Subarachnoid and intra-cerebral hemorrhage in young adults: rare and underdiagnosed.

OBJECTIVES: Convexity subarachnoid and intra-cerebral hemorrhages, in patients aged<50 years, are always a diagnostic challenge. This condition is characterized by acute headaches with or without neurological symptoms and/or seizures, and by the radiological demonstration of subarachnoid and/or intra-cerebral hemorrhages and, more rarely, by the association of ischemic events. PATIENTS AND METHODS: In a prospective series of 30 consecutive patients (median age 31 years; 22 women) with a subarachnoid and intra-cerebral hemorrhages, 19 were diagnosed with reversible cerebral vasoconstriction syndrome (RCVS), 7 with cerebral venous sinus thrombosis (CVST), and 4 with a bleeding mycotic aneurysm (MA). RESULTS: RCVS appeared spontaneously in 16 patients and was related to the postpartum period in three cases. Subarachnoid hemorrhage (SAH) was demonstrated in 24 patients as follows: 18 cases were in cortical areas, 4 were in the polygon of Willis, one was inter-hemispheric, and one was inter-hemispheric/intra-cerebral. A convexity pure intra-cerebral hemorrhage (ICH) was recorded in 6 cases. Among the 7 patients suffering from CVST, the superior sagittal sinus was involved in 4 cases, the transverse sinuses (TS) in 2, and the TS plus sigmoid sinus (SS) in one. CONCLUSION: The three most common causes in this series were RCVS, followed by CVST and bleeding from MA. Because of atypical clinical or radiological presentations, this large spectrum of etiologies can cause diagnostic difficulties. Therefore, careful analysis is needed to ensure correct and prompt diagnosis and to avoid any dangerous delays in management.

Neuroendoscopic management of posterior third ventricle and pineal region tumors: technique, limitation, and possible complication avoidance.

The endoscopic approach has gained an increased popularity in recent years for the biopsy and, in selected cases, the removal of tumors of the posterior third ventricle and pineal region. The authors report their experience on a series of 20 patients discussing also the technical limitations and complication avoidance. This is a prospective study of 20 patients with posterior third ventricle and pineal region tumors surgically managed by endoscopic biopsy and/or excision and simultaneous third ventriculostomy. The removal of the lesion could be achieved in 12 cases whereas in 8, only a biopsy could be performed. A histological diagnosis could be obtained in all cases. No delayed third ventricular stoma failures were recorded in any patient at the latest follow-up (mean follow-up, 39 months). Severe postoperative complications were recorded in 2 out of 12 cases of tumor removal attempt and in zero out of eight cases of biopsy. A delayed (3 weeks) postoperative mortality occurred in a patient harboring a GBM that developed an intratumoral hematoma 48 h postoperatively, one patient was in a vegetative state. Transient postoperative complications included: nausea and vomiting (five cases) and diplopia (two cases). One patient developed a bilateral ophthalmoplegia that recovered within 6 months due to residual tumor hemorrhage. Higher rate of complications was found in the case of vascularized and/or larger lesions. Endoscopic management of posterior third ventricle lesions may represent an effective option. However, though biopsies remain often a safe procedure, tumor excision should be limited to highly selected cases (cystic, poorly vascularized, and/or smaller than 2.5-cm lesions).

Intrathecal baclofen therapy for severe spasticity: analysis on a series of 112 consecutive patients and future prospectives.

