[Persistent scalp infestation by Dermanyssus gallinae in an Emilian country-woman]. / Infestazione persistente del cuoio capelluto da Dermanyssus gallinae in una contadina emiliana.

A case of persistent infestation of the scalp due to Dermanyssus gallinae.--Infestation due to Dermanyssus gallinae, the common red mite of poultry, in a country-woman aged 69 years from Crevalcore (Emilia-Romagna region, Northern Italy), is described. The case was unusual either for its location, the scalp, and for its persistence, 9 months. Specimens of the mite were also found in the henhouse adjacent to the patient's house. Apparently the woman recovered by means of daily washing of camomile tea.

Progressive neuronal degeneration of childhood with liver disease ("Alpers' disease"): characteristic neurophysiological features.

A clinico-pathological entity of progressive neuronal degeneration of childhood with liver disease has now been recognised. Onset is in early childhood with intractable fits and progressive dementia. EEG/ERG/VEP studies have been carried out in 12 children with this condition. In most patients the EEG showed strikingly similar and unusual abnormal patterns (high amplitude slow activity together with smaller polyspikes). The flash VEP was usually abnormal and often asymmetrical. In the appropriate clinical setting the neurophysiological features are sufficiently characteristic to aid the clinician in early diagnosis of this autosomal recessive disorder.

Infantile spasms syndrome in monozygotic twins. A 7-year follow-up.

The Infantile Spasms Syndrome is a fairly common form of seizures in infancy. Many papers and several books have been published on this syndrome but several aspects are still obscure. In particular, there is some controversy about anticonvulsant treatments and on the question of improvements in mental status. An unusual case of 2 monozygotic twins with by this syndrome, both with clinical manifestations appearing within a few hours on the same day, at 6 months has been followed up for 7 years, giving us the opportunity to understand some aspects of the clinical course of the disease and long term treatment.

Neurophysiological investigations in GM1 and GM2 gangliosidoses.

Neurophysiological studies (EEG, ERG, VEP and BAEP) have been carried out on a total of fifty-four patients (fourty-five GM2 and nine GM1 gangliosidosis) at various stages of the disease process. In infantile GM2 gangliosidosis, the EEG was midly abnormal from an early age but by the age of one year there was a rapid and progressive deterioration. EEG changes in late onset GM2 gangliosidosis were very variable and unrelated to age or enzyme defect. In both Type 1 and Type 2 GM1 gangliosidosis there was a progressive deterioration of the EEG. Paroxysmal features were not prominent in any of the gangliosidoses, despite the occurrence of seizures. The ERG remained normal in both GM2 and GM1 patients. In the infantile GM2 patients there was progressive loss of the VEP between nine and fifteen months of age but the timing of VEP changes were more variable in all the other groups. Evidence of brainstem dysfunction was found in one of the two TSD patients tested. The combined neurophysiological features appear to be characteristic for each group of gangliosidosis and differ from other neurometabolic disorders of childhood.

'Posterior temporal fast' EEG activity in childhood.

Posterior temporal fast (PTF) EEG activity has been studied in 352 children (706 EEGs) from 1967 to 1982. During this period a total of 42,600 EEGs have been recorded. The rhythmic activity detected as PTF was of 18-30 c/sec but usually at 20-26 c/sec, varying in amplitude between 50 and 300 microV. It appears in runs lasting seconds, often in some sort of irregular spindling. Its frequency but not its amplitude tends to be fixed in each child. This EEG activity (not previously described) appears, or disappears, in response to a variety of environmental stimuli. PTF was seen in over 1% of children between 1 and 3 years of age becoming rare in older children.

Neurophysiological studies in GM1, gangliosidosis.

Neurophysiological studies (EEG, ERG, VEP) have been carried out on 8 children with proven GM1 gangliosidosis (3 of Type I and 5 of Type II). All the EEGs were abnormal showing an increasing amount of irregular slow activity as the disease progressed. Around 2 to 3 years of age, Type II patients often showed a fluctuating 4-5 c/s rhythmic activity especially prominent in the temporal regions. Paroxysmal activity was not a conspicuous feature in any of the patients. The ERG was normal in all cases but the VEP was variably altered. The EEG/ERG/VEP findings in GM1 gangliosidosis differ from those seen in most other neurometabolic disorders of childhood.

"Mitochondrial myopathy" or mitochondrial disease? EEG, ERG, VEP studies in 13 children.

