[Pharmacist's interview with type 2 diabetes: Development of a follow-up form]. / L'entretien pharmaceutique pour le patient diabétique de type 2 : élaboration d'une fiche de suivi.

OBJECTIVES: Type 2 diabetes is a major public health concern because of its prevalence, the severity of complications and the financial implications. Compliance and patient's autonomy in medications intake play key roles in the success of treatment. Pharmacists' interviews ensure an optimized and individual follow-up. Type 2 diabetes is not one of the targeted diseases to perform pharmacists' interviews on under Health Insurance. We thus judged useful to contribute to their development. METHODS: We applied a cross-disciplinary methodological process in order to define the specifications of the follow-up form useful to conduct the pharmacist's interview 1 by focusing on the identification of a non-compliance and its origins. A feasibility study was carried out in order to check its workability to the pharmacy practice. RESULTS: The follow-up form, associated with a pharmacist practical guide, includes 3 parts: (1) General informations, (2) Survey establishing patient's knowledge, (3) Summary including a level of knowledge assessment grid. Outcomes provide a long but appropriate-felt duration, few difficulties to conduct the interview and a proven usefulness in 90% of all cases that make the follow-up form suitable to the pharmacy practice. CONCLUSIONS: This tool could serve as a model for the pharmacist to conduct his future interviews for the type 2 diabetes patients, thus improving patient care, together with other health professionals.

Cryptic 6q subtelomeric deletion associated with a paracentric inversion in a mildly retarded child.

We report on a girl with minor anomalies and developmental delay carrying an apparently balanced paracentric inversion of chromosome 6q (q22qter). Fluorescent in situ hybridization analysis demonstrated a deletion of the subtelomeric region of 6q. This illustrates the use of specific subtelomeric fluorescent in situ hybridization probes to detect cryptic deletions as an important cause of mental retardation in seemingly balanced chromosome rearrangements.

Incontinentia pigmenti: MR demonstration of brain changes.

PURPOSE: To describe the MR findings in eight girls and women with incontinentia pigmenti, from two families. Four had skin lesions and neurologic disease, and four had only skin lesions. METHODS: Eight patients had physical examination, family history, electroencephalogram and MR examination of the brain. MR was repeated in the two cases with more severe changes several years after the first study. RESULTS: MR revealed brain changes only in the four patients who had neurologic disease associated with the cutaneous lesions of incontinentia pigmenti. Abnormalities were located in the cerebral hemisphere contralateral to the most affected side of the body. In two cases, the MR changes were subjacent to the scalp areas where the most severe cutaneous lesions were located in the neonatal period. Hypoplasia of the corpus callosum, probably secondary to atrophy of one or both cerebral hemispheres, and abnormal signal and atrophy of the lateral regions of one of the cerebellar hemispheres also were found in all four cases. Although the changes were seen in both the T1- and T2-weighted images, they were most evident in the latter. The four patients in the fourth stage who had only cutaneous lesions without neurologic problems did not reveal any MR abnormalities. CONCLUSIONS: This study demonstrates MR signal changes and focal atrophy of the cerebrum, cerebellum, and corpus callosum in patients with incontinentia pigmenti and neurologic disorders. The MR images appear normal in patients with incontinentia pigmenti who have no neurologic abnormalities.

Hemangiomas of the head, neck, and chest with associated vascular and brain anomalies: a complex neurocutaneous syndrome.

PURPOSE: To describe the vascular and nonvascular intracranial and extracranial anomalies associated with hemangiomas and vascular malformations of the face, neck, and/or chest. METHODS: Seventeen patients had a physical examination and imaging studies consisting of one or more of the following: pneumoencephalography, conventional carotid and vertebral arteriography, CT, MR imaging, and MR angiography. RESULTS: Conventional arteriography revealed persistence of the trigeminal artery in 5 cases, absence of internal or external carotid and/or vertebral arteries in 11 cases, persistence of intervertebral arteries in 1 case, deformities of the aortic arch in 3 cases, and anomalies of the intracranial arteries in 3 cases. MR angiography revealed persistence of the trigeminal artery in 1 case in which conventional arteriography failed to show the malformation, and permitted visualization of narrowing of the intracranial arteries. CT and MR imaging showed a cerebellar anomaly in 8 cases and cerebral cortical dysplasia with cerebral hemispheric hypoplasia in 1 case. Vascular and nonvascular anomalies appeared ipsilateral to the external vascular abnormalities in most cases. CONCLUSION: This study demonstrates the association of cutaneous angiomas with anomalies affecting intracranial and extracranial arteries, the cerebellum, and, less frequently, the cerebral hemispheres and aortic arch. This association constitutes a relatively frequent neurocutaneous disorder, which we call the cutaneous hemangioma-vascular complex syndrome.

