Search PubMed⌕ Search

Biomedical subjects

A M Laverda

Publications and source records attributed to A M Laverda.

At least 37 records · Page 2Linked to original sources

Hypomelanosis of Ito and hemimegalencephaly.

Hypomelanosis of Ito is a congenital neurocutaneous syndrome with a particular pattern of swirling hypopigmentation. Multiple extracutaneous abnormalities involving the central nervous system, the eyes, and musculoskeletal structures occur in over two-thirds of the cases. This report describes two patients with typical unilateral cutaneous lesions associated with extracutaneous features, including hypertrophy of the cerebral hemisphere contralateral to the cutaneous hypopigmentation. Magnetic resonance imaging and EEG findings support the diagnosis of hemimegalencephaly, as has recently been reported in other isolated cases of this rare phakomatosis.

Brain↗

Neurogenic vesico-urethral dysfunction in children with cerebral palsy.

20 children with severe cerebral palsy and history of urinary incontinence and recurrent urinary infection underwent radio--and neuro-urologic evaluation. Vesico-ureteral reflux was found in 7 patients. In 9, who had presented episodes of urine sub-retention, a urodynamic study demonstrated detrusor muscle hyperreflexia in all, deficit of vesicourethral sensation in 5 and detrusor-sphincter dyssynergia in 2. These functional findings are compatible with an upper motor neuron lesion, and may be the cause of episodes of altered bladder emptying, and consequently, urinary infection.

Adolescent↗

Impairment of polymorphonuclear leucocyte function during therapy with synthetic ACTH in children affected by epileptic encephalopathies.

Therapy with synthetic ACTH (zinc tetracosactide) in children affected by epileptic encephalopathy is often associated with a large number of infectious complications. We studied the phagocytic activity of polymorphonuclear leucocytes (PMN) in 9 children with West or Lennox-Gastaut syndrome, measuring PMN superoxide anion production during the phagocytosis of particles of Zymosan and after phorbol myristate acetate (PMA) stimulation. The test was performed before, during and after therapy with zinc tetracosactide (0.02 mg/kg/day for 15 days). At the same time plasma immunoglobulins, C3, C4, C3 activator and cortisol were determined. During treatment PMN phagocytic function was significantly reduced but returned to normal levels after suspension of therapy. The other hematological parameters considered remained within the normal range. During the follow-up of the patients we observed 15 infectious episodes (3 mucocutaneous candidiasis, 2 enterocolitis, 4 urinary tract infections, 1 otitis media, 3 bronchiolitis, 2 pneumonia). One of the patients died of a bilateral pneumonia. Three children were treated with ACTH on alternating days. In these patients PMN phagocytic activity was less impaired and 2 infectious episodes rapidly resolved. Alternate day ACTH therapy seems to be preferable.

Adrenocorticotropic Hormone↗

Risk factors in long-term sequelae of central nervous system prophylaxis in successfully treated children with acute lymphocytic leukemia.

Seventy-two successfully treated patients with acute lymphocytic leukemia, all in first complete remission and all off therapy, who had received CNS prophylaxis (radiotherapy, 2,400 rad, plus intrathecal methotrexate), were studied by computed tomography (CT) of the brain, EEGs, and neurologic evaluations 3 to 9 years after the end of prophylaxis. Thirty-five patients showed CT brain scan abnormalities: intracranial calcifications (twelve); widening of the subarachnoid spaces (eight); isolated dilatation of ventricular spaces (three) and with frontal periventricular hypodensity (two); dilatation of ventricular and subarachnoid spaces (nine); and a hypodense area (one). Only 17 patients showed aspecific EEG abnormalities which were never linked to CT scan findings. None of our patients presented major motor deficits at the neurologic examination. A stepwise logistic regression technique showed that age less than 5 years at the time of prophylaxis was the most important risk factor (p = 0.008) of CT brain scan abnormalities followed by neurets (p = 0.037) and sex (p = 0.10). Furthermore, the multivariate analysis pointed out that the interactions between these variables were not significant and the effects were only of the first order.

Acute Disease↗

Acute-onset transient hydrocephalus after suspension of ACTH therapy for infantile spasms: a case report.

A baby with infantile spasms (West's syndrome) who developed acute-onset transient hydrocephalus 10 days after suspending ACTH treatment is described. Hydrocephalus is an unusual complication of ACTH therapy, the more common complication being benign intracranial hypertension. The probable common pathogenic mechanism of altered CSF reabsorption which may lead to the two different clinical states, depending on age of patient, is discussed.

Acute Disease↗

[Postasphyxia cerebral changes in the newborn infant. The significance of tomographic hypodensity].

