Miguel A. Pérez-Espejo

Neurotrophic bone marrow cellular nests prevent spinal motoneuron degeneration in amyotrophic lateral sclerosis patients: a pilot safety study.

The objective of this article is to assess the safety of intraspinal infusion of autologous bone marrow mononuclear cells (BMNCs) and, ultimately, to look for histopathological signs of cellular neurotrophism in amyotrophic lateral sclerosis (ALS) patients. We conducted an open single arm phase I trial. After 6 months observation, autologous BMNCs were infused into the posterior spinal cord funiculus. Safety was the primary endpoint and was defined as the absence of serious transplant‐related adverse events. In addition, forced vital capacity (FVC), ALS‐functional rating scale (ALS‐FRS), Medical Research Council scale for assessment of muscle power (MRC), and Norris scales were assessed 6 and 3 months prior to the transplant and quarterly afterward for 1 year. Pathological studies were performed in case of death. Eleven patients were included. We did not observe any severe transplant‐related adverse event, but there were 43 nonsevere events. Twenty‐two (51%) resolved in ≤2 weeks and only four were still present at the end of follow‐up. All were common terminology criteria for adverse events grade ≤2. No acceleration in the rate of decline of FVC, ALS‐FRS, Norris, or MRC scales was observed. Four patients died on days 359, 378, 808, and 1,058 post‐transplant for reasons unrelated to the procedure. Spinal cord pathological analysis showed a greater number of motoneurons in the treated segments compared with the untreated segments (4.2 ± 0.8 motoneurons per section [mns per sect] and 0.9 ± 0.3 mns per sect, respectively). In the treated segments, motoneurons were surrounded by CD90+ cells and did not show degenerative ubiquitin deposits. This clinical trial confirms not only the safety of intraspinal infusion of autologous BMNC in ALS patients but also provides evidence strongly suggesting their neurotrophic activity. STEM CELLS2012;30:1277–1285

The child with a mass on its head: diagnostic and surgical strategies

A series of 65 pediatric patients with scalp or calvarial masses is reported on. The majority of children presented with a disfiguring or painful mass on the head. Clinical findings suggested the correct diagnosis in 39/65 cases, skull radiographs in 46/65, and CT in 49/65. Taking the combined results of clinical and radiological studies, 54/65 of the lesions were accurately diagnosed. Tumor excision was curative in 43 of 48 patients who were operated on. Most scalp and calvarial neoplasms were benign; only 5/65 children harboured a malignant lesion. There was no mortality related to surgery in the series. Surgical intervention seems to be indicated in most cases, both for diagnosis and for treatment.

Autologous tissues for dural grafting in children : a report of 56 cases

BackgroundDiverse materials have been used for dural closure, including grafts of cadaveric origin. Some 168 cases of Creutzfeldt–Jakob disease have been reported following the implant of human dura mater. A few publications have addressed the use of dural grafts in children, but none has analyzed the feasibility of autologous tissues for this purpose.AimSince 1994, the authors have utilized autologous tissues for dural grafting in children undergoing neurosurgical procedures, aimed at studying the feasibility of its use in this age group.Material and methodsWe studied 56 children submitted to neurosurgical procedures who underwent an implant of a patch of autologous tissue for dural closure. Epidemiological, clinical, and neuroimaging data were processed using statistical methods.ResultsThe study group was composed of 29 boys and 27 girls, with ages ranging from 1 month to 17 years (mean 7.6 years). The procedures corresponded to lesions at the posterior cranial fossa (n=41), supratentorial cranial compartment (n=12), and spine (n=3). Seventy-five percent of the children had no complication. Four patients had adverse effects unrelated to dural grafting, while ten children had complications that might be associated with it. The most frequent complication was pseudomeningocele (n=6), followed by infection (n=3) and CSF leak (n=1). Although hydrocephalus seemed to have an influence on the occurrence of complications, their incidence in patients without hydrocephalus did not differ statistically.ConclusionsAutologous grafts of fascia or pericranium can be safely used for dural reconstruction in children. Given the reported complications associated with the use of foreign materials for duraplasty, the authors recommend the use of autologous tissues for dural repair, reserving other types of dural grafts for cases in which its usage is unavoidable.

