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Biomedical subjects

J M Richardet

Publications and source records attributed to J M Richardet.

At least 19 recordsLinked to original sources
PubMed2002

[Mental retardation and environment: contribution of growth curves to etiological diagnosis].

Although deprived environment is known for more than 50 years to be one of the etiology of mental retardation in infancy, this remains unrecognized by many paediatricians and family practitioners. Yet if appropriate therapeutic measures are instituted early enough, future development of the child is good. A study of the growth curves in relation with the environmental changes, can help to diagnose environmental mental retardation. As an illustration, we present the cases of 5 children first addressed for developmental delay, and secondly removed from their usual environment for psychosocial reasons. Mean duration of follow-up was 10 years, including at least 5 years after the removal. All 5 children had non-organic failure to thrive which was displayed only on the retrospective study of their records. Four recovered from their developmental retardation.

Adolescent↗
PubMed1998

[Spasmus nutans: apropos of 16 cases].

UNLABELLED: Spasmus nutans is a syndrome occurring in early childhood. It consists of a triad of symptoms: head nodding, ocular oscillations and anomalous head position. Ophthalmologic and neurological findings are otherwise normal. This syndrome is benign and has spontaneous resolution. PATIENTS AND METHOD: Sixteen patients with spasmus nutans seen from 1980 to 1995 were retrospectively studied. Their present status was evaluated by clinical examination or questionnaire. RESULTS: The age at onset ranged from 1 to 15 months (average 7 months). Thirteen of 16 patients were referred for head nodding, which was a constant manifestation; its direction was horizontal, vertical or rotatory. Nystagmus was present in 14 infants. It was acquired, asymmetrical, bilateral (or unilateral in three cases), rapid, fine, pendular and horizontal. Both head nodding and nystagmus were intermittent. Anomalous head position was present in seven cases, consisting of head tilt or a chin upon/chin down posture. Neuroimaging (13 cases) was always normal. The follow-up in 12 children (up to 2 years) showed a complete resolution of the syndrome in 6 months to 6 years (average 2.5 years). DISCUSSION: The diagnosis was established by the constancy of the characteristic triad and the elimination of the other causes of nystagmus. Isolated head nodding had to be distinguished from bobble head syndrome. In several reported cases, electronystagmography recordings have suggested that head nodding is a compensatory process against nystagmus and that the head tilt allows transient resolution of the nystagmus. CONCLUSION: Spasmus nutans is a self-limiting benign clinical entity. Normal complete ophthalmologic and neurological examination, as well as magnetic resonance imaging (MRI) are necessary to confirm the diagnosis.

Age of Onset↗
PubMed1993

[Dengue. Apropos of 2 cases].

BACKGROUND. Dengue is an acute febrile illness caused by several arthropod-born viruses and characterized by biphasic fever, myalgia or arthralgia, rash, leukopenia and lymphadenopathy. Its diagnosis is based on knowledge of the geographic distribution of dengue viruses. CASE REPORTS Case no 1: A 11 year-old boy suffered from sudden onset of fever accompanied by retro-orbital headache, arthralgia and diffuse myalgia. There was no rash. Hemogram showed: hemoglobin: 11.6 g%; leukocytes: 3,400/mm3 (PMN: 76%); platelets: 190,000/mm3. A diagnosis of viral infection was considered, but, as the boy had recently been to the French West-Indies, a serologic study was performed. This was negative 2 days after the onset of disease and positive (specific IgM for the 4 dengue types), 13 days later. Case no 2: A 7 year-old boy suffered from sudden onset of fever. Severe calf muscle pain 4 days later led to his admission. Creatine phosphokinase activity was very high: 83,100 units (N: 30-120). Hemogram showed: hemoglobin: 11.4 g%; leukocytes: 2,500/mm3 (PMN: 60%); platelets: 124,000/mm3. A diagnosis of acute myositis was considered, but as the patient had recently visited Venezuela, a serologic study was performed. This was negative 8 days after the onset of disease and positive (specific IgM for the 4 dengue types) 16 days later. CONCLUSION. The first case is characteristic of the classical form of dengue fever. The second patient presented with very localized myalgia. The diagnosis in both cases was facilitated by the knowledge that the patient had recently stayed in an endemic area.

Acute Disease↗
PubMed1992

[Creutzfeldt-Jakob disease in 4 children treated with growth hormone].

Creutzfeldt-Jakob disease was diagnosed in four growth hormone recipients at the age of 10, 11, 18 and 19 years. To our knowledge, the two first cases are the first instances of Creutzfeldt-Jakob disease recorded in children. Three of them were still being treated with synthetic hormone at the onset of the disease. Neurological disorders: ataxia and diplopia, appeared first, dementia and myoclonus appeared later. Eighteen cases of Creutzfeldt-Jakob disease in growth hormone recipients are now recorded, and the present risk of Creutzfeldt-Jakob disease in pituitary growth recipients is estimated to be 1/300. Because of the long incubation period, new cases are to be feared. Other causes of iatrogenic Creutzfeldt-Jakob disease are reviewed. These facts incite to consider carefully using products of human origin in human therapy. The interactions between growth hormone, prion and host's genomic make-up are still not clear.

