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Duchenne muscular dystrophy (DMD) is a lethal X-linked recessive muscle disease due to defect on the gene encoding dystrophin. The lack of a functional dystrophin in muscles results in the fragility of the muscle fiber membrane with progressive muscle weakness and premature death. There is no cure for DMD and current treatment options focus primarily on respiratory assistance, comfort care, and delaying the loss of ambulation. Recent works support the idea that stem cells can contribute to muscle repair as well as to replenishment of the satellite cell pool. Here we tested the safety of autologous transplantation of muscle-derived CD133+ cells in eight boys with Duchenne muscular dystrophy in a 7-month, double-blind phase I clinical trial. Stem cell safety was tested by measuring muscle strength and evaluating muscle structures with MRI and histological analysis. Timed cardiac and pulmonary function tests were secondary outcome measures. No local or systemic side effects were observed in all treated DMD patients. Treated patients had an increased ratio of capillary per muscle fibers with a switch from slow to fast myosin-positive myofibers.

To determine the influence of the APOEgenotype on functional and cognitive outcome and on the incidence and prognosis of clinical vasospasm (delayed ischemic neurologic deficit DIND) in noncomatose patients with aneurysmal subarachnoid hemorrhage (SAH).

Summary: ¶ International co-operative studies have demonstrated a benefit from surgery for symptomatic and asymptomatic patients affected by internal carotid artery stenosis of 60–70%. The presence of a tandem lesion, intracranial or extracranial, may annul the benefit of surgery. Such patients may thus represent a challenging problem for management if age, good general conditions and a normal neurological status favour a therapy. A 54-year-old man developed transient ischaemic attacks of the left hemisphere; his general condition was good, and neurological status was normal. Angiography showed a tight stenosis at the left common carotid artery near the ostium and at the homolateral carotid bifurcation. At first, a self-expanding wall stent was placed at the level of the common carotid artery stenosis, and immediately after a standard endarterectomy under general anaesthesia was performed. The postoperative course was normal and was complicated only by the presence of a mild deficit of the hypoglossal nerve due to the presence of a high bifurcation. The early and late outcome of our case suggests that stenosis of the proximal common carotid artery may be successfully treated by stenting. While awaiting additional data about this new technology, endovascular techniques and surgery may be complementary in the management of patients suffering from such tandem lesions.

Abstract: Thirteen patients with tumors in the pineal region were submitted to pre‐ and post‐operative blood sampling (08:00, 14:00, 20:00, and 02:00 hr) for three or four consecutive days. A single cerebrospinal fluid (CSF) sample was collected at surgery, and melatonin levels determined. In all patients, serum and CSF beta subunit of human chorionic gonadotrophin (βHCG), carcino embryonic antigen (CEA), and alpha‐fetoprotein (AFP) levels were measured. Histology revealed four pineocytomas, one pineoblastoma, four germinomas, one immature teratoma, one pilocytic astrocytoma, one lymphoma, and one meningioma. Serum and CSF levels of serological biomarkers were normal, except for one of the germinoma cases. In most patients, alteration either in the circadian rhythm or in the melatonin concentration was observed before surgery. In benign neoplasms the circadian rhythm was conserved. In pineoblastoma, lymphoma, and three out of four germinomas, melatonin concentrations were undetectable. In one case of germinoma, melatonin levels were high, with the circadian rhythm being abolished. According to conventional histology, all germinomas were similar. Therefore, in a rare case of pineal germinoma with high melatonin levels, the tissue was subjected to an in depth investigation (immunohistochemical and ultrastructural) in order to determine the pathology and the possible differences from the other typical germinomas. Results were compared to those provided from other pineal neoplasms. Electron microscopy examination detected the presence of clusters of intermediate filaments and numerous electrondense granules only in the case of a germinoma producing melatonin.

Summary Over the past 4 years 11 patients with Cushing's disease have been operated on by a transsphenoidal microsurgical technique. All patients had small intra-hypophyseal adenomas found at operation. Eight of the 11 patients had a remission of the disease on the basis of clinical evaluation and hormonal studies. The endocrinological results revealed a transient state of hypocorticism in all successfully treated patients. Within 6–8 months after operation recovery of hypothalamic-pituitary-adrenal function and other tropin function took place.

The content and distribution of phospholipids, as well as their fatty acid composition, were studied in 16 human meningiomas in comparison with normal leptomeninges.

Multimodality evoked potentials (somatosensory and acoustic) have been recorded before surgery in children selected for surgical treatment for different kinds of craniostenosis and craniofacial stenosis. Evoked potentials were found to be deranged on at least one recording in 50 of cases, suggesting a significant incidence of neurophysiological alterations in these patients. No correlation was found between evoked potentials' derangements and clinical status, radiological findings, or different kinds of craniostenosis; moreover, a significant correlation was found between neurophysiological alterations and the age of the children. These preliminary data might define the possible role of neurophysiological tests-in the presurgical-evaluation of craniostenosis and craniofacial stenosis.