Cerebrospinal fluid lactate levels in term infants with perinatal hypoxia.

The prognostic value of cerebrospinal fluid (CSF) lactate concentration after an hypoxic-ischemic brain insult was evaluated. Three groups of neonates were studied and followed. Group 1A included 19 asphyxiated neonates who subsequently developed normally, Group 1B included 6 asphyxiated neonates who died or developed neurologic sequelae, and Group 2 consisted of 20 control (comparison) infants. A CSF sample was obtained within the first day of life for cytochemical analysis, culture, and lactate determination with simultaneous determination of serum lactate. The mean CSF lactate level in Group 1B (4.5 mmol/L) was significantly higher than in Group 1A (2.5 mmol/L) (p less than .001). In comparison to the highest CSF lactate concentrations in the normal range in Group 2 (2.7 mmol/L), all Group 1B neonates had elevated CSF lactate levels, and eight neonates (34%) of Group 1A had increased levels. A correlation was found between blood and CSF lactate levels in asphyxiated infants (r = .42, p less than .05), but its meaning is uncertain. We conclude that CSF lactate determinations may be helpful in the evaluation of perinatal hypoxic-ischemic encephalopathy.

[The course of psychomotor development in different encephalic malformations]. / Evolución del desarrollo psicomotor en los diferentes procesos malformativos encefálicos.

INTRODUCTION: Congenital malformations of the brain often are complex disorders which display a wide spectrum of neurological manifestations. The presence of this diversity makes it difficult to correlate clinical findings with neuropathological abnormalities or through neuroimaging studies. DEVELOPMENT: Furthermore, the complexity of the etiological problem creates difficulty to differentiate those disorders having a primary cause, usually implying a genetic origin, from those secondary to a intrauterine insult occurring during the developmental brain process. In this study we have made a approach to mental and motor development in patients with typical or isolated brain malformations. To provide focus, we purposely excluded patients with complex anomalies, neurological disease, chromosomal disorder, syndromic picture or those who had brain malformations secondary to a known insult. CONCLUSIONS: Our exposition come from our review of the current related literature and our own clinical experience, and we have chosen to classify the brain malformations into these three groups: those occurring before the 20th week of pregnancy, those occurring after the 20th week of pregnancy, and the malformations of the posterior fossa [REV NEUROL 1999; 28: 130-5].

[Botulinum toxin as a treatment for infantile cerebral palsy]. / La toxina botulínica como tratamiento de la parálisis cerebral infantil.

