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Biomedical subjects

J Grippo

Publications and source records attributed to J Grippo.

28 records · Page 2Linked to original sources

[Acute encephalitis: bilateral lesions of the basal ganglia].

OBJECTIVE: To refer two children with acute encephalitis and bilateral basal ganglia lesion and its neurological outcome. CLINICAL CASES: Two girls, one of 9 years and the other of 15 months of age were affected by acute encephalitis, bilateral basal ganglia lesion was found on MRI in both children. Abnormal movements (tremor, choreoatetosis) and dystonia were the main symptomatology. In the first girl a mild dystonic posture on her hand and minor bradykinesia was the only found after a 4 year follow up. The other child, after 17 months of the beginning of her disease, still has generalized dystonia and choreoatetosis movements. Control MRI studies, in both patients remain without changes. CONCLUSION: In acute encephalitis, basal ganglia lesion in two children, produced different neurological sequelae, probably related to the age of the presentation of the disease.

Acute Disease↗

[Channelopathies in neurology].

INTRODUCTION: The main function of ionic channels are the conduction, recognition and selection of specific ions. They open and close in respond answer to electrical, mechanical and chemical stimulus, acting in the excitation or transmission of diverse tissues. DEVELOPMENT: The clinical and molecular manifestations of channelophathies are varied and use to shown up in continuous or paroxystic ways. Alteration of Ca channels cause muscle dysfunction periodic paralysis with or without potassium changes, myasthenia or myasthenic disorders, like Lambert Eaton syndrome, amyotrophic lateral sclerosis, Central Core disease, malignant hyperthermia. Cl and Na channels alterations produce myotonic diseases: Thomsen, Becker and paramyothonies, potassium sensible paralysis, fluctuant congenital myotonic, Andersen s syndrome. Channelopathies also produce various episodic ataxia type 1, type 2, spinocerebellar 6 and familial hemiplegic migraine. Abnormal paroxystic movements are present as channelophaties: episodic nocturnal dystonia, paroxystic dyskinesia. In some families are associates abnormal episodic movements and epilepsy. Several epileptic syndromes are also related with channels dysfunction: frontal lobe nocturnal epilepsy, choreoatetosis epilepsy, benign neonatal convulsions, generalized epilepsy with febrile convulsions plus. CONCLUSIONS: Voltage gated channels dysfunction are related to diseases with episodic phenomena or permanent conditions on muscle or neuronal tissues, with clinical and genetic heterogenous manifestations.

Humans↗

[Hyperattentional syndrome in children].

INTRODUCTION: The attentional mechanism is complex, it depends of multiple encephalic structures, acting all of them together. While using the attentional mechanism we accumulate information from the outside world, this trough the memory of work. The frontal lobe s integrity allows the development of the learning and attentional mechanism, its malfunction is associated with the attention deficit hyperactivity disorder. The attention is generally selective to different kind of stimuli. In this disorder the children have fixed and sustained visual to a variety of stimuli. CLINICAL CASES: We present three children, with vigilant alterations characterized on having uniform selective visual attention to stimuli from the exterior, with prolonged latency. Without any other neurological manifestation. CONCLUSIONS: Children with attentional deficits, generally present selective attention to certain outside world stimuli. The hyperattentional disorder is a variety of selective visual attention with prolonged latency on the object selected.

Attention↗

[Hereditary progressive levodopa sensible: Segawa's syndrome].

INTRODUCTION: Hereditary progressive childhood dystonia with diurnal fluctuation of symptoms, belongs to the dopa responsive dystonias. It is dominantly inherited with variable penetrance, with deficiency in the cyclohydrolase I GTP gene. OBJECTIVE: Levodopa treatment is useful and diagnosis may be done on fluctuant dystonia in the childhood. CASE REPORTS: . We present four patients, one boy and three girls (two sisters) between 7 and 17 years of age. Neurological symptoms appears at 5, 15, 2.5 and 4 years of age respectively, with incoordination of movements ataxo paretic gait and postural dystonia. Symptoms were progressive with diurnal fluctuation. All laboratory test and image diagnosis were normal. Levodopa response, with lower doses (30 60 mg/day), were excellent. The four patients respectively are asymptomatic after 8, 4, 6 and 5 years of treatment. CONCLUSIONS: Hereditary progressive dystonia, with diurnal fluctuations is dopa responsive at lower doses, with neurological normalization and without side effects during a prolongated treatment.

