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A case of meningo-angiomatosis (MA), in a 2.6 -years-old girl with refractory focal and secondary generalized seizures, starting at the age of 2 years, is presented. MRI evaluation revealed a lesion located at the left temporal lobe; the patient underwent surgical intervention. Histology revealed the lesion to have the features of MA.

Aphasia is a rare neurologic disorder in childhood. Nevertheless some authors believe that some subtle aphasic syndromes as anomic aphasia are not always identified and the real prevalence of aphasia in childhood is higher. We observed a case of aphasia during the acute period of Herpes Simplex Encephalitis in a 5 years old, right handed, boy with a good level of fluent speech. Six weeks after his initial assessment language was spontaneous, fluent, with normal prosodhy and articulation, but reduced, without content words, with frequent circunlocutory speech, semantic paraphasias, anomic latencies, real anomias, "pass partout" words. This picture was suggestive for anomic aphasia in a normally fluent context. Anomic aphasia can be produced by the disconnection between cortical and subcortical systems. Documented cases of anomic aphasia in childhood are rare. Our case present great similarities with adult cases in terms of localisation and denomination difficulties.

Guillain-Barré syndrome's diagnosis is not usually difficult, but diagnostic difficulty occurs for the variable initial presentation. Pain may be predominant as an initial symptom. Pain may be the primary complaint in the week preceding the onset of paralysis in some children. When irritability and withdrawal from child's usual activities is predominant the clinician is first concerned about an encephalopathic process. When the primary problem at presentation is limb and back pain the pathology appears to be in the musculoskeletal rather than in neurological system. The awareness of this presentation of Guillain-Barré syndrome will eliminate delay in diagnosis.

Fainting syncopal events are caused by a transient functional neuronal paralysis. Reflex syncope happens for brainstem involving mediated by peripherical afferents. Sometimes gastroesophageal reflux (GER) has been implicated in the development of obstructive apnea. Gastroesophageal reflux, despite the absence of a clinical history of vomiting and regurgitation, is observed in a significant proportion of infants presenting with ALTE (Apparent Life Threatening Event): an episode characterized by some combination of apnea, color change, marked change in muscle tone, choking or gagging. Though a cause-and-effect relationship between GER and the development of ALTE remains to be established a possible direct relationship between oesophageal acidification and the onset of alterations in cardiopulmonary function and impairment of consciousness can be hypothesized. We refer the case of two female infants that developed recurrent ALTE(s) characterized by paleness, change in muscle tone and loss of consciousness. The infants resulted affected respectively by a mild and severe gastroesophageal reflux (score: 40, > 50); in one case an episode of GER was recorded by the intraoesophageal pH-monitoring during a syncopal episode. The treatment with antiacid drugs was effectual and the infants did not present ALTE(s). The cases presented are in favour of a routine search of gastroesophageal reflux in infants presenting with one or recurrent ALTE(s). The identification of these infants will permitt to develop a correct strategy of treatment.

Intussusception is the most frequent cause of intestinal occlusion in children aged 3-5 years. Diagnosis is easy to perform in cases with typical clinical presentation. However diagnosis may be difficult in rare cases with atypical symptoms characterized by SNC involvement, that could delay a convenient treatment. We report 4 cases of intussusception presenting initially with consciousness abnormalities, hypotonia and vomiting. In these cases differential diagnosis with other conditions associated with comatose states has to be performed. Some hypothesis may be considered to explain these clinical pictures: 1. increased endorphins excretion during abdominal pain; 2. neurotoxins of bacterial origin released and absorbed by altered bowel; 3. intestinal hormones abnormally produced during the disease. As recommended by Rachmel, the condition should be suspected in all children presenting with the association of vomiting and lethargy and a radiological or tomographic study of abdomen should be performed also in cases without intussusception typical symptoms.

Migraine is a variant of headache often associated with neurologic and/or vegetative symptoms mainly represented by abdominal pain. This symptom may occur some hours before migraine manifestation and in these cases the differential diagnosis with other clinical conditions characterized by abdominal pain, which is very common during childhood, may be difficult. Abdominal migraine can be diagnosed only if a close relationship is demonstrated between the abdominal symptoms and migraine. Alteration of consciousness is a well known feature during migraine and in some cases EEG may show SNC involvement during the attack. We report a case of abdominal migraine attack evaluated by EEG. The patient, a 10 years old male, presented with a picture of acute abdomen. An EEG performed at the occurrence of the early headache symptoms and of consciousness alteration demonstrated a pattern characterized by a lowering in the electric activity on the left hemisphere. Some hours later he developed a clear migraine followed by disappearance of the abdominal symptoms. This observation confirms the possible association of migraine with a picture simulating an acute abdomen and suggests that the differential diagnosis with a true surgical condition may be achieved by the observation of the progression of symptoms and by early evaluation of patient with EEG.

Grisel's syndrome is an atlanto-axial dislocation affecting children between 6 and 12 years. The outstanding symptom is a spontaneously arising torticollis. The most likely etiology seems to be an inflammation of the retropharyngeal space caused by upper respiratory tract infections or by adenotonsillectomy. Some A.A. consider Grisel' syndrome clinical features strictly due to predisposing conditions. The anamnesis and clinical signs are important clues to diagnosis. The A.A. report a case of an apparently spontaneous torticollis occurred few days after adenotonsillectomy in a 7 years old girl.

The authors describe one case of partial 9q trisomy they observed. The malformations they observed are correspondent to the very little amount of existing documented cases. And just because we have only a few observations, we thought useful publishing this case, to better define the clinical features among the alterations of chromosome 9 (trisomy 9 p and 9q). Head, neck, bones, heart and urogenital apparatus seen to be the most frequently involved in the phenotypic expression of the 9q trisomy.

The authors describe the case of a five-year old girl presenting short stature. The evidence of small hypoplasic nose, of abnormally small hands and feet with typical radiological alterations and psychomotor retardation, leads to the diagnosis of acrodysostosis. Moreover, the endocrinological investigations disclosed a dysequilibrium in the pituitary-thyroid axis, which, to our knowledge, has never been described to date in the syndrome of acrodysostosis.

Kawasaky's disease is a multisystem widespread vasculitis. Besides the mucocutaneous patterns, symptoms related to various organs have been observed in the medical literature. Lethargy, irritability, meningism and cranial nerves paralysis occur in the acute phase of central nervous system involvement. The A.A. report a rare cerebellar syndrome caused by vasculitis in a seven years old girl's cerebellum. Surveillance of tardive complications must be undertaken in patients affected by Kawasaky's disease.