Acute Korsakoff-like amnestic syndrome resulting from left thalamic infarction following a right hippocampal hemorrhage.

Korsakoff-like amnestic syndromes have been rarely described following structural lesions of the central nervous system. In this report, we describe a case of acute Korsakoff-like syndrome resulting from the combination of a left anteromedian thalamic infarct and a right hippocampal hemorrhage. We also review the literature relevant to the neuropathology and pathophysiology of Korsakoff syndrome and anterograde amnesia.

Use of desmopressin as an alternative to corticotropin-releasing hormone during inferior petrosal sinus sampling in a child with Cushing's disease.

Cushing's syndrome (CS) is rare in children. Information on bilateral inferior petrosal sinus sampling (BIPSS) in children with CS is limited. In the procedure CRH is always used to stimulate ACTH values. In addition, growth failure is the main complication of pediatric CS, mainly due to a profound GH suppression that persists for a few months after cure of the disease. Early treatment with recombinant GH after CS cure may partly reverse this phenomenon. We report herein a case of Cushing's disease (CD) in a 7-yr-old child, presenting with severe growth failure. No pituitary adenoma was shown on magnetic resonance imaging and a BIPSS using desmopressin allowed the identification of a central to peripheral (C/P) gradient; however transphenoidal surgery (TSS) did not cure the disease thus requiring the performance of bilateral adrenalectomy. After cure of the disease, a partial catch up of the growth delay occurred without any GH treatment. Our case reinforces the fact that BIPSS can be performed safely in very young children with CD. It also suggests for the first time that the use of desmopressin during the procedure gives the same information as CRH, as well as confirming the fact that the success of TSS is poor in very young children. Finally, it suggests that growth failure in children with CS can be partially reversed after surgical cure of the disease without any GH treatment and that the high IGF-I observed during corticosteroid replacement therapy is due to a state of IGF-I resistance.

Elephantiasis neuromatosa in neurofibromatis type I. MRI findings with review of the literature.

We report the case of a patient with NF-1 who presented with gross elephantiasis neuromatosa of her right leg. Prior to plastic surgery, Magnetic Resonance Imaging and Angiography (MRI and MRA) were performed to provide a detailed assessment of the extension as well as the vascular and muscular involvement of the neurofibroma.

[Megalencephalic leucoencephalopathy with subcortical cysts: a study of a Lebanese family and a review of the literature]. / La leucoencéphalopathie mégalencéphalique avec kystes sous-corticaux: étude d'une famille libanaise et revue de la littérature.

INTRODUCTION: Megalencephalic leukoencephalopathy with subcortical cysts is a rare disease with autosomal recessive inheritance. MATERIALS AND METHODS: Two brothers born from a consanguineous marriage, presenting with the phenotype of the disease, their parents, brothers and sisters were examined. Magnetic resonance imaging of the brain was performed for the two patients. Sequence analysis of MLC1 (GenBank mRNA accession no. NM_OI5166) was performed for the patients using intronic primers. PCR restriction fragment length polymorphism analysis was done in patients, their parents and in 100 Lebanese controls in order to exclude gene polymorphism. RESULTS: The clinical features were characteristic of the disease, consisting of an early-onset macrocephaly followed by slowly progressive ataxia, pyramidal tract involvement and epileptic seizures. In one patient, the clinical manifestations were aggravated by a trivial brain trauma. In his brother and in one female cousin, a status epilepticus was precipitated by a febrile syndrome. The diffuse cerebral white matter lesions and the subcortical temporo-polar and frontal cysts, best seen on MRI, allowed making the diagnosis. Molecular genetics revealed a new mutation involving the MLC1 gene (263G-->T, exon 3). As a consequence, it affects the second transmembrane domain predict (G88V) of the MLC protein (protein sequence NP_055981). The mutation was confirmed by PCR restriction fragment length polymorphism analysis. CONCLUSION: Megalencephalic leucoencephalopathy with subcortical cysts may be individualized on clinical and radiological basis and confirmed by molecular genetics. In this Lebanese family, a new mutation of the MLC1 gene is reported.

Frozen shoulder.

The term Frozen Shoulder (FS) is a medical colloquialism rather than a diagnosis. It is usually used as a clinical description with pathogenetic inferences, as suggested by the alternative designations of periarthritis, pericapsulitis, adhesive capsulitis and obliterative bursitis. Our understanding of the basic pathology and natural history of FS is limited, and this is reflected in the wide assortment of treatments which have been advocated. In this review the present state of knowledge of this disorder will be presented in a critical fashion.

The cortical vibrissae representation is normal in transgenic mice lacking the 5-HT(1B) receptor.

Recent studies have suggested that 5-HT may modulate thalamocortical development in somatosensory cortex (S-I) of rats and mice, and that the 5-HT(1B) receptor may play a critical role in this process. Analysis of CO-stained sections through lamina IV of S-I in perinatal and adult 5-HT(1B) knockout mice revealed a normal vibrissae-related pattern, indicating that activation of the 5-HT(1B) receptor is not necessary for the normal development of the vibrissae representation in S-I.