Adolescent↗

[Behçet's disease in children: cortical calcifications].

INTRODUCTION: Between 5 and 48% of the patients with Behçet s disease have neurological abnormalities. In children, meningoencephalitis as an initial manifestation of the disease is not frequent. CASE REPORT: We present a four years old girl with a Behçet s disease. When she was six months of age, during an hospitalization caused by malnutrition and respiratory infection, she had developed an aseptic meningoencephalitis and laboratory test showed hypergammaglobulinemia. When she was 21 months old, she had seizures as epilepticus status, and good response was achieve treating with anticonvulsive drugs. She did not repeat episodes of epileptic seizures, but the patient showed language and neuromotor problems. CT was abnormal with asymmetric ventricular dilatation and increased subarachnoides cortical sulci with multiple calcifications in those areas. She also suffered respiratory and gastrointestinal infections that required hospitalization. At the age of 3 years and 6 months, she had occular affection with uveitis, oral and genital ulceration. The clinical history of the girl consisting of meningoencephalitis, gastrointestinal and respiratory disorders, later presentation of uveitis and oral and genital ulcers confirmed the Behçet s disease diagnosis. CONCLUSION: We present a girl with multisystemic disorders characteristic of the Behçet s disease. Initially she had an aseptic meningoencephalitis developing cortical calcifications.

Behcet Syndrome↗

[Subacute sclerosing panencephalitis: fulminant form].

INTRODUCTION: Subacute sclerosing panencephalitis (SSPE) has become less frequent as a consequence of massive anti measles vaccination. Early infection or immunological factors could increase the risk of its appearing and of evolved forms of the disease. CASE REPORTS: We describe the cases of four patients with fulminating forms of SSPE: a girl who had measles at the age of eight months; a male who, without actually suffering the clinical disease, came into contact with measles in the family and, despite having been vaccinated, presented SSPE 18 months after the contact; a boy aged 4 years and 4 months who had measles at the age of 6 months, and a boy of a similar age who had the illness when he was one year old. They all developed ataxia, with focal and generalised neurological signs, myoclonic and atonic seizures with rapid deterioration of language and the cognitive functions. In the four cases, the computerised tomography scans were normal, the electroencephalograms showed bilateral paroxysms and periods of recurrent bioelectrical attenuation. The magnetic resonance images of the four patients revealed disseminated hyperintense lesions, and one of the patients presented hyperintense lesions in the cervical spinal cord. The anti measles IgG titres were high in the cerebrospinal fluid. Anti convulsive drugs were ineffective. In the third and fourth patients, intrathecal intraventricular treatment with interferon did not modify the course of the disease and neurological deterioration was seen in the subsequent follow up of all the cases. CONCLUSION: As a consequence of vaccination against measles, SSPE has become less frequent. Infection of infants, prior to the immunisation stage, can induce SSPE with periods of latency that are shorter than usual and with a fast progression of the disease.

Child, Preschool↗

[Acute benign ataxia in childhood].

The patogenesis and etiology of acute ataxia in childhood is not well known. It may occur without previous symptoms or may be the expression of specific infectious diseases. Forty patients hospitalized at the Hospital de Niños de Buenos Aires en 1972-1978, were studied. The neurological manifestations showed an acute onset, being ataxia the main sign, associate to tremor, nystagmus, dysartria, oculo-motor paresia, muscular weakness, and hyporeflexia. Most of the patients (82%) became cured within the first four weeks. It is advisable to establish a follow-up with periodic controls, mainly in those patients in whom an association with previous infectious diseases did not exist to be able to detect an association with degenerative or desmyelinizing diseases.

Acute Disease↗