Neurophysiological investigations (EEG, ERG, VEP) were carried out in 13 patients with proven "mitochondrial myopathy". There were nine girls and four boys. Varied abnormalities were seen in the EEGs of all cases, and in one patient unusual repetitive bursts of irregular slow waves and spikes were observed. The ERG was abnormal in five of the 12 cases tested, while the VEP (flash) was definitely abnormal in six out of these 12 cases. These neurophysiological findings suggest some involvement of both the brain and the visual system. It seem therefore appropriate that this condition be considered a "mitochondrial disease" affecting many systems rather than only muscles.

Early diagnosis of herpes simplex encephalitis in childhood. Clinical, neurophysiological and neuroradiological studies.

In the initial phase of HSE the clinical symptomatology is more variable and insidious in babies and young children than in older children and adults. Combined clinical, neurophysiological and neuroradiological studies have been carried out in 12 children with proven HSE. Ten patients had the first EEGs taken during the acute phase of the illness and all showed large amplitude irregular slow activity, sharp waves and often spikes with variable distribution; in 7 cases periodic phenomena were recognisable. At a later stage localised low amplitude EEG activities were found in children with focal neurological symptoms. Areas of low attenuation were seen in the CT scans of the 7 children who had this investigation done at an early stage of their illness. Such low density regions persisted at follow-up and eventually cerebral atrophy with irregular features became obvious. Prompt EEG investigations combined with CT scans provide an early diagnostic clue for treatment. Follow-up EEG studies (including VEP) and CT scans may help assess the severity of residual cerebral damage in the survivors.

Adrenoleucodystrophy: neurophysiological aspects.

Neurophysiological investigations (EEG, ERG, VEP) were carried out in 14 boys with adrenoleucodystrophy, and in two siblings with adrenocortical deficiency, but without neurological symptoms. Irregular large amplitude (200-800 microvolts) slow activity was found in the EEG of all adrenoleucodystrophy patients, usually more prominent over the posterior regions of the brain. No short duration spikes or complex wave were seen in any of the EEGs, even in those patients who had had seizures. Clinical deterioration was not always accompanied by an increase in EEG abnormalities. The ERG was of usual amplitude and wave form, while the VEP (flash) was altered in four cases. The two clinically unaffected siblings had normal ERG/VEP, and only a modest excess of slow waves in the EEG. The neurophysiological findings in adrenoleucodystrophy are not seen in other diseases with similar clinical symptoms in the same age group.

Infantile spasms syndrome in monozygotic twins.

The infantile spasms syndrome appeared on the same day in a pair of monozygotic twins at age 6 months. Clinical, electroencephalographic, and neuroradiological findings during the development of the disease are reported. One of the twins was treated with ACTH and in his case clinical and electroencephalographic improvement was more rapid than in the other who was treated with clonazepam. While the computer tomography scan initially showed in each twin an area of low density in the right fronto-parietal region, this had disappeared in the tomograms obtained about 8 months later.

Accidental glibenclamide ingestion in an infant: clinical and electroencephalographic aspects.

The clinical and EEG features of an infant during and after a severe episode of glibenclamide-induced hypoglycaemia are reported, with a 12-month follow-up. The very few cases reported in the literature, together with the present report, suggest that the neurological sequelae of severe hypoglycaemia resulting from ingestion of this drug are due to more patchy involvement of the central nervous system than would be expected from experimental work on hypoglycaemia.

Neurophysiological investigations in two cases of Alexander's disease with infantile onset.

Repeated neurophysiological investigations over a six year period are reported in two children with histologically proven Alexander's leucodystrophy. The EEG alterations were already detectable at an early stage of the disease with subsequent progressive deterioration in both cases. There was no definite abnormality in the ERG-VEP studies which were only carried out in the younger patient. The EEG features in the course of the illness are different from those seen in most of the identified genetically determined neurometabolic diseases as well as in the group of so-called leucodystrophies of similar age groups. However, the EEG-ERG-VEP features are not sufficiently characteristic to be diagnostic.

Electro-encephalographic investigations in myalgic encephalomyelitis.

The main EEG features are described of thirty-six young adults who were examined at the Royal Free Hospital between 1960 and 1964 and twelve children seen at the Hospital for Sick Children, Great Ormond Street, London, between 1957 and 1977. It is important in the future, if a plan is considered for the study of a fresh epidemic, to include systematic EEG studies covering a period of 2 to 3 years. The EEG alterations found in this limited survey, though modest, would suggest that cerebral function was disturbed with somewhat variable distribution by an insidious illness which has not yet been identified.