OBJECTIVE: Intrathecal baclofen therapy (ITB) is a well-known treatment for spasticity. Despite this fact, several topics have to be still discussed: new indications and screening tools, appropriate surgical timing and complicance avoidance. METHODS: A total of 112 consecutive patients all with a severe, progressive and refractory to medical therapy spasticity from different causes were treated using ITB, after a bolus test. Every patient was assessed by means of Modified Ashworth Scale (MAS), Penn spasm frequency scale (SFS) and Visual Analog Scale for pain. Since available, a Gait analysis was also performed. RESULTS: There were 63 males (56%) and 49 females (44%). Seventy-four (66%) had a quadriparesis, 34 (30.4%) had a paraparesis and 4 (3.6%) were hemiplegic. Among these patients 77 (68.7%) were non ambulatory, while 35 (31.3%) were ambulatory. These patients suffered from spasticity due to many different diseases. Mean follow-up was 55 months. The mean Modified Ashworth score decreased from 4.5±0.5 preoperatively to 1.2±0.4 on chronic intrathecal baclofen. Daily baclofen dose varied between 23 and 500 mcg. Drug-induced complications and catheter related problems occurred, respectively in 7 (6.3%) and 10 patients (8.9%). CONCLUSIONS: Although ITB is a well known and good treatment option in the management of severe spasticity, because of the different goals and subgroups of patients treated, a variety of techniques are needed to evaluate the benefits of this therapy. New indications, effects of ITB on central nervous system and cognitive functions needs yet to be fully clarified.

Dorsal epidural spinal lipomatosis.

The authors report a case of a thoracic epidural spinal lipomatosis causing severe neurological deficits along the review of pertinent literature. The patient is a 56-year-old woman who presented with acute onset of severe paraparesis; she was investigated with cervical and thoracic MRI and then surgically managed because of an intraspinal mass compressing the cord. The operation consisted in the excision of the mass confirmed to be a fibrolipoma by pathological analysis. The patient attained complete neurological recovery and at 18 months follow-up she reported a generalised well-being. Thoracic lipomas are rare lesions that presenting mostly with back pain; however, in rare instances they may cause progressive and/or abrupt neurological dysfunction. Appropriate imaging can help in the diagnosis and management of such cases.

Neurosurgery and elderly: analysis through the years.

The aging of the population in westernized countries constitutes an important issue for the health systems struggling with limited resources and increasing costs. Morbidity and mortality rates reported for neurosurgical procedures in the elderly vary widely. The lack of data on risk benefit ratios may result in challenging clinical decisions in this expanding group of patients. The aim of this paper is to analyze the elderly patients cohort undergoing neurosurgical procedures and any trend variations over time. The medical records of elderly patients (defined as an individual of 70 years of age and over) admitted to the Neurosurgical and Neuro-ICU Departments of a major University Hospital in Paris over a 25-year period were retrospectively reviewed. The analysis included: (1) number of admissions, (2) percentage of surgically treated patients, (3) type of procedures performed, (4) length of hospital stay, and (5) mortality. The analysis showed a progressive and significant increase in the proportion of elderly presenting for neurosurgical elective and/or emergency procedures over the last 25 years. The number of procedures on patients over 70 years of age increased significantly whereas the mortality dropped. Though the length of hospital stay was reduced, it remained significantly higher than the average stay. The types of procedures also changed over time with more craniotomies and endovascular procedures being performed. Age should not be considered as a contraindication for complex procedures in neurosurgery. However, downstream structures for postoperative elderly patients must be further developed to reduce the mean hospital stay in neurosurgical departments because this trend is likely to continue to grow.

Double skull lymphangioma. Case report and review of the literature.

OBJECTIVE: It is still controversial whether lymphangiomas are a true entity or a combination of congenital venous and lymphatic malformations. Lymphangioma diagnosis is made on a multifactorial basis and surgery should provide cosmetic and functional preserving results. Only few multiple localization have been described in the literature and to our knowledge this is the first case reported on a double localization (orbital and temporal) in the skull. CASE DESCRIPTION: A 10-year-old girl was referred to our department with a rapidly enlarging subcutaneous mass in the right eyebrow and upper eyelid, displacing the eyeball and causing proptosis and diplopia that occurred synchronous with her menarche. A head MRI scan showed two distinct lesions, one into the orbit causing bony destruction and the other one in the sub-temporal area. The little girl underwent an open biopsy, suggesting the diagnosis of lymphangioma. Surgery through an anterior approach with total removal of both lesions was performed. Pathological examination disclosed a lesion consisting of multiple cysts with erythrocytes and lymphs, confirming the diagnosis. At a 6- and 12-months follow-up, the girl was clinically intact with normal ocular movements and a follow-up MRI showed no evidence of residual disease and/or recurrence.