Are the calcium antagonists really useful in cerebral aneurysmal surgery? A retrospective study.

From 1983 to 1990, 234 patients with one or several cerebral arterial aneurysms were surgically treated in our department. Since 1983, we have been performing surgery as early as possible. As soon as the subarachnoid hemorrhage diagnosis is confirmed by computed tomography (or if unconfirmed, by lumbar puncture), we assume that each patient may have an aneurysm. Between 1987 and 1990, 111 patients were treated by vascular volume expansion (maintenance of central venous pressure above 5 cm H2O with 4% albumin or Ringer-lactate or, if necessary, with 20% albumin), which we supplemented with calcium antagonists (nimodipine in 60 patients and nicardipine in 51 patients). Two months after being discharged, each patient is examined by a neurosurgeon and, on the same day, is subjected to a neuropsychological evaluation and a computed tomographic scan of the brain. A few months after this consultation, a working-position/family-activities questionnaire is issued to the patient. All of the results studied on the basis of postoperative mortality, second-month computed tomographic scan ischemia, neuropsychological evaluation, and return to work show no significant difference between the groups with or without calcium antagonists or between the nimodipine and nicardipine subgroups.

Using a saphenous vein branch for a patch.

The majority of vascular surgeons find that the autologous saphenous vein is the conduit of choice for femoropopliteal and femorotibial bypasses. The branches of the saphenous vein are eventually ligated but can serve several useful purposes, such as completing an angiogram, passing a valvulotome, or widening the anastomosis. Another use for the branches consists of using the branch as a patch when the vein needs to be reopened, i.e., for thrombectomy, during the postoperative period. This is done by making a venotomy adjacent to a branch. The surgical technique, as practiced by us, is described and illustrated by a schematic diagram.

Pitfalls in the detection of lipid vectors in neural cell culture and in brain tissue.

Lipid particles (liposomes and lipid-coated microbubbles) are currently studied as vectors for drug delivery to the central nervous system. The visualization of these particles is usually based on their labeling with a lipophilic fluorescent dye (3,3'-dioctadecycloxacarbocyanine perchlorate) or staining with Oil Red O. The purpose of this article was to highlight the difficulties and pitfalls encountered with the use of these techniques in the detection of lipid particles in neural cell cultures and in brain tissue. In vitro and in vivo studies were conducted on different neural cell cultures (rat and human tumors, microglial cells) and animal models of brain lesion (lipopolysaccharide and quinolinic acid-induced lesion, induced brain tumor). The cells or brain slices were observed with optical microscopy after staining with Oil Red O, fluorescent microscopy, or scanning electron microscopy. Intra and extracytoplasmic lipid particles (stained with Oil Red O or autofluorescent or visualized by scanning electron microscopy) were naturally found in the cells and tissues studied. Intracytoplasmic lipid microparticles were present in tumoral and microglial cells. These lipid microparticles were also observed with some extracytoplasmic lipid droplets in the induced brain lesions. These images could be misinterpreted as lipid vectors if the cells or animals would have been treated with such a vector.

[Tramadol-induced epileptic seizures]. / Crises d'épilepsie induites par le tramadol.

A previously non-epileptic 17-year-old patient presented with 2 generalized epileptic seizures, which occurred at 6 months of interval, following the oral intake of 200-250 mg of tramadol. Urine analysis showed only the product and its metabolites. Epileptic seizures induced by tramadol and high risk factors are described and discussed.

[Frontal pseudo-tumoral form of adrenoleukodystrophy]. / Adrénoleucodystrophie à forme frontale pseudo-tumorale.

A case of adrenoleukodystrophy in a 9-year old boy revealed by a predominant frontal syndrome is reported. Brain MRI showed an unusual pseudo-tumoral frontal lesion. The diagnosis was confirmed by increased plasma levels of very long chain fatty acids. His young brother had an isolated adrenal insufficiency with normal brain MRI. The frontal predominance of the lesion and the clinical polymorphism of the disease in this family are discussed.

[Vacuolar leucoencephalopathy induced by heroin: 4 cases]. / La leucoencéphalopathie vacuolaire induite par l'héroïne: 4 cas.

We report 4 cases of toxic leucoencephalopathy after heroin inhalation. The clinical features, which usually occur some days or even longer after the last heroin consumption, are dominated by a cerebellar syndrome. The cerebellar hemispheres are almost always affected; the cerebral hemispheres, the cerebellar peduncles and the pyramidal tract may be affected. Vacuolar demyelination is the morphological substract of the lesions, which are symmmetrical, not contrast enhancing, hypodense on CT scan and hyperintense on T2-weighted MRI. The pathophysiology is unknown and seems different from post-anoxic leucoencephalopathy. The disease is usually progressive leading sometimes to death, but some cases show slow recovery.

[Cerebrovascular reactivity to CO2 during general anesthesia maintained with either isoflurane-N2O or propofol-N2O. A comparative study by transcranial Doppler velocimetry]. / Réactivité vasculaire cérébrale au CO2 sous anesthésie générale entretenue par isoflurane-N2O ou propofol-N2O. Etude comparative par vélocimétrie Doppler transcrânienne.

