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Biomedical subjects

P Landrieu

Publications and source records attributed to P Landrieu.

At least 19 recordsLinked to original sources
PubMed1992

Coma associated with intense bursts of abnormal movements and long-lasting cognitive disturbances: an acute encephalopathy of obscure origin.

We report six previously healthy children who several days after a prodromal illness had an acute encephalopathy that ran a biphasic course. It appears to constitute a recognizable syndrome with a good prognosis that can be differentiated from other encephalopathies of obscure origin as previously defined by Lyon et al. The active phase was dominated by coma or confusion and by abnormal movements, including disordered gesticulation and attacks of orofacial dyskinesia or limb dystonia associated with permanent rigidity and culminating in opisthotonic posturing. Repeated seizures were observed in only two patients. Permanent slow waves were recorded on the electroencephalogram in all patients, even during bursts of abnormal movements. Cerebrospinal fluid and results of serologic studies were normal throughout the course of the disease, and attempts at viral isolation and antiviral antibody detection yielded negative results. Brain imaging either showed no abnormalities or suggested a moderate degree of brain edema. The recovery phase, which extended for several weeks, was characterized by a rapid return of motor function and persistent behavioral and cognitive disturbances. Nonverbal reasoning recovered long before verbal expression returned to normal. Four patients eventually recovered fully, whereas two had mild sequelae.

Acute Disease↗
PubMed1992

[Cerebral complications of incontinentia pigmenti. A clinicopathological study of a case].

A baby born with the typical features of incontinentia pigmenti, developed convulsions from the 5th day onward. CSF was normal. CT scan showed diffuse hypodensities in the left hemisphere. The baby died at 2 months of age of primary pulmonary hypertension. The neuropathological examination showed a normal morpho-histogenesis of the brain. There were necrotic, non inflammatory changes in the cortex and white matter, which did not affect a vascular topography. The lesions appeared to have occurred in the neonatal period and were markedly predominating in the left hemisphere.

Abnormalities, Multiple↗
PubMed1992

[Genetic counseling in neurology].

Recent advances in biological techniques have resulted in an ever increasing number of neurogenetic diseases being characterized at molecular level or mapped by polymorphic markers which provide the diagnosis. Thus, side by side with the management of the patients, a genetic management extended to the family is progressively taking shape. This familial care is not without technical and ethical problems.

Female↗
PubMed1991

Pelizaeus-Merzbacher disease: a valine to phenylalanine point mutation in a putative extracellular loop of myelin proteolipid.

In the central nervous system, myelin proteolipid protein isoforms (PLP and DM20) play an essential structural role in myelination. It has been shown in several species that myelination is impaired by molecular defects resulting from single base mutations in the PLP gene. We have used DNA amplification by polymerase chain reaction to study the PLP gene coding regions from 17 patients in 15 unrelated families with similar Pelizaeus-Merzbacher phenotype. In one case amplification of peripheral nerve PLP/DM20 cDNAs revealed that a silent T----C transition was unrelated to the disease. In one family a nonsilent mutation was identified that leads to a phenylalanine substitution for valine-218 in PLP/DM20 proteins. We investigated the inheritance of the mutant allele in 19 subjects of this four-generation family and found a strict cosegregation of the Phe218 substitution with transmission and expression of the disease. The effect of the Val218----Phe mutation is discussed in the frame of a recently suggested topological model of PLP/DM20, according to which Val218 is part of an extracellular loop that connects the last two of four membrane-spanning alpha-helices.

Animals↗
PubMed1990

Dominantly transmitted congenital indifference to pain.

Two patients from a family with dominantly inherited indifference to pain were investigated. Perception of the other sensory modalities was normal as was the remainder of the neurological examination. Electrophysiological studies and morphometric evaluation of myelinated and unmyelinated fibers of nerve biopsy specimens were normal. This is the first morphometric study of peripheral nerve in dominantly inherited indifference to pain.

Adult↗
PubMed1990

[Prognostic evaluation of severe head injuries in children].

In 34 children overcoming a severe head injury (coma greater than 1 d, mean: 10 d), the follow-up (mean: 25 m) has been correlated with several data. 1) The quality of life, according to a 3 grade-score, is mostly dependent upon the degree of neuropsychological sequelae. By decreasing frequency were noted disturbances of: memory, intelligence (the most significantly correlated with the quality of life), attention, rapidity of performances, behaviour, visuoconstructive activities. Most often several disturbances were associated, but without systematization. 2) Correlations between general outcome and early findings: a coma greater than 10 d, a subdural collection on the CT scan at 2-6 weeks have a pejorative meaning. Age does not appear to be relevant. 3) In a sequential study of neuropsychological functions, an early evaluation is poorly predictive, except in case of rapid normalization. Evaluation at 6 months gives a good picture of the outcome. Sectorial improvements can occur beyond the 1st year but do not appear to modify dramatically the general outcome.

Adolescent↗
PubMed1990

[Encephalitis with viral replication. Clinical aspects, prognosis and treatment].