OBJECTIVE: To review the results and adverse effects to botulinum toxin type A (BTA), Botox, in cerebral palsy (CP) spastic and/or dystonic in an open prospective study. MATERIAL AND METHODS: The first 39 cases treated were analyzed. They received 1-2 doses and were followed up to 12 months. BTA indications were wide: to improve limb function, to avoid surgical orthopedics or improve hygienics or dressing. O'Brien Global Assessment Scale (scored by neurologist, physiotherapist or parents), Ashworth spasticity scale, functional scale for dystonic upper limb (Sindou-Millet) and exam of position of foot, knee and hip, were used. RESULTS: Total doses/session was 1-10 U/kg. We observed adverse effects in 6 cases (15.4%), always mild and lasting only few days (general weakness, tiredness, instability). Positive effects lasted 4 months in upper limbs and 4.5 months in lower limbs. In upper limbs (9 cases injected) it was observed a global positive result of mild grade in 11-40%, moderate without functional improvement in 11-22%, and moderate-important with functional improvement in 40-78% of patients, being patient's evaluation the best and physiotherapist's one the worst. Spasticity improved 2 or more grades in Ashworth scale in 7/9 cases. Dystonia improved in proportion to dose. In lower limbs gastrocnemius muscles were injected in 29 cases (55 sessions), adductors in 14 cases (33 sessions), ischiotibialis in 8 cases (27 sessions), posterior tibialis in 8 cases (12 sessions). It was observed a global improvement null or mild in 20%, moderate without functional change in 35-44%, and moderate or important with functional improvement in 35-44%, with significative correlation between parent's, physiotherapist's and neurologist's scores. Spasticity was also significatively reduced after treatment. It went down 2 or more grades in Ashworth scale in 40% of ischiotibialis, 60% of adductors and 65% of gastrocnemius, in general with a doses-effect association. Foot position in walking improved from moderate to important grade in 2/3 of cases, as improved foot position while standing. Knee flexion and hip hyperadduction were reduced moderate-importantly in 60% and 40% of cases respectively. CONCLUSION: BTA is highly effective in the treatment of CP, and if associate with physiotherapy long and even permanent effect can be achieved.

[Neuronal migration disorders: clinical-radiological correlation]. / Trastornos de la migración neuronal: correlación clínico-radiológica.

We present the correlation of imaging findings with clinical deficits in 34 children with several anomalies of neuronal migration which were studied retrospectively. The cases studied corresponded to: Schizencephaly 10, lissencephaly 4, heterotopy 9, and hemimegalencephaly 2. The diagnosis of the malformations was performed by computerized tomography (CT) in a few patients and by magnetic resonance (MR) in most cases. Among the clinical alterations, psychomotor delay and seizures of difficult control were the most severe, both appeared at early age. The findings observed in this study suggest the correlations between extension, location and gray matter displasticity and the neurological manifestations.

[Bilateral hypodensity of the basal ganglia. Clinico-evolutionary correlation in children]. / Hipodensidad bilateral de ganglios basales en neuroimagen. Correlato clinicoevolutivo en la edad pediátrica.

INTRODUCTION: The presence in neuroimaging of areas of symmetrical bilateral hypodensity in the basal ganglia (SBHBG) is a striking and unusual finding. OBJECTIVE: To determine the aetiology, clinical significance and evolution of a group of paediatric patients with SBHBG. PATIENTS AND METHODS: We made a study of 21 patients with neuroimaging studies (CT or MR) showing SBHBG. The affected area was related to the aetiology, clinical features and evolution. RESULTS: The ages varied between 4 months and 16 years. In 7 cases Leigh s disease was diagnosed, 5 had had acute hypoxia, 4 type I glutaric aciduria, and 1 case each of methylmalonic aciduria, Ia gluconeogenesis, CO intoxication, acute striatal necrosis and bacterial meningitis. The putamen was affected in 6 cases, globus pallidus in 4 cases and the lenticular nucleus was damaged in the rest. Three cases also had lesions in the caudate nucleus. MR was better than CT for localization of the precise area involved. Clinically, 13 cases had extrapyramidal signs. We found no relation between the size, localization of the lesion and the prognosis, which was more dependent on the aetiology, only one patient (CO intoxication) recovered and eight died (Leigh s disease and 1 case of hypoxia). CONCLUSIONS: The presence of SBHBG in a patient makes extensive study necessary to find the aetiology. It is a nonspecific finding, usually of metabolic origin and with little correlation with the clinical condition. Its presence implies a poor prognosis and raises suspicion of the presence of certain neurological disorders.

[Hepatic distomatosis caused by Clonorchis/Opisthorchis spp. in refugees from South-East Asia. Effects of treatment with praziquantel]. / Etude des distomatoses hépatiques à Clonorchis/Opistorchis spp. chez des réfugiés d'Asie du Sud-Est. Effets du traitement par le praziquantel.

