Hydrocephalus and intraspinal tumor in childhood

Hydrocephalus with spinal tumor is rare, and its cause is obscure. We report one patient with a thoracic astrocytoma who developed ventricular dilatation and intracranial hypertension. The pathophysiology is reviewed.     

Status epilepticus in infancy and childhood

Status epilepticus implies a prolonged seizure or recurrent seizures with persistent decreased consciousness, lasting at least 30 minutes. Both convulsive and nonconvulsive forms exist, either of which may lead to death or additional neurologic deficit. Therapy involves careful supportive care plus some combination of benzodiazepines, phenytoin, or phenobarbital. The outcome depends first on the etiology and second on the rapidity with which seizures are controlled.     

Epileptic syndromes in infancy and childhood

PURPOSE OF REVIEW: The aim of this article is to review new epilepsy syndromes, acquire a new understanding of older ones and emphasize the impact of this concept on basic research regarding aetiology and treatment. RECENT FINDINGS: In addition to those included in the classification of the International League Against Epilepsy, new epilepsy syndromes comprise febrile seizures plus, benign familial neonatal-infantile seizures (BFNIS), benign infantile focal epilepsy with midline spikes and waves during sleep (BFIS), malignant migrating partial seizures in infancy, devastating epilepsy in school age children and late onset cryptogenic spasms. Genetics played a central role in identifying some new entities (BFNIS, BFIS with choreoathetosis), to delineate older syndromes (Dravet syndrome and myoclonic astatic epilepsy) and determine their mechanisms (infantile spasms, pyridoxine dependent seizures, neonatal encephalopathy with suppression bursts). SUMMARY: A significant number of children, mainly infants, do not fit in any of the described epilepsy syndromes. Still many patients with infantile epilepsy require the identification of cause or recognition of an epilepsy syndrome.     

Extradural haemorrhage in infancy and childhood

One hundred and two paediatric cases of extradural haemorrhage (EDH) were treated in Adelaide, South Australia, during the period 1954–1988; 10 were infants (0–2 years) and 92 were children (2–14 years). There were 9 deaths (mortality 8.8%). Long-term disabilities severe enough to interfere with school and/or employment were seen in 8 (7.8%) survivors. This relatively low number of adverse outcomes is partly an expression of a low incidence (5.9%) of associated intradural haematomas and few high-velocity impacts due to vehicular accidents. There is reason to believe that the results of treatment have improved in the decade 1977–1988. We attribute this in part to early diagnosis by computed tomography (CT), but a contributory factor may be earlier referrals from country centres to a paediatric trauma centre and rapid transfer, by air or road, by medical retrieval teams.The substance of this paper was presented to the 12th Congress of the European Society for Paediatric Neurosurgery, Warsaw 1990     

Epilepsies of infancy and childhood

This review deals with selected forms of epilepsy beginning after the neonatal period, with special emphasis on those that are highly age dependent for their expression. Experience accumulated in the past decade provides helpful data concerning the clinical-electroencephalographic correlations, remission rate, differential response to medications, and prognosis, although many questions remain unanswered.     

Posterior fossa lesions in childhood and infancy

Forty-three children with CT studies demonstrating abnormalities in the posterior fossa are presented. Tumors constitute the largest group of lesions (53.5%). Their mean size at time of diagnosis was 39.5 mm. Astrocytomas, medulloblastomas, and ependymomas were the most frequent tumors and their CT features are described and discussed. The congenital group of lesions comprised 18.6% of the cases — the most frequent anomaly being arachnoid cyst. In the miscellaneous group (23.3%), cases with atrophy were the most frequent. The cerebellar hemispheres were the most involved sites (39.5%). Lesions located at the tentorial hiatus or near the clivus were rather rare.     

Choroid plexus papillomas in infancy and childhood

The present study deals with 15 cases of choroid plexus papilloma, out of approximately 500 cases of brain tumors observed in children up to 16 years old. Several features are considered, including clinical symptoms and signs—mostly related to increased intracranial pressure—radiological diagnosis, pathology and surgical treatment, and results. Surgery may be radical in most cases, with the exception of histologically malignant papillomas. The management results are usually satisfactory. An adequate choice of surgical approach is mandatory and surgical technique should be meticulous, also in order to avoid the risk of intraoperative tumoral seeding.Presented in part at the 11th Meeting of the European Society for Paediatric Neurosurgery, Naples 1988     

Spongy glio-neuronal dystrophy in infancy and childhood

Summary A clinico-pathological report is given of three children from two different families who, after normal birth and retarded early infantile progress, developed myoclonic and generalized seizures and neurological disorders at the age of one, two and five years, respectively. The progressive malady was characterized by repeated myoclonic jerks, focal seizures, motor weakness with spasticity or flaccidity, abnormal movements, cerebellar symptoms, blindness, deafness, and death in a decerebrate state at the age of 3 to 7,5 years. Necropsy disclosed severe fatty transformation of the liver in case 1. The brains showed spongy degeneration of the grey matter, particularly of the isocortex, with neuronal loss and diffuse astroglial proliferation, spongy change of the deep grey nuclei and spongy atrophy of the cerebellum, most prominent in case 2. The initial changes appeared to be spongy transformation of the neuropil due to vacuolation of neuronal processes and hydropic swelling of astroglia. The grey matter lesion was associated with diffuse gliosis and slight degeneration of the cerebral and cerebellar white matter. In case 1 and 2 peripheral nerve lesions were noted. These observations are compared with other cases from the literature, which are referred to as the idiopathic type of progressive poliodystrophy. From the presumed basic affection of the glio-neuronal unit, the term Spongy Glio-Neuronal Dystrophy in Infancy and Childhood is proposed for this group of cases, which are separated from symptomatic forms of progressive grey matter degeneration in early age. Although a genetically induced metabolic disorder is suggested, the aetiology of this condition is unknown.