Encephalites with viral replication are due to multiplication of a virus within the central nervous system. Diagnosis and initial therapeutic decisions rest on simple clinical and paraclinical findings: age of the child, presence of high-grade fever, presence and localization of seizures, CSF characteristics, and EEG findings. Acyclovir is always indicated in a drowsy child with a high fever who has even a brief seizure and CSF abnormalities.

Adolescent↗
PubMed1990

[Acute hydrocephalus due to Candida meningitis in a 2-month-old infant].

A case of triventricular acute hydrocephalus is reported in a 2 month-old male. The etiology was a Candida sepsis with neonatal onset and subacute course of meningitis and arthritis. No immune deficiency was detected and antibiotic treatment appeared to be the only predisposing factor to systemic candidiasis in this neonate. The condition was treated successfully with amphotericin B, fluocytosin and ketoconazole. At follow up, 17 months later, the development of the child appeared normal.

Acute Disease↗
PubMed1989

Vein of Galen aneurysmal malformations. Report of 36 cases managed between 1982 and 1988.

The authors report a series of 36 vein of Galen aneurysmal malformations (VGM) diagnosed in the paediatric (78%) and adult (22%) populations that were referred to them for therapeutic management between 1982 and 1988. The clinical signs leading to the diagnosis were variable: 36% of systemic manifestations, 22% of neurological symptoms, 17% of hydrocephaly and 11% of intracranial haemorrhage. 30 angioarchitectural analyses could be obtained and allowed to classify these VGMs into 5 different types: 44% parenchymatous AVMs, 20% mural AVFs, 30% choroidal arteriovenous fistulas, 3% dural AVFs, 7% vein of Galen varices. This series demonstrates that the paediatric population is most sensitive to shunt effect whatever its type. Systemic manifestations and hydrocephaly are the most common signs encountered in the newborn and infants; whereas neurological signs and symptoms and haemorrhage belong mostly to the adult symptomatology. Because of the poor outcome of VGMs, all authors believe that these malformations have to be treated aggressively. However, we found contra-indications to be represented by pretherapeutic demonstration of cerebral tissue damage, or uncontrollable systemic failure, thus treatment is indicated to compensate for cardiac failure previously responding (even partially) to medical treatment. Secondly, appearance of sub-cortical calcifications, resistance to medication or clinical deterioration will also lead to urgent treatment. The endovascular method represents at present the best treatment with an overall low mortality (13%) and a 0% technical morbidity in children compared to the surgical one of (91% mortality in newborns and 38% in infants). The results achieved by embolization in this series were as follows: 27% satisfactory results with complete or almost complete occlusion of AV Shunt, 53% significant clinical improvement, 7% of patients were unchanged. The authors believe fundamentally that these patients (specially those belonging to the paediatric population) have to be treated in a centre where a paediatric intensive care unit, neurological, neurosurgical and surgical neuro-angiographic departments coexist, in order to assure the best possible management of these children.

Adolescent↗
PubMed1989

Short- and long-term prognostic value of the electroencephalogram in children with severe head injury.

To determine the prognostic value of the EEG in severely head-injured children, 24 patients were studied for 8-36 months. During coma, 4 EEG patterns were found: borderline, sleep-like, changeable and slow monotonous (SM). For the short-term prognosis, we conclude that the SM pattern (12/24 patients) indicates a bad prognosis because it was associated with a longer coma and awakening period than that of other EEG patterns and because it was observed in the 3 patients who died from brain injury. In contrast, we describe a 'prewake' pattern (11/22 survivors) which, when it occurs, always announces the onset of a complete awakening. For the long-term prognosis, only 50% of the survivors who had an SM pattern during coma have as good an intellectual and motor outcome as the survivors who displayed other EEG patterns. No other EEG features recorded during coma have short- or long-term prognostic significance.

Child↗
PubMed1989

Treatment of vein of Galen aneurysmal malformation.

The authors report the case of a vein of Galen aneurysmal malformation in a 1-year-old baby presenting with an enlarging head. The lesion was a direct arteriovenous fistula in a dilated vein of Galen and was treated by complete embolization in one session. Four months after occlusion of the shunt, the ectatic vein of Galen and torcular were normal, and the head circumference had stabilized. This case serves as an opportunity to emphasize the quality of results that can be obtained with endovascular techniques. Proper analysis of the vein of Galen angioarchitecture allows planning for appropriate treatment with the lowest possible risk of morbidity and mortality.

Cerebral Angiography↗
PubMed1988

Tuberculous meningitis due to BCG in two previously healthy children.

Tuberculous meningitis with favourable outcome has been observed in two immunocompetent previously healthy children. The mycobacteria isolated from the cerebrospinal fluid of both patients proved to be Mycobacterium bovis BCG. The patients had been inoculated with BCG, one 5 and the other 6 months before onset of the disease.

Antibodies, Bacterial↗
PubMed1987

Paralytic poliomyelitis in vaccinated children.

Paralytic poliomyelitis were observed in two healthy children who both had previously received four doses of standard inactivated poliovaccine (IPV). These children, whose immune defenses were normal, failed to respond to IPV. This absence of antibody response might be related to an insufficient antigenicity of inactivated vaccines, justifying the extensive use of the more potent IPV, now produced on continuous cell lines.

Child, Preschool↗