Adolescent↗
PubMed1991

[Management of abused children: at what cost? what for?].

The prospective follow up of abused children after their hospitalization is illustrated by the report of a sibship. Dysfunction in management and therapy is often observed and is detrimental to the children. Many levels of our society are implicated. This means that there is still a lot to do to obtain adequate therapy for these children.

Child↗
PubMed1989

[Acute isolated myelitis due to measles].

We report a case of acute myelitis that developed in a seven-year-old girl with measles. Attention is drawn to the isolated occurrence of this complication in our patient who had no other neurologic abnormalities, and specifically no encephalitis, whereas spinal cord involvement in measles is usually overshadowed by prominent manifestations of encephalitis. Furthermore, we point out that intrathecal production of antibodies may occur but does not militate against the immunoallergic mechanism usually thought to be involved in this type of disease.

Child↗
PubMed1988

[Progressive bulbopontine paralysis in children].

A case of pons-bulbar palsy in a 10 year-old girl is reported. The authors insist on the fact that motor cranial nerve nuclear dysfunction was isolated, without any pyramidal tract disorder. They also emphasize the slow course of the disease, which after one year of quick progression was stable for the next 5 years. Nineteen identical cases were found in the literature. From this nosographic group, one must exclude patients with associated lateral spinal tract dysfunction: these patients must be discussed with the juvenile or infantile amyotrophic lateral sclerosis group. The heterogeneity of the pons-bulbar paralysis group is also underlined: the course may be quickly fatal or, on the contrary, protracted for a long time; genetic transmission is also quite variable.

Bulbar Palsy, Progressive↗
PubMed1985

[Bobble-head doll syndrome].

A new case of Bobble-head doll syndrome with aqueductal stenosis is presented in a 14 year-old boy. Ventriculocisternostomy performed 8 years after the onset of the abnormal movement resulted in moderate reduction of the head bobbling. Twenty-two cases were found in a review of the literature. In all cases there was a chronic slowly progressive hydrocephalus with usually a cyst of the third ventricle; aqueductal stenosis was less frequent. When recorded, psychomotor development was impaired. Treatment is neurosurgical. Pathogenesis remains unknown.

Adolescent↗
PubMed1984

[Sarcoidosis in a child disclosed by blindness with major hypercalcemia].

We report here a case of sarcoidosis, observed in a 12 year-old algerian girl. When the child was examined, at an advanced stage of the disease, her clinical condition was very critical, associating blindness, cachexia, hepatic and splenic enlargement, and major hypercalcaemia. Though chest roentgenograms were normal, the results of both functional pulmonary tests and broncho-alveolar lavage were pathologic. The diagnosis of sarcoidosis was confirmed by the finding of an elevated level of Angiotensin-converting enzyme, and by histologic lesions observed in liver, kidney and conjunctiva biopsies. The corticosteroid treatment improved the general condition, but it failed to better the visual state.

Blindness↗
PubMed1983

[Distal 1q monosomy. 2 new cases and description of the syndrome].

In two unrelated girls, each with severe mental deficiency and craniofacial dysmorphism, deletion of chromosome segment 1q4 had occurred de novo. These two observations together with seven others from the literature allow delineation of a syndrome, as follows. Growth retardation is marked at birth and remains beyond the 3rd percentile. Mental retardation is severe. Seizures are frequent. The cry may be unusually high pitched. Craniofacial dysmorphism includes brachymicrocephaly, a round and flat facies, a high forehead with a prominent metopic suture, thin and scarse hair, palpebral fissures slanted upward, hypertelorism and epicanthal folds, a bulbous nose with a flattened nose-bridge, a large and prominent philtrum, well-defined Cupid's bow, and retrognathia. Other malformations include various skeletal malformations, hypospadias with or without cryptorchidism, a consistent feature, and congenital heart disease in a third of the cases. The deletion occurred de novo in 8 out of the 9 cases. The breakpoint is, according to the several authors, in 1q42 or q43.

Adolescent↗
PubMed1982

Relationship between sleep stage IV deficit and reversible HGH deficiency in psychosocial dwarfism.

Polygraphic sleep recordings were done in four children with psychosocial dwarfism. The first recordings, performed within the first days after hospitalization, exhibited a gross deficit of stage IV sleep and a decrease of the durations of slow wave sleep episodes (SWS = stage III and IV). The second recordings were performed after several wk (3-15) in the new environment, during the growth recovery period. A clear improvement of sleep quality was observed, namely an increase of stage IV amounts. In two of the four patients, hGH release was studied by means of the ornithine test. A low response was found in the first (2) days of hospitalization whereas normal response was observed during the recovery period suggesting a partial and reversible hGH deficiency. A simultaneous recovery of both stage IV sleep and the growth rate was observed in the four patients. These data suggest that sleep disturbances are strongly linked to growth failure in psychosocial dwarfism.

Child, Preschool↗