The CT findings in 37 asphyctic newborns are presented, particularly concerning the hypodense lesions. 11 out of 27 term infants and 4 out of 10 preterm neonates had 2 or 3 serial scans, the last when they were 2-5 months old. In most of cases a long run normalization of former periventricular hypodensity has been observed, with normal clinical follow-up, 6 cases (2 preterm and 4 term neonates) had cortical hypodensity too: 2 of these revealed afterwards atrophy and hydrocephalus, 2 ventricular asymmetry and slight cortical spaces enlargement; in 1 case the cortical parietal hypodensity, formerly showed, was confirmed; 1 case finally was normal at three month scan. The relevance of the cortical hypodensity in most serious brain damage is underlined; the hypodensity of the white matter, on the contrary, could mean wether a postasphyctic lighter, reversible brain damage, or the physiologic postnatal condition, such a still unfilled myelinization.

Asphyxia Neonatorum↗

Neonatal tuberous sclerosis presenting with intractable seizures.

A 2-day old girl with status epilepticus, unresponsive to maximum pharmacological intervention, is reported. Findings of brain and cardiac lesions pointed to the diagnosis of tuberous sclerosis. One of the brain lesions was unusually large, occupying most of the right temporo-parietal lobe.

Anticonvulsants↗

Intracranial hypertension and cryptococcal meningitis in a girl with AIDS.

A girl with HIV infection acquired at birth by blood transfusion, was admitted at the age of 10 years for diplopia, vomiting, headache and papilledema. CT scan was negative. A lumbar puncture revealed clear CSF, protein 0.40 g/l, glucose 2 mmol/l, 5 mononuclear cells/mm3. The Indian ink preparation and the latex agglutination antigen test were positive for Cryptococcus n. Treatment with amphotericin B and flucytosine was started. After 10 days, since the in vitro susceptibility testing of the isolates showed resistence to both drugs, fluconazolo (400 mg/day) was started. Acetazolamide, furosemide and spironolactone were then added to the antifungal therapy for the persistence of severe intracranial hypertension. Diuretics were maintained for 10 weeks. The patient returned to school two and half months after the admission to the hospital. After 19 months, she is doing well and she is on maintenance of fluconazole (200 mg/day). We hypothesized that the increased intracranial pressure would be due to an impaired CSF reabsorption probably as a consequence of a direct cryptococcal infiltration of the villi.

Acquired Immunodeficiency Syndrome↗

Macrocephaly and chromosome disorders: a case report.

We report the case of a young patient with macrocephaly. After excluding the most frequent causes of macrocephaly (hereditary disorders, degenerative, osseous and metabolic diseases, neurocutaneous syndromes and cerebral malformations), the likelihood of a chromosome disorder was investigated, revealing an unbalanced de novo translocation: 46,X,der(X),t(X;7) (q13 or q13.2; q11.23 or q21.11), i.e., a partial trisomy of the long arm of chromosome 7, associated with a partial monosomy of the long arm of chromosome X. Though this chromosome disorder is relatively rare, it should be considered in the differential diagnosis of patients under one year of age presenting with macrocephaly, scoliosis and non-progressive psychomotor retardation.

Brain↗

Immunological markers in the cerebrospinal fluid of HIV-1-infected children.

Several immunological abnormalities were detected in the cerebrospinal fluid (CSF) of human immunodeficiency virus type 1 (HIV-1)-infected children. Intrathecal synthesis of immunoglobulins, free light chains (FLC), IL-1 beta, IL-6, and M-CSF were demonstrated both in asymptomatic children and children with subacute encephalopathy. Our findings further support the hypothesis that an immunopathological subclinical process within the central nervous system (CNS) may be an early manifestation of acquired immunodeficiency syndrome (AIDS). Cytokine detection in the CSF may represent a useful diagnostic tool in evaluating the outcome of HIV-1-infected patients.

AIDS Dementia Complex↗

CT and ACTH treatment in infantile spasms.

Computed tomography of 8 cases with West's syndrome before, during and after ACTH treatment are reported. The scans, performed at the third week of therapy, showed consistent widening of the sulci, cisterns and ventricles in all the patients. Of these, 2 patients underwent ICP monitoring which showed higher than normal values. A return to the normal ICP values in association with the disappearance of the CT findings was observed in both cases. It is concluded that widening of the sulci, cisterns and ventricles are not findings of atrophy, but a condition of initial communicating hydrocephalus, which is in accordance with the hypotheses of Riikonen and Lyen.

Adrenocorticotropic Hormone↗