Summary Of 600 patients submitted to radioisotope cisternography carried out with radioiodinated human serum albumin (I131-HSA) a group of 140 had head injuries. Our investigation was intended to study the modifications of CSF circulation and absorption which accompany cranio-cerebral trauma. Of 88 head injured patients who did not undergo operation 44 had transient loss of consciousness, and 44 were in prolonged coma. Fifty-two patients underwent operation. Of these 5 had skull fractures with dural lesions, 7 had extradural haematomas, 19 had subdural haematomas, and 21 had brain contusions.

Summary There are at present strong indications for surgery in patients suffering from symptomatic extracranial carotid stenoses of >70%. Surgery of coincidental aneurysms is a still debated problem, but there is general agreement that it is indicated in selected cases according to the patient's life-expectancy and size and site of the aneurysm. The coexistence of these two lesions raises a decisionmaking problem. We reviewed 389 endarterectomies and found 12 intracranial berry aneurysms in 10 (2.6%) patients. All the 10 patients were harbouring a symptomatic carotid stenosis of >70%. Since the correction of a stenosis increases blood flow to an aneurysm, our approach was to first operate on the intracranial lesion and then the stenosis in 7 patients harbouring aneurysms >5 mm. Two patients affected by small aneurysms <5 mm of an A2 azygos and left internal carotid artery underwent left endarterectomy only. The last patient was submitted first to percutaneous angioplasty of a left stenosis, then to open surgery of a contralateral middle cerebral aneurysm and finally to intravascular occlusion of a small aneurysm of the left internal carotid bifurcation by menas of a coil; this policy was adopted in order to restore normal haemodynamic conditions before the intracranial procedure. There was no mortality or permanent morbidity following surgery for aneurysm or endarterectomy. Transient morbidity occurred in 2 cases after clipping of aneurysms of the anterior communicating and middle cerebral arteries. Our results suggest that surgery of coincidental aneurysms may give good results even when there is a severe symptomatic stenosis in the neck. Moreover, the presence of a small intracranial aneurysm does not seem to be an additional risk factor for endarterectomy. When the lesions are on different sides, it may be better to treat the stenosis first if it decreases the ipsilateral cerebral blood flow.

Although patients with brain lesions of neurosurgical interest may sometimes present with a cognitive profile very similar to a primary dementia, they usually have a history and/or a neurologic examination which alert the clinician. The occurrence of mass lesions with purely isolated cognitive deterioration has been mainly reported for locations at the level of the frontal lobes. A potentially positive prognosis is advocated in these cases, in terms of even complete reversibility of the mental impairment when surgical removal is feasible and successful. We report two rare cases of posterior intraventricular meningiomas presenting with isolated cognitive and behavioural changes consistent with a frontal type dementia; the outcome was favourable in one patient, whereas the second one suffered from massive intracerebral bleeding during the neurosurgical removal and required prolonged hospitalization and subsequent intensive neurorehabilitative efforts. By comparing our cases with previously published decision‐rules regarding neuroimaging in dementia, we suggest that criteria should be enlarged, especially those regarding the age of the subject and the time elapsed from the first signs of the cognitive deterioration.

Forty-one children with severe head injuries and diffuse brain lesions were selected from a consecutive series of 62 children in traumatic coma (21 focal mass lesions) and studied. According to the CT pattern, two main types of intracranial lesions were considered: diffuse axonal injury (DAI) and diffuse brain swelling (DBS). High mortality, due to secondary increases of intracranial pressure (ICP), correlated well with the patterns of severe DBS, absence of perimesencephalic cisterns, and obliteration of the ventricles. However, children with normal CTs, and/or obvious shearing injuries indicative of DAI, had favorable outcomes; there was no mortality if increased ICP was not present. We conclude that although there does not seem to be any routine indications for ICP monitoring in children with pure DAI, early ICP monitoring and aggressive management of increasing ICP should be considered in comatose children with DBS, especially when associated with subarachnoid hemorrage and respiratory or circulatory failure.

Summary The authors report their experience of isotope cisternography and ventriculography with I131 or Tc99m labelled albumin. Eighty-nine patients with different neurosurgical diseases responsible for alterations of the C.S.F. circulation and absorption were studied during a twenty months' period. The codition studied included; communicating hydrocephalus, obstrucsive hydrocephalus, pseudotumor cerebri, hemispherectomy, alteration of C.S.F. circulation following neurosurgical procedures, and C.S.F. rhinorrhoea.

Summary The authors describe the techniques of isotope cisternography and ventriculography with high specific activity I131 and Tc99m albumin in children and adult patients. They have studied 35 patients including different type of obstructive and communicating hydrocephalus, microcephalic patients and patients with no intracranial pathology. The value, the limitations, and the potential damage of the test are discussed and the proper use of the two different tracers is assessed.