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Zusammenfassung Klinisch-pathologischer Bericht über 3 Kinder, davon 2 Geschwister, bei denen nach normaler Geburt und verzögerter Frühentwicklung Myoklonien und generalisierte Krämpfe sowie neurologische Störungen im Alter von 1, 2 und 5 Jahren einsetzten. Das progrediente Krankheitsbild war gekennzeichnet durch Myoklonien, focale Anfälle, Muskelschwäche mit Spastizität oder schlaffer Quadruparese, Bewegungs- und Haltungsstörungen, Kleinhirnsymptome, Blindheit und Taubheit. Tod im Decerebrationszustand im Alter zwischen 3 und 7,5 Jahren. Autoptisch bot Fall 1 eine schwere Leberverfettung. Alle Gehirne zeigten spongiöse Degeneration der grauen Substanz, besonders des Isocortex, mit diffusem Nervenzellausfall und Gliaproliferation. Als Initialveränderung imponierte eine spongiöse Umwandlung des Neuropils durch Vacuolisierung von Nervenzellfortsätzen sowie Astrogliahydrops. Spongiöse Veränderungen betrafen auch die Basalkerne und das Kleinhirn, besonders im Fall 2. Daneben lag eine diffuse Gliose und Marklichtung im Groß- und Kleinhirnmarklager vor. Peripher-nervöse Veränderungen fanden sich in Fall 1 und 2. Diese Beobachtungen werden mit anderen vergleichbaren Fällen des Schrifttums als idiopathische Form der progressiven Poliodystrophie zusammengefaßt. Da die Grundstörung vermutlich die glio-neuronale Funktionseinheit betrifft, wird die Bezeichnung Spongiöse glio-neuronale Dystrophie im Kindes-(und Jugend)alter für diese Syndromgruppe vorgeschlagen. Sie wird von symptomatischen Formen progressiver Schädigung der grauen Hirnsubstanz im frühen Lebensalter abgegrenzt. Obwohl für diese Krankheitsgruppe genetisch bedingte Stoffwechselstörungen vermutet werden, ist ihre Ätiologie bisher unbekannt.

     

Spinal lipomas in infancy and childhood.

The clinical course of 42 children with lipomatous lesions of the lower spine is reviewed. Good neurological exam, IVP and myelography are considered essential components of the preoperative studies. The surgical aim is to free all intra- to extradural adhesions with dural grafting, if necessary. The results suggest that early corrective surgery within the first few weeks to months of life is the most satisfactory approach to preserving normal neurological and urological function in these children. 88% of children not operated on early developed progressive loss of neurological function and while delayed surgery frequently stopped progression of the disease it rarely returned the children to a normal state.     

Cystic cerebral astrocytomas in infancy and childhood: long-term results

24 cases of cystic cerebral astrocytoma represent 11% of 217 supratentorial tumors of the pediatric age group operated. Both macro- and microscopically, such tumors resembled in most cases the well-known cerebellar astrocytoma, presenting as a large cyst with a mural nodule with the pattern of a pilocytic astrocytoma (i.e. spongioblastoma of the German school). The preferential site of incidence was the temporal lobe and the age peaks were of 7 and 16 years without sex prevalence. The surgical technique consisted in the extirpation of the mural nodule, preceded by emptying the cyst, followed by opening the contiguous ventricular wall. The extirpation was partial in one-third of the cases of whom the minority underwent radiotherapy. 1 patient died after the operation while 2 others died from tumor recurrence after 3 and 4 years, respectively, both being mixed tumors (oligoastrocytoma) that were irradiated postoperatively. The follow-up ranged from 10 to 29 years in 70% of the cases. The long-term functional results of the available 17 patients are as follows: good (perfectly well), 12 cases (70%); fair (minor troubles), 5 cases (30%). 3 of the 5 cases with a fair result all had a partial resection.     

Neurodegenerative diseases of infancy and childhood

The neurodegenerative diseases of infancy and childhood include disorders in which there is progressive loss of neurological function due to structural abnormalities of the central nervous system. Well over six hundred disorders, many of which are rarely seen, can be included in this category. Yet, the conditions represent collectively over one-fourth of all admissions to pediatric neurology services. Five-year samples of admission characteristics of 1218 patients from two medical centers over twenty-two years permit an estimate of the frequency of the neurodegenerative diseases. The six most-encountered diagnoses, in declining order, were: subacute sclerosing panencephalitis; neuronal ceroid lipofuscinosis; tuberous sclerosis with degeneration; West disease, or idiopathic degenerative encephalopathy associated with infantile spasms; Werdnig-Hoffmann disease, and hereditary spastic paraplegia. A classification is offered grouping the neurodegenerative disorders into five major categories: polioencephalopathies, leukoencephalopathies, corencephalopathies, spinocerebellopathies, and diffuse encephalopathies. Disorders in each subgroup may be either genetic or nongenetic. Neurodegenerative diseases have multiple causes, including metabolic, viral, immunopathic, environmental, and epileptogenic. The cause of many remains unknown.