The prevalence of liver infection (percentage of patients voiding Clonorchis or Opistorchis spp. eggs in faeces) is 18.5% in the South East Asian refugees. Both sexes are equally affected and the older the refugees are, the more often they are found infected. Laotians, who like to eat the fishes raw, are harboring leverflukes more often than other Far-east inhabitants. Praziquantel, prescribed at a dose of 25 mg/kg body weight given 3 times on a single day, is very efficient in the treatment of the Far-east hepatic distomatosis. Tolerability to praziquantel is excellent. Side effects are few and of moderate intensity.

Measurement of hepatic blood flow in the unanesthetized rabbit using 198Au and 125I Rose Bengal clearance technique.

Hepatic blood flow was measured in the unanesthetized rabbit using the clearance technique of 198Au and 125I RB. The values are: 71.82 +/- 16.24 ml-min-1-kg-1 for 198Au, and 60.21+/-9.94 ml-min-1-kg-1 for 125I RB (P less than 0.01). The overestimation of HBF with colloidal gold is probably due to extra splanchnic sequestration which has been estimated to be 15+/-3%. The week extraction of RB limits the use of this dye for measurements of HBF. HBF is easier to measure with 198 Au.

Primary intracranial arachnoidal cysts. A study of 67 childhood cases.

Sixty-seven cases (41 males and 26 females) of arachnoidal cysts in children under 11 years are reported. About 53% of cases were diagnosed before 1 year of life. Thirty-one (42.2%) were supratentorial (interhemispheric 9, temporal fossa 10, convexity 5, sylvian fissure 3, supra- and/or retrosellar 4); 31 (46.2%) infratentorial (supra- and/or retrocerebellar 22, foramen of Magendie 3, quadrigeminal cistern 5, pontocerebellar 1); 5 (7.5%) supra- and infratentorial. Macrocephaly was the presenting symptom in 48 cases (71.5%). Associated features were frequent: cranial asymmetry in 24; aqueductal stenosis in 10; agenesis of corpus callosum in 8; deficient cerebellar lobullation in 4; Chiari I malformation in 2; neurofibromatosis type 1 with dysgenetic zones of the brain in 1; arteriovenous malformation in 1. Diagnosis was made at autopsy in six cases in the days before computed tomography and magnetic resonance: three patients had a cyst in the supra- and retrocerebellar midline; two had a cyst in the quadrigeminal cistern and the sixth was a rare case with the cyst passing from the posterior fossa to the left lateral ventricle through a hole in the basal surface of the brain. Small and some middle-sized cysts were not treated. Big and some middle-sized cysts were usually treated by cysto- and/or ventriculoperitoneal shunts. Arachnoidal cysts of the quadrigeminal cistern usually present with aqueductal stenosis and have to be treated with ventriculoperitoneal shunt. Craniotomy and fenestration of the cysts were performed in some cases with good results. The average mental level of these children is usually moderately low.

Dandy-Walker malformation: analysis of 38 cases.

Thirty-eight cases of Dandy-Walker malformation (DWM) are presented. A female predominance of 3:1 was found. Thirty-two cases (84%) were diagnosed within the 1st year of life. Of these, 17 cases (44.7%) were diagnosed at birth. Ten (26%) were delivered by cesarean section. Thirteen infants (34%) had a birth weight below 3000 g. Several associated malformations were observed, the most frequent being capillary angioma (6 cases); cardiac malformations, ophthalmic anomalies, agenesis of the corpus callosum, malformed limbs, and occipital meningocele were also seen. These observations indicate that DWM represents a disorder of the midline central nervous system indicative of marked genetic and etiologic heterogeneity with the possibility of showing clinical and pathological alterations intra- and extracranially. Macrocephaly was the most frequent physical finding, appearing in 31 cases (82%). Seventeen (44.7%) patients died, 11 before 6 months of age, 3 between 6 and 12 months, and 3 after 1 year. Postmortem studies were performed in 13 patients. Three cases have been lost to follow-up. Mental retardation (IQ below 70) was found in 11 cases (58% of survivors), low intellect (IQ between 70 and 85) in 4, and only 2 patients showed normal intellectual development (IQ more than 85). The high incidence of malformations having several genetic and environmental origins, as well as the high early mortality of patients with DWM, indicate the complexity of this syndrome, which involves the midline developmental field structures. It is not an isolated malformation of the posterior fossa in most cases.

