Childhood moyamoya disease.
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Arterial Occlusive Diseases↗
Biomedical subjects
W Isler
Publications and source records attributed to W Isler.
Computed tomography in Hallervorden-Spatz disease.
We report computed tomography (CT) findings of an autopsy-proven case of late-infantile Hallervorden-Spatz disease (HSD). The patient's symptoms started in preschool age with dystonic posture, leading shortly to complete loss of verbal communication and ambulation with marked torsion dystonia. She died aged 23 years. Cranial CT, performed at the age of 18 years, showed moderate infratentorial atrophy, but cortical cerebral atrophy, ventricular enlargement and caudate atrophy were all absent; there were symmetrical areas of increased density in the globus pallidus, and ironstaining pigment deposits at this site were confirmed post mortem. In the appropriate clinical setting such CT findings may be diagnostically helpful in the late-infantile form of HSD. However, experience with CT as well as with magnetic resonance imaging in HSD is still very limited.
Adult↗
Stroke in childhood and adolescence.
Presentation of the author's series (87 cases) of acquired cerebral arterial occlusion in childhood. In the majority of the cases the etiology is unknown. Vasculitis represents a rare category. Follow-up revealed a poor outcome: 11% died, 90% of the survivors are severely disabled.
Adolescent↗
[Congenital analgesia (CA)].
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Diagnosis, Differential↗
Cerebrovascular Doppler-ultrasound examination in children: principle, indication and findings.
The cerebrovascular Doppler examination (cv-Doppler) is a reliable noninvasive method for the diagnosis of obstructions in the extracranial cerebral arteries, and of major arteriovenous shunts in adult patients. The method was applied in 38 children aged 4 months to 17 years. 25 patients with symptoms of cerebrovascular disease underwent cerebral angiography. Six patients had extracranial arterial obstruction, all correctly diagnosed by Doppler. Six children had obstruction of the middle cerebral artery, four were indirectly predicted by Doppler. Four of these twelve patients had extra-intracranial bypass surgery. The patency of the anastomosis could be documented in all cases by Doppler. Ten children had arterio-venous shunts. Of these, 7 patients with an av-angioma and one patient with a carotid-cavernous-sinus fistula were diagnosed correctly by Doppler, as was the cessation of pathologically increased blood flow in the feeding and draining vessels in the 5 patients who had surgical intervention. cv-Doppler results were normal in 3 cases with normal angiography. These results demonstrate that cv-Doppler examination can be useful also in children for the diagnosis of cerebrovascular disease and noninvasive documentation of the hemodynamic effect of neurovascular surgery.
Adolescent↗
Permanent flaccid paraplegia in children with thoracic spinal cord injury.
From January 1960 to March 1979 25 children with spinal cord injury were admitted to our hospital (10 newborns with birth injury to the spinal cord were excluded). Among 12 patients with complete thoracic lesions four remained permanently flaccid. These four cases who had sustained relatively minor trauma showed marked muscular atrophy of the lower limbs, areflexia, absence of anal and cremasteric reflexes, no response to plantar stimulation and no foot deformities. In contrast to children with spastic traumatic paraplegia, motor nerve conduction velocities and H-reflexes were not measurable in these flaccid patients. Myelography was performed in two, this showing myelomalacia below the level of injury. All four patients had clinically an autonomous bladder and voided by gentle manual pressure. The clinical, neurophysiological and radiological findings are consistent with a lower motor neurone lesion below the level of cord injury, resulting presumably from an extensive longitudinal cord lesion on a vascular basis. Judging from Guttmann's experience, flaccid paraplegia occurs in about 12 per cent of adults with complete thoracic cord lesions. The literature is too scant to give an estimate of this complication in children with traumatic paraplegia.
Child↗
Joubert syndrome: clinical and polygraphic observations in a further case.
To our knowledge, only 10 cases of Joubert syndrome have been published so far. In this paper, we describe the clinical, radiological (computerized tomography) and polygraphic findings in an additional patient. The female presented here is the product of consanguineous parents and a sibling of a previously reported infant. In addition to the well-known episodic tachypnea in an awake state, representing the clinical hallmark of this syndrome, this child also had bouts of tachypnea while asleep. Interestingly enough, these were confined only to non-REM sleep.
Cerebellar Ataxia↗
[Traumatic juvenile paraplegia].
We describe the special features and complications connected with the acute phase and long-term course in 28 children with traumatic paraplegia seen for an average duration of 9 1/2 years. Recognition of spinal cord injury was delayed in a third of the cases due to skull and brain trauma and/or multiple injuries. There was subsequently a partial improvement in the neurological status of 7 of 23 children who were initially considered to have sustained complete paraplegia. The extent of independence, education, further training and social integration achieved is impressive and emphasizes the enormous significance of rehabilitation which is particularly complex and laborious in the case of paraplegic children.
Accidents, Traffic↗
Cerebrovascular diseases in the first three years of life.
Congenital and acquired cerebrovascular diseases in the very young age group are reviewed and discussed. Whilst saccular aneurysms are rare arteriovenous malformations and cavernous hemangiomas represent the most frequent types of all congenital anomalies. The aneurysm of the great vein of Galen manifests in the newborn period and mostly is mistaken for congenital heart disease. If the infant survives this angioma causes hydrocephalus. A particular type of phakomatosis combines intracerebral pure venous malformation with homolateral port-wine nevus on the front, often causing epileptic seizures. Spontaneous intracerebral hemorrhage occurs in children with small cavernous hemangiomas. Acquired arterial lesions may develop during fetal life by embolism, causing porencephaly or unilateral brain atrophy. The "Moyamoya" syndrome represents a frequent multi-arterial lesion causing characteristic ischemic episodes. Etiology is still obscure. We do not even know if the disease is acquired or congenital.