Shunt-related craniocerebral disproportion: treatment with cranial vault expanding procedures.

Two patients with intracranial arachnoid cysts, one with myelomeningocele–hydrocephalus and the other with a subdural fluid collection, were given a cerebrospinal (CSF) extracranial shunt. All four patients developed features of CSF overdrainage following shunting and were treated by cranial vault expanding procedures. Before undergoing decompressive craniotomy, the patients were treated by a variety of procedures, including changing of obstructed ventricular catheters (n=4), insertion or upgrading of programmable valves (n=3), and foramen magnum decompression (n=1). Clinical manifestations of these four patients were attributed to craniocerebral disproportion caused by chronic and progressive skull changes due to dampening of the CSF pulse pressure, which is necessary for maintaining normal cranial growth. On the basis of our previous experience with expanding craniotomies in cases of minimal forms of craniosynostosis, we treated these patients with bilateral parietal craniotomies, with satisfactory results. In conclusion, biparietal decompressive craniotomy constitutes a useful and safe procedure for relieving the clinical manifestations of some CSF overdrainage syndromes, especially in cases with slit-ventricle syndrome and craniocerebral disproportion that prove to be refractory to simpler management procedures.

Hydrocephalus and arachnoid cysts

AimThe management of arachnoid cysts (AC) remains controversial. An additional problem derives from the management of hydrocephalus associated with an AC. In this work, we discuss existing procedures proposed in the current literature for their treatment.MethodsWe reviewed selected reports on intracranial ACs placing special interest in those about the association of hydrocephalus and ACs. We also briefly surveyed data of our patients with this association.Results and discussionHydrocephalus is often found in midline and posterior fossa ACs. Interhemispheric lesions may also evolve with ventriculomegaly, while middle fossa lesions rarely produce hydrocephalus. Patients’ age, cyst location and size, and macrocephaly have all been related to the development of hydrocephalus. Some authors remark on the role played by hydrocephalus and hypothesize that some ACs would result from disturbed cerebrospinal fluid (CSF) dynamics. They also propose that ACs might represent a localized form of hydrocephalus. We also comment on hydrocephalus in relation to the diverse locations of ACs. Neuroendoscopic techniques have transformed previous ways of management as cystoperitoneal shunting and open fenestration.ConclusionsACs may be pathogenetically related with hydrocephalus, and conversely, ACs may cause hydrocephalus. In some patients, aberrant CSF dynamics seems to play a major role in the development of both cyst and hydrocephalus. Hydrocephalus and ACs may be treated exclusively with neuroendoscopic procedures, although some patients will still require CSF shunting. The ideal option seems to consist of choosing the method that offers the highest success with a single procedure for treating the hydrocephalus and the AC simultaneously.

A surgical technique of spinal cord cell transplantation in amyotrophic lateral sclerosis.

We report an original method for implanting bone marrow stem cells within the spinal cord parenchyma. This method was used for the experimental treatment of patients diagnosed with amyotrophic lateral sclerosis. The methodology is reproducible and devoid of major complications even in patients showing significant spinal atrophy. Therefore, this report describes a surgical procedure that could be used in other experimental treatments involving the intraspinal delivery of stem cells.