LDH isoenzymes in CSF in the diagnosis of neonatal brain damage.

Lactate dehydrogenase isoenzymes in cerebrospinal fluid were measured during the first 24 h of life in 25 asphyctic neonates. The infants were subjected to clinical and developmental examinations for a mean follow-up period of 15.2 months. The mean values for cerebrospinal LDH2(MH3) and LDH3(M2H2) isoenzymes were significantly higher in asphyctic infants who died from hypoxic-ischemic encephalopathy or who survived but suffered neurologic sequelae, than they were in those asphyctic infants who survived and were normal in the follow-up studies. Therefore, the authors conclude that the lactate dehydrogenase isoenzyme pattern in cerebrospinal fluid may be of value when assessing anoxic brain damage.

[Endocarditis due to Hemophilus para-influenzae associated with mitral valve prolapses (author's transl)]. / Endocardite à Hemophilus para-influenzae sur prolapsus de la valve mitrale.

The authors report one observation of endocarditis due to Hemophilus para-influenzae associated with mitral valve prolapsus. This germ is difficult to isolate and is found late in cultures using standard techniques. The dicovery of germs resistant to ampicillin requires the search for the presence of a betalactamase. When resistant germs are found, the antibiotic of choice is chloramphenicol or cefamandole. This type of endocarditis different from the others on account of the risk major of embolism (60-85 p. 100) which justifies valve replacement when the echography reveals persistant vegetations.

Bilateral obstruction of the vertebral arteries in a three-year-old child.

A three-year-old boy developed gait instability, nausea, vomiting, cranial nerve disturbances, hypotonus and dysarthria. Angiography of the four main cranial arteries showed complete obstruction of both vertebral arteries at the level of C1 to C2. Abundant collateral circulation was observed, which by-passed the obstruction to the vertebral arteries before their enterance into the posterior cranial fossa. The left vertebral artery was hypoplastic and both internal carotid arteries showed coiling in their extracranial portions. A high erythrocyte sedimentation rate at the beginning of the disease suggests an inflammatory alteration of both dysplastic vertebral arteries. The child recovered completely one month after the onset of symptoms. All other reported cases of childhood vertebro-basilar obstruction are reviewed and it is emphasized that the site of arterial obstruction has an important bearing on the clinical outcome.

Choroid plexus papillomas of the fourth ventricle. Report of 3 cases.

3 cases with benign choroid plexus papillomas (CPP) in the fourth ventricle are described. All patients were less than 2 months of age. They were among 14 cases with intracranial CPP in a series of 100 tumors of the posterior fossa. Correct diagnosis was not obtained by means of pneumoencephalography, with apparently normal repletion of the fourth ventricle being visualized. 2 of the 3 patients died after application of the ventriculoperitoneal shunt and before extirpation of the tumor. All 3 patients soon presented hydrocephalus in 1 case already 'intra utero'.

Occipital osteodiastasis. A rare complication in cephalic delivery.

A case of occipital bone osteodiastasis in an infant born by cephalic vaginal delivery is presented. Cerebral echography and CT scan did not show posterior fossa haemorrhage. Neurological assessment at three years of age was completely normal.

[Vertebral and basilar arterial oclusions in children. A case report and review of literature (author's transl)]. / Oclusiones del territorio vértebro-basilar en la infancia. Presentación de un caso y revisión de la literatura.

A nine-year-old boy with thrombosis in the right vertebral artery is presented. When child was 20 months old symptoms began suddenly with loss of consciousness, hemiparesia in the left side and later on psychomotor involution. The various aspects of the vertebral and basilar occlusions are discussed when they happen before the age of fourteen, of which there are 19 other cases published already. Etiology of this affection is very mixed in children and frequently unknown. There are more than one obstruction in 50% of patients and very often the nearest one is located at the level of the axis. Evolution is better in children than in adults, being dependant on such factors as the type of initial symptom, location of occlusion and quantity of collateral circulation present.