Arterial Occlusive Diseases↗
[Dysplasia opticoseptalis].
Dysplasia opticoseptalis was diagnosed in a one-year-old girl. At autopsy an agenesis of the olfactory bulb and other developmental anomalies of the brain were found.
Brain↗
Hemiparesis--an uncommon symptom of hydrocephalus or shunt dysfunction.
We describe a boy with asymmetrical hydrocephalus in whom a marked hemiparesis of recent origin resolved completely following insertion of a shunt. In 2 children with shunted obstructive hydrocephalus due to a midline tumour a newly developed hemiparesis improved markedly in one and resolved in the other case subsequent to revision of a blocked shunt. A hemiparesis should therefore not necessarily be attributed to assumed progression of an underlying disease. Other unusual symptoms of hydrocephalus or shunt dysfunction reported in childhood as well as possible causes of hemiparesis in shunted patients are listed.
Brain Neoplasms↗
Intracranial haemorrhage in the term infant confirmed by computed tomography.
In 8 singleton term infants and 1 postterm infant diagnosis of intracranial haemorrhage (ICH) was clinically established on the 2nd to the 12th day of life and subsequently confirmed by computed tomography (CT). Birth asphyxia was present in 1 infant only. 1 patient died, another developed progressing post-haemorrhagic hydrocephalus requiring a shunt. On follow-up at 2 1/2 to 30 months, psychomotor development was found to be normal in 6 children, mildly delayed in 1 and markedly delayed in another. On comparing our results of ICH in term infants to other published data on both term and preterm infants, we found that in both groups of patients ICH usually occurred within the first 7 days of life and that CT findings were often identical. Mortality rate of preterm babies with ICH was found to be 4.5 times higher than that of term babies while in surviving patients incidence of posthaemorrhagic hydrocephalus was approximately the same. Repeated lumbar punctures were shown to be therapeutically successful in some preterm infants with posthaemorrhagic hydrocephalus. We consider it worth while to use this approach in term infants, too, before placement of a shunt is considered. Etiologic factors and pathogenesis of ICH are briefly discussed at the end of the paper.
Cerebral Hemorrhage↗
[Computertomography for diagnosis of optic nerve and chiasmal gliomas (author's transl)].
Discussion of the clinical symptomatology of optic nerve and chiasmal gliomas based on personal observations. Evaluation of plain X-ray diagnostic procedures, A-scan ultrasonography and especially computertomography for diagnosis of optic nerve and chiasmal gliomas. Discussion of modern attitude in treatment of these tumors: surgical, irradiation therapy.
Cranial Nerve Neoplasms↗
Birth injury to the spinal cord.
11 cases of children with birth injury to the spinal cord born between 1960 and 1970 are presented in review and compared to about 200 previously published cases. 8 children presented at delivery with one or both feet and 2 with breech. 9 of these infants were born by difficult extraction and needed resuscitation due to primary asphyxia. One child had an easy vertex delivery without evident risk. Diagnosis was established within the first days of life, based on flaccid motor and sensory paralysis below a defined level, mostly in the cervical or upper thoracic spine, with bladder and bowel paralysis, and confirmed by autopsy or by follow-up study. One child with disruption of the spinal cord above C4 survived only a few hours despite artificial ventilation. 4 children died within the first three years of life, 3 of them due to acute pneumonia. 5 of 6 surviving children were followed regularly and are now 10 to 18 years old. They are ambulant with crutches and are well integrated in their families. 4 children attend normal school, and one girl gets special training for mentally retarded children.
Adolescent↗
Computed tomography in neurodegenerative disorders in childhood.
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Age Factors↗
Giant axonal neuropathy. Report of a case with normal hair.
A further case of giant axonal neuropathy is described. The diagnosis was made by sural nerve biopsy in a boy presenting signs of a progressive spinocerebellar syndrome with polyneuropathy. Ultrastructurally a severe abnormality of this peripheral nerve, with loss of thick myelinated nerve fibers and the presence of giant axonal swellings was evident. The axonal swellings appeared to be caused by an accumulation of filaments which were also present in Schwann cells, as well as in endothelial and perineurial cells, justifying the term of "generalized microfibrillar hyperplasia". It is most remarkable that the patient's hair was not unusual, in contrast to the previously reported cases who had "kinky hair".
Adult↗
Joubert syndrome: episodic hyperpnea, abnormal eye movements, retardation and ataxia, associated with dysplasia of the cerebellar vermis.
A unique syndrome consisting of episodic hyperpnea, abnormal eye movements, ataxia and mental retardation, associated with agenesis of the cerebellar vermis, has been delineated in four siblings by Joubert et al. (1969). We describe three other children with this clinically recognizable condition which we suggest to call Joubert syndrome. There is good evidence that it is inherited as autosomal recessive. Two of out patients were brothers, the third child's parents were related. Recognition of this syndrome is important in view of prognosis and for genetic counseling.
Adolescent↗
Letter: Computerised axial tomography in spongy degeneration.
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Brain Diseases↗