Spontaneous intraparenchymal tension pneumocephalus triggered by compulsive forceful nose blowing.

The case is described of a 50-year-old man, treated for 10 years in an outpatient psychiatric clinic for an obsessive compulsive disorder, who presented with acute loss of consciousness after forceful nose blowing. A CT scan revealed an intraparenchymal air collection with tension signs in the left frontal lobe and a bone defect in the roof of the ethmoid sinus. After emergency left frontal craniotomy and dura opening, the gaseous collection was evacuated by a ventricular catheter inserted into the brain and the bone defect was repaired with pericranium flap and muscle. The postoperative course was uneventful with neurocognitive improvement and regained motility. Spontaneous tension pneumocephalus is a rare life-threatening condition which is often caused by a bone defect near the tegmen tympani. This case illustrates both an unusual cause and a unique surgical treatment for spontaneous tension intraparenchymal pneumocephalus. It can be a dangerous entity with potential for early mortality and long-term morbidity if not promptly decompressed. The pathogenesis, diagnosis and surgical strategies for spontaneous tension pneumocephalus are briefly discussed.

Primary malignant rhabdoid tumour of the brain in adults.

Malignant rhabdoid tumour (MRT) was described for the first time in the kidney, and is rarely reported in the brain. Most rhabdoid tumours affect infants and young children and there have been only isolated adult patients reported. The optimal treatment for this very aggressive tumour has not yet been established. We describe the clinical and pathological features of a rare primary malignant rhabdoid tumour of the brain in a 27-year-old pregnant female. The literature is reviewed briefly and the role of the INI1 gene in adult MRTs and the also possible interactions between MRTs and pregnancy are discussed.

Skull bone flap fixation--comparative experimental study to assess the reliability of a new grip-like titanium device (Skull Grip) versus traditional sutures: technical note.

INTRODUCTION: After completing a craniotomy, whenever possible, it is crucial to replace and fix the removed bone flap to the cranium; this in order to keep the brain's protection as well as for cosmetic purposes. Visible skull defects might cause patients psychosocial problems and, most importantly, expose the brain to accidental damage. A fixation device should not only provide optimal attachment of the flap to the skull but also allow fast bony healing to avoid possible pseudoarthrosis and/or osteolytic changes. METHODS: After performing 12 different craniotomies on 4 human cadaver heads the skull flaps were replaced using traditional sutures and a new skull fixation device; for each fixation technique a load-bearing test was performed and the results compared. RESULTS: Bone flaps fixed with the "Skull Grip" showed a strong fixation with optimal plastic deformation when compared to flaps held by sutures that showed less resistance to pressure and could be easily dislocated. CONCLUSION: The "Skull Grip" has shown to be a reliable, effective, and stronger bone flap fixation superior to suturing technique.

Peripheral median nerve stimulation for the treatment of iatrogenic complex regional pain syndrome (CRPS) type II after carpal tunnel surgery.

We report on the use and follow-up of direct peripheral nerve stimulation of the median nerve for the treatment of iatrogenic complex regional pain syndrome (CRPS). A 56-year-old woman presented with CRPS type II in the right forearm and hand, which had started after multiple carpal tunnel surgeries and had lasted for 2 years. The visual analogue scale (VAS) score was 8-10 out of 10. After a successful 15-day trial of median nerve peripheral nerve stimulation via a quadripolar lead in the right carpal tunnel space, an implantable pulse generator was inserted in the right infraclavicular space. The VAS score decreased to 1-2 out of 10 and the patient regained the ability to sleep. After 36 months of follow-up, the patient was still experiencing good pain relief without other treatment. We conclude that peripheral nerve stimulation is easy to use in pain management and could offer a valid treatment option for iatrogenic CRPS type II.

