CT. Functional diagnosis: severe ocular visual impairment caused by ROP, which was aggravated by visual perceptual-cognitive problems in combination with attention deficit disorder and severe learning disability. These children present with a variety of disabilities due to cerebral dysfunction, and their functional diagnoses may be debated. Although they all have visual problems, only two children (1 and 3) fulfil our proposed criteria for the functional diagnosis of cerebral visual impairment. The practical visual problems described in child 2 correspond to the subnormal Performance IQ, but cannot be evaluated or graded by the ophthalmologist, because all tests of vision and the eye examination were normal. In this case, the dysfunction fulfils the criteria for a neuropsychiatric diagnosis. Child 4 represents a group of visually impaired children with lesions of both the eyes and the posterior visual pathways. Here, the severe retinal disease is obviously the main cause of low visual input (i.e. ocular visual impairment), but in daily life, visual problems are aggravated by the inability to interpret visual information. All four children have problems with reading. Child 1 prefers braille to print. The basis for the functional diagnosis of cerebral visual impairment calls for a multi-disciplinary approach: a task for paediatric ophthalmologists in collaboration with psychologists, paediatric neurologists, paediatric psychiatrists, and neuroradiologists. In the future we need a model to quantify the degree of this functional disorder. A standard assessment procedure that includes visual, ocular motor, cognitive, and behavioural functions is also required. The aim of such a diagnostic procedure is to give support to the child, parents and teachers, and to work out strategies for optimal habilitation and for adapted education. Lena Jacobson MD PhDa Ulla Ek PhD Jan Ygge MD PhD Mette Warburg MD DMedSci