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Biomedical subjects

B Kendall

Publications and source records attributed to B Kendall.

At least 19 recordsLinked to original sources
PubMed1995

Cortical dysgenesis: serial EEG findings in children and adults.

Cortical dysgenesis (CD) is becoming increasingly recognised as a cause of epilepsy in otherwise cryptogenic cases. We describe the serial EEG findings in 22 patients with focal/localised CD. The EEGs covered a minimum period of 5 years in each case (median = 13 years, range: 5-30 years), beginning in childhood. Median age at seizure onset was 3 years (range: 3 weeks-10 years, n = 21). The EEG was normal in the one patient, a 6 year old, who did not have epilepsy. Background rhythms appropriate for age were preserved in the majority of patients (18/22). Slow activity localised to the area of CD was seen in 11 patients; in 3 patients, this did not appear until the second decade of life. Epileptiform discharges were seen in at least one EEG in 20 patients: these were continuous or near-continuous (6 patients) or occurred recurrently in short runs (6 patients). In 6 patients, these discharges appeared only after the second decade of life and in 11 patients, they became more widespread over time. In the remaining patients, the EEG changes did not evolve. Sleep failed to produce new abnormalities (n = 15). None of the patients showed EEG features characteristic of lissencephaly or evolution to the Lennox-Gastaut syndrome. Even in this selected cohort of patients who had undergone serial clinical EEGs, the EEG abnormalities in focal/localised CD appeared relatively stable and showed only moderate changes over time. CD must be included in the differential diagnosis of any patient who presents with localised slow activity on EEG.

Adolescent↗
PubMed1995

Visual evoked potentials in phenylketonuria: association with brain MRI, dietary state, and IQ.

At separate institutions, pattern reversal visual evoked potentials (VEPs) were recorded in children and older patients with phenylketonuria and compared with MRI of the brain. In nine patients aged less than 14 years, who were still on a diet low in phenylalanine, VEPs were clearly abnormal in only one and the abnormalities seen on MRI were mild. In 27 patients aged 14-31 years VEPs were abnormal in more than 80%, with significant reduction of amplitude and prolongation of latency despite the general absence of visual symptoms and abnormalities on routine neuro-ophthalmological examination. Among the older patients there was no significant correlation between VEP measures and plasma phenylalanine or tyrosine concentrations; neither was the incidence of VEP abnormalities dependent on whether or not the patients were still on a low phenylalanine diet. Some VEP amplitude measures were inversely correlated with the MRI lesion score, perhaps reflecting the severity of white matter abnormalities in the parieto-occipital region. In the older patients the amplitude of VEPs to stimulation of the central 8 degrees of the visual field was significantly correlated with IQ. The study confirms the high incidence of subclinical visual pathway involvement in older children and adults with phenylketonuria, and suggests the possibility of a link between the abnormal appearance of subcortical white matter on MRI and a physiological index of function of the CNS. As there was no evidence of general intellectual decline, it is suggested that the correlation between central field VEP amplitude and IQ may reflect abnormal development during infancy. Abnormalities on MRI, on the other hand, seem to be more closely related to current dietary state and phenylalanine concentration.

Adolescent↗
PubMed1995

Cognitive functioning and magnetic resonance imaging in chronic fatigue.

BACKGROUND: This study examines whether cognitive dysfunction in chronic fatigue may be accounted for by depression and anxiety or is due to brain pathology evident on magnetic resonance imaging (MRI). METHOD: Twenty-six subjects with chronic fatigue, with and without coexisting depression, and 18 age-matched normal controls were recruited from primary care following a presumed viral illness six months previously. Comparison was made with 13 psychiatric controls with depressive illness on standardised cognitive tests. MRI determined the presence of cerebral white-matter lesions. RESULTS: No substantial differences in performance were shown between subjects with chronic fatigue, most of whom met the criteria for chronic fatigue syndrome, and controls. Subjective cognitive dysfunction increased with psychopathology. White-matter lesions were found in a minority from all groups. Improvement in fatigue and depression coincided with improved performance on cognitive measures. CONCLUSIONS: Subjective complaints of cognitive impairment are a prominent feature of chronic fatigue, but objective cognitive and MRI abnormalities are not. Such complaints probably reflect psychopathology rather than a post-viral process.

Adolescent↗
PubMed1994

New autosomal dominant form of spondyloepiphyseal dysplasia presenting with atlanto-axial instability.

We present a family with a radiologically distinct new form of autosomal dominant spondyloepiphyseal dysplasia, presenting with cervical instability and attendant neurological compromise and emphasise the radiological characteristics which delineate this condition. Cervical vertebral abnormalities, including malformation of the odontoid process, have been observed in some forms of spondyloepiphyseal dysplasia, but rarely lead to neurological sequelae, in contrast to the pedigree we describe.

Atlanto-Axial Joint↗
PubMed1994

Autosomal recessive congenital intrauterine infection-like syndrome of microcephaly, intracranial calcification, and CNS disease.