Positional skull deformities in children: skull deformation without synostosis

BackgroundPatients with craniosynostosis are readily diagnosed by clinical and neuroimaging findings. Surgical treatment is indicated for preventing neurological deficits and for correcting esthetically unacceptable head deformities. In recent years, we have witnessed a progressive number of neurosurgical consultations for abnormal head shapes unrelated to premature fusion of the cranial sutures, especially of positional plagiocephaly. There have been descriptions of abnormalities in the cerebrospinal fluid (CSF) spaces in children with craniosynostosis.ObjectivesThe aim of the present study was to investigate the role of the changes of the CSF spaces in the development of positional skull deformities in children.Patients and methodsThe authors reviewed demographic, clinical, and neuroimaging data of 23 patients assessed for some form of nonsynostotic skull deformity (group A). The results were compared with those of a simultaneous group of nine infants diagnosed with benign extracerebral collections of fluid (group B).ResultsThe study group was composed of 11 boys and 12 girls, aged 3 years or younger. Seventeen children had plagiocephaly, four scaphocephaly, and two brachycephaly. Sixteen children (15 with plagiocephaly and 1 with brachycephaly) exhibited enlarged subarachnoid CSF spaces. In group B, the boy/girl ratio was of 7:2. Infants in group A presented at an older age (mean 12.7 months) than group B (mean age at presentation of 7.17 months). Children with benign extracerebral fluid collections were born with a head circumference (HC) greater than infants with positional skull deformities (p=0.005). The percentile of the children’s HC at consultation was also larger for children of group B (p=0.03). The form of clinical presentation differed between the two groups. Most infants of group A were seen because of a type of head deformity, and children in group B were studied for macrocephaly. Long-term follow-up assessment showed better outcomes for patients in group B than for children of group A in regard to regression of initial symptoms (p=0.03).ConclusionsMost positional head deformities appear to be related with the children’s positioning for sleeping. We have not confirmed macrocephaly as a contributing factor for positional deformities. The distribution of extracerebral CSF and the presence of abnormal collections of fluid in children with positional head deformities do not seem to be related with the findings of pericerebral CSF encountered in children with benign extracerebral collections of fluid. In our view, brain pulsations, transmitted to these accumulations of CSF, play an important part in the development of the infants’ skull deformities (p=0.02). The findings of enlarged CSF spaces in children with nonsynostotic skull deformation constituted an age-related event, as these collections tended to disappear as the children grew older (p=0.04).

Thalamic tumors: clinical presentation.

AbstractIntroduction.Thalamic tumors (TTs) account for approximately 1% of all intracranial neoplasms. TTs are seen predominantly in children and young adults. Most childhood neoplasms in this location are of glial lineage, a large proportion being low-grade tumors. The evolution of symptoms before diagnosis is characteristically shorter in children than in adults. Clinical presentation.Clinical features of TTs reflect the pressure of the mass on the cerebrospinal fluid pathways, the pyramidal tracts, the thalamic nuclei and the optic radiations. In this paper, we review the clinical data of 20 children with TTs seen at our hospital and compare the findings with those in the current literature. An acute presentation is not rare and was encountered in 20% of our patients, although most individuals had a subacute or slow evolution. Symptoms and signs of raised intracranial pressure (65%), motor deficits (40%), and seizures (35%), alone or in combination, were the most frequent manifestations of TTs. Behavioral and mental changes are not uncommon in TTs and were found in 25% of our patients. Involuntary movements (10%) and the classic thalamic syndrome (5%) were quite exceptional. Infants and young children with TTs may present with macrocephaly, psychomotor delay, and failing vision or disorders of ocular movements.

Rhabdoid meningioma : a new subtype of malignant meningioma also apt to occur in children

Case reportThe case of a 14-year-old girl who presented with a 2-week history of raised intracranial pressure is reported. A left frontal extra-axial tumor was totally removed, whose histopathologic diagnosis was rhabdoid meningioma (RM).Discussion Rhabdoid meningiomas constitute a special malignant phenotype of meningioma that has been recently included in the WHO classification of tumors of the nervous system. Usually, RMs affect middle-aged and elderly individuals. We report the fourth case of a RM occurring in a child to illustrate that the diagnosis of this tumor subtype, given its prognostic implications, must also be considered in pediatric patients

Midline cutaneous lumbosacral lesions: not always a sign of occult spinal dysraphism

ObjectiveThe authors report two unusual cases of extraspinal midline lumbosacral lesions that resembled the usual skin markers of occult spina bifida.Patients and methodsThe pathological diagnosis of the masses was plexiform neurofibroma and mastocytoma, respectively.ResultsSome lesions, as those we are reporting, although occurring at the lumbosacral midline, do not necessarily belong to occult spinal dysraphism, as was initially suspected. Plexiform neurofibroma and skin mastocytoma are very rare indeed in this spinal location.ConclusionThe two cases represent an important addition to the differential diagnosis of a congenital dorsal midline mass.