Dumbbell-shaped jugular foramen schwannomas: surgical management, outcome and complications on a series of 16 patients.

Schwannomas of the lower cranial nerves are very rare and the dumbbell-shaped ones are even rarer. The authors report their experience in managing such lesions, usually presenting either with intra- and/or extra-cranial extension through an enlarged jugular foramen. The juxtacondylar approach without sacrificing the labyrinth was used; clinical, radiological and complication features are discussed and analysed. This is a case series study on 16 consecutive patients with lower cranial nerves schwannomas surgically managed during a 14-year period using the juxtacondylar approach. In 13 cases, a complete resection has been achieved whereas in three, the excision was near total. The tumour nerve origin has been identified only in ten cases (62.5%). No death or additional post-operative cranial nerve deficits occurred. Aspiration pneumonia developed in one patient and cerebrospinal fluid leak in another. Pre-operative lower cranial nerve deficits improved in all patients. At a mean follow-up of 6.6 years (range 2-14 years), no radiological tumour recurrence was recorded amongst the patients having complete resection as well as no tumour progression in the group of near total removal. Jugular foramen schwannomas can be radically and safely resected with no additional neurological deficit if a careful pre-operative evaluation and the appropriate surgical approach is implemented. Finally, full cranial nerve functional recovery may be expected after complete resection.

Pediatric primary pilocytic astrocytoma of the cerebellopontine angle: a case report.

INTRODUCTION: We describe a rare case of pediatric pilocytic astrocytoma presented as a right cerebellopontine angle (CPA) mass, completely separated from the brain stem and arising from the proximal VIII nerve portion. CASE REPORT: A 12-year-old boy, with no evidence of neurofibromatosis type 2, presented with progressive hearing loss at the right ear and headache. An initial enhanced magnetic resonance examination suggested the diagnosis of schwannoma. The tumor was resected by a suboccipital retrosigmoid approach. DISCUSSION: Our case seems to be the first report of a primary pediatric CPA pylocitic astrocytoma arising from the VIII nerve complex and presenting internal auditory canal enlargement. It represents the third reported case of a primary CPA pilocytic astrocytoma (the second pediatric case with the first arising from V nerve) and the eighth report of primary CPA glioma, overall. We discuss the clinical, neuroradiological, and intraoperative findings, and we review the different hypothesis about the origin of these rare tumors.

A rare symptomatic presentation of ecchordosis physaliphora: neuroradiological and surgical management.

We report a case of ecchordosis physaliphora, an uncommon benign lesion originating from embryonic notochordal remnants, intradurally located in the prepontine cistern, that unusually presented associated with symptoms. MRI detected and precisely located the small mass. At surgery, a cystic gelatinous nodule was found ventral to the pons, contiguous with the dorsal wall of the clivus via a small pedicle. Histological examination diagnosed the lesion as an ecchordosis physaliphora. Here we focus on the analysis of the neuroradiological aspects that play a crucial role from both a diagnostic and a therapeutic standpoint.

Pathophysiology of the immune system in elderly subjects with or without diabetes and variations after recombinant interleukin-2.

This study reports two groups of elderly diabetic patients and normal subjects, with or without hypercholesterolemia and hypertriglyceridemia, who presented a decrease of the T lymphocyte-mediated function, proliferative capacity, phagocytosis, cytotoxicity and surface markers. This fall was more evident in hypercholesterolemic and hypertriglyceridemic subjects. The humoral responses and other parameters studied did not reveal significant variations. The authors also observed that recombinant interleukin-2 (rIL-2) stimulation determined a satisfactory response in healthy and diabetic subjects, while it did not normalize values in patients with altered lipid balance.