We present data on 10 patients from 5 families with a condition of microcephaly, intracranial calcification, and a clinical course resembling congenital TORCH infection. Repeatedly, negative TORCH investigations are a prerequisite for the identification of this disorder and the value of disturbed liver function and thrombocytopenia as aids to diagnosis is emphasised. Several similar families with recurrence of the disease in sibships are identified in the literature and the genetic implications of our observations are considered.

Brain↗
PubMed1994

Unusual appearances of primary central nervous system non-Hodgkin's lymphoma.

There is an increasing incidence of primary central nervous system (CNS) non-Hodgkin's lymphoma (NHL) in immunocompromised and immunocompetent patients. The lymphoma commonly abuts a ventricular or meningeal surface and enhances homogeneously after injection of intravenous contrast medium. The radiological features of 39 patients with cerebral lymphoma were reviewed and five patients with less commonly observed manifestations of this disorder are presented. A differential diagnosis of lateral and third ventricular masses is given.

Aged↗
PubMed1994

Parotid haemangiomas in childhood: a case for MRI.

Seven infants with unilateral parotid haemangiomas seen at one centre are presented. Their case notes and special investigations are reviewed. Magnetic resonance imaging (MRI) is revealed as the investigation of choice because of picture quality, definition of soft tissues and lack of exposure to ionizing radiation. MRI allows a definite diagnosis to be made without any invasive procedure being required. A future study following up these infants may be of interest because of controversy regarding the spontaneous resolution of these lesions.

Age of Onset↗
PubMed1994

Dysembryoplastic neuroepithelial tumor. Features in 16 patients.

Dysembryoplastic neuroepithelial tumour (DNT) is a newly recognized brain mass lesion with distinctive pathological features and a favourable prognosis. We reviewed the clinical, electroencephalographic, neuroimaging and pathological features of 16 patients with DNT who underwent surgery; only one patient did not have epilepsy. Mean age at seizure onset was 9.5 years (range: 1 week to 30 years) and surgery 17 years (range: 7 months to 37 years). The mean verbal IQ was 94.6 (range: 79-110) and performance IQ 105 (range: 79-130) (n = 10). The EEG was abnormal in all cases reviewed (n = 13): localized slow activity was seen in 12 and interictal spiking in 10 patients, being less extensive than or concordant with the lesion in three and more extensive than or distant to the lesion in seven. X-ray CT was normal in three out of 11 patients. Magnetic resonance imaging provided detailed anatomical information: the lesion was predominantly intracortical, although in six patients, there was also white matter involvement. The lesion involved the temporal lobe in all but one patient where it was in the cingulate gyrus. Of the temporal lobe cases, MRI showed that the lesion involved, or was in close proximity to, mesial temporal structures in 11 out of 14 patients. Other magnetic resonance features included: circumscribed hyperintensity on long TE/TR images (10 patients), hypointensity on short TR images (12 patients), and cyst formation (five patients). Calcification was seen on CT in four patients. Post surgical follow-up ranged from 8 to 30 months (mean 16.2 months): 12 patients are seizure free and two have a > 80% reduction in seizure frequency (n = 14). Histopathological characteristics included a heterogeneous composition in all cases, calcification (13 cases), dysplastic features (12 cases) and isolated foci of subpial spread (five cases). The presence of occasional mitoses in 12 cases and immunoreactivity to the proliferating cell nuclear antigen in six cases indicate that these lesions have cellular proliferative activity and that there may be a need to follow these patients postoperatively.

Adolescent↗
PubMed1992

Magnetic resonance imaging in the assessment of craniosynostosis.

Modern interest in craniosynostosis began when clinicians, regardless of their individual specialty but united in their interest in the problems raised by patients with this condition, first realised that if treatment was confined simply to opening up the fused sutures of the skull vault, success in terms of both function and the restoration of a more normal appearance was likely to be limited. Indeed, the grouping together of such specialists into formal craniofacial units was tacit recognition of this, as was the acceptance that many affected children had evidence of clinical problems that could not be explained on simple mechanical grounds alone. It is not surprising, therefore, that the advent of any new method of investigation has been welcomed by craniofacial surgeons eager to learn anything that might lead to an improvement in management, particularly of the more complex syndromes.

Brain↗
PubMed1991

CT and MR imaging in lateral sinus thrombosis.

Establishing a pre-operative diagnosis of lateral sinus thrombosis can be difficult, as the clinical features are non-specific and laboratory tests unhelpful. CT may demonstrate abnormal high density of the lumen of the sinus, which does not enhance after intravenous contrast medium. Enhancement of the dura surrounding the sinus may be prominent, causing the 'empty triangle' or 'delta sign' which may suggest the diagnosis. Magnetic resonance (MR) imaging may show both lack of flow and abnormal signal from the sinus, thus providing definitive evidence of thrombosis.

Adolescent↗
PubMed1991

Pelizaeus-Merzbacher disease: classical or connatal?

The clinical features and investigation results of 7 patients with Pelizaeus-Merzbacher disease (PMD) are described; one patient had a brain biopsy and two patients had an autopsy. This paper tries to differentiate the clinical features of the connatal and classical types of PMD. Transient stridor and nystagmus were early signs in both types of PMD. Our findings support the view that the more severe connatal form shows rapid neurological deterioration from an early age leading to death usually in the first decade. In younger patients in whom the evolution is still unclear, severe feeding problems and extrapyramidal features may suggest the connatal form. By contrast, in the classical form of PMD, cerebellar signs and cognitive deterioration are more prominent with a more slowly progressive course. Nuclear magnetic resonance imaging and brainstem auditory evoked potentials were very helpful in supporting the diagnosis of PMD either in a known affected family or in sporadic cases, but were not useful in distinguishing between the two types of PMD. Genetic counseling in this condition is difficult, particularly in the connatal form in which inheritance may be either X-linked or autosomal recessive.

Age Factors↗
PubMed1991

The clinical significance of seizures in critically ill young infants requiring intensive care.

The aetiology, severity of systemic and biochemical abnormalities, seizure duration, EEGs and CT scans have been reviewed in previously normal young infants with an acute critical illness occurring after the first week of life; none of whom had birth asphyxia. Findings were related to outcome in an attempt to evaluate the significance of seizures during the acute phase of severe illness. In three years seizures occurred in 54/251 (22%) young infants requiring ventilatory support. In these patients the acute illness was most commonly infection and encephalitis/encephalopathy. Twenty-one died, 24 had good or moderate outcome and 9 poor outcome (follow-up 6-27 months). The outcome was not directly related to diagnosis, but to systemic and biochemical changes, the most important being severe hypotension (40/54). After correction of these factors, in survivors, increasing number of days over which seizures occurred during the acute phase of illness was related to worsening outcome (tau(c) = 0.66, p less than 0.0001). In many of these patients one of three abnormal low density changes (generalized, boundary zone and focal) were seen on CT scan and were not uncommonly associated with focal/multifocal clonic seizures and characteristic type, distribution and evolution of EEG discharge. In the 45 patients with EEGs from presentation, severity of encephalopathy assessed by predominant background EEG activity was most closely related to outcome, irrespective of aetiology and seizures. There was a significant relationship between graded severity of background EEG activities and outcome both in the initial and serial recordings (tauB = 0.70, p less than 0.0001 and 0.75, p less than 0.0001 respectively). Seizures are a common occurrence in the previously well young infant with an acute critical illness necessitating intensive care. Prompt recognition and treatment may influence outcome in patients with a potentially reversible encephalopathy. However, in many patients seizures reflect severe, often multifactorial cerebral insult with variable morphological changes, EEG patterns and clinical outcome.

Anticonvulsants↗
PubMed1991

Intracranial neurenteric cysts. Report of two cases.

Two cases of intracranial neurenteric cysts are reported and the literature is reviewed. Neurenteric cysts are rare congenital lesions that tend to occur in the spinal intradural space. An intracranial location is exceptional.

Adult↗
PubMed1990

Neurological deterioration in young adults with phenylketonuria.

7 patients with phenylketonuria who developed neurological disability in adolescence or early adult life are described. 4 had been diagnosed by routine neonatal screening and started a low phenylalanine diet in infancy. 3 were diagnosed in early childhood because of developmental delay, and then started dietary treatment. Dietary control deteriorated in later years and was withdrawn in mid to late childhood. The late neurological deterioration cannot be directly ascribed to poor compliance with or cessation of dietary treatment in this small, retrospective study--but other likely causes have been excluded and 2 patients showed a striking clinical improvement when a strict diet was resumed. Serial magnetic resonance images from one of these patients show abnormalities that appeared after cessation of dietary treatment and resolved after diet was resumed. If these findings are confirmed, strict dietary control into adult life would be indicated for at least some patients with phenylketonuria.

Adult↗
PubMed1990

Convergence nystagmus associated with Arnold-Chiari malformation.

A case of convergence nystagmus associated with an Arnold-Chiari type I malformation is presented. The nystagmus appeared in the absence of fixation, was provoked during Valsalva's maneuver and neck flexion and extension, and attenuated on deep inspiration. Sagittal magnetic resonance images showed that the diameter of the cerebral aqueduct increased with the neck in full flexion and in full extension. Surgical foramen magnum decompression considerably reduced the nystagmus and abolished the postural variation of aqueduct diameter. It is postulated that this nystagmus was due to a combination of mechanical distortion and abnormal transmission of cerebrospinal fluid pressure to the aqueductal region.

Adolescent↗
PubMed1990

Hemifacial spasm in infancy.

Three infants presented with the onset of hemifacial spasm after birth and at the age of 10 months. One patient was found to have occlusion of the straight sinus and large collateral veins at the base of the brain, presumably due to venous sinus thrombosis, supporting the concept of vascular compression of the facial nerve at its exit from the brain stem as a mechanism for the production of hemifacial spasm. The other patients each had an intrinsic mass compressing the fourth ventricle, located in the lower pons and extending into the cerebellar vermis and right cerebellar peduncle in one; in the other patient, the mass involved the cerebellar vermis and right middle cerebellar peduncle alone. These patients widen the etiologic spectrum of the syndrome and show that serious intracranial diseases may underlie hemifacial spasm in infancy.

Brain Neoplasms↗