OBJECTIVES: To compare, using transcranial doppler velocimetry (TDV), the cerebral blood flow velocity and CO2 reactivity during general anaesthesia maintained with either isoflurane-N2O-O2(IF) or propofol-N2O-O2 (PF) in adults with a normal brain. STUDY DESIGN: Nonrandomized controlled trial. PATIENTS: Forty ASA I patients (mean age 41 +/- 13 yrs, 15 F/35 M) undergoing surgery of the lumbar spine in prone position. The first 20 were allocated into the IF group and the subsequent 20 into the PF group. METHODS: General anaesthesia was induced with midazolam (0.05 mg.kg-1), phenoperidine (0.03 mg.kg-1), thiopentone (5 mg.kg-1), vecuronium (0.1 mg.kg-1) and maintained with N2O (50 vol %) and O2 (50 vol %) and either isoflurane (0.8 < Fet < 1 vol %) in IF group or propofol (6 mg.kg-1.h-1) in PF group. The vascular reactivity was assessed with velocimetry measurements (Angiodine DMS, with a probe transmitting a 2-MHz pulsed wave) of flow in the middle cerebral artery at a given PetCO2 (obtained by adjustments of VT) during systole (SV) and diastole (DV). Three measurements were made: at T1 (PetCO2 = 30 +/- 2 mmHg), at T2 (PetCO2 = 40 +/- 2) and at T3 (PetCO2 = 30 +/- 2 mmHg). RESULTS: In the IF group, VS increased by +32% at T2 (P = 0.006) with an increase of + 3.4 %/1 mmHg of PetCO2. Similarly, in the PF group VS increased by + 31 % at T2 (P < 0.0001) with an increase of 2.9 %/1 mmHg of PetCO2. In both groups the VS returned to baseline values at T3. In the IF group, VD increased by + 66% at T2 (P < 0.0001), with an increase of + 7%/1 mmHg of PetCO2. Similarly in the PF group, VD increased by + 61% (P < 0.0001) with an increase of + 5.7%/1 mmHg of PetCO2. In both groups the VD returned to baseline values at T3. CONCLUSIONS: During anaesthesia maintained with either isoflurane-N2O-O2 or propofol-N2O-O2, a change in PetCO2 results in similar changes in VS and VD. These anaesthetic agents preserve the cerebrovascular reactivity of the normal brain. The results of this study are in accordance with those obtained with other reference techniques in healthy volunteers. Transcranial doppler velocimetry can be a useful noninvasive tool of clinical research in neuroanaesthesia.

[Infections after missile head injury. Experience during the Lebanese civilian war]. / Infections et plaies crAnio-cérébrales par arme à feu.

OBJECTIVE: Establish the risk factors for infection following missile head injuries (MHI). METHODS: Between 1975 and 1990, 500 cases of MHI were admitted, with only 272 responding to inclusion criteria. After initial evaluation including CT scan for 177 patients, all underwent craniectomy with debridement and duroplasty. A retrospective study was undertaken in order to identify the risk factors that increase the infection rate. RESULTS: The global infection rate was 11.39%. Among the studied factors, those increasing the infection rate were: coma on admission (17.6% vs 7.6%), penetrating wounds (12.93% vs 7% for tangential wounds), intracerebral trajectory length over 6 cm (18.42% vs 6.32%), air sinuses effraction (25.8% vs 9.54%), a surgical delay over 72 hours (41.6% vs 10.6%), inadequate duroplasty (28% vs 7.33%), cerebrospinal fluid (CSF) fistulae (58.62% vs 5.76%). The presence of postoperative bone fragments did not increase the infection rate (11.4% vs 11.2%). DISCUSSION AND CONCLUSION: Adequate duroplasty and aggressive treatment of CSF fistulae decrease the infection rate. There is no need to reoperate on residual bone fragments after adequate debridment. A delay of 24 to 48 hours should be considered, to facilitate the procedure without increasing the infection risk.

[Temporary occlusion in surgical management of intracranial aneurysm. Report of 54 cases]. / Le clampage temporaire réglé dans la chirurgie anévrismale intracrânienne. A propos de 54 cas.

Temporary arterial occlusion (TAO) is commonly used in the surgery of intracranial giant aneurysms. Its usefulness and safety in the surgical management of all cases of aneurysms remains to be proved. We report a series of 54 patients operated on for an intracranial aneurysm with the use of TAO. Among the 27 patients, admitted before the 4th day following post subarachnoid hemorrhage with I or II on WFNS score clinically, 24 had early aneurysm surgery. The size of the aneurysm was small in 16 cases, medium in 22, large in 13 and giant in 3 cases. The protocol proposed by Batjer in 1988 for large and giant aneurysms (etomidate, normotention and hypervolemia) was used without any electrophysiological monitoring. All patients underwent a post-operative cerebral CT scan to evaluate the incidence of a cerebral ischemia. Serial transcranial doppler was used to evaluate the severity of vasospasm. Clinical results were assessed using the GOS. TAO was elective in 51 patients and done after peroperative aneurysm rupture in 3 patients. The duration of TAO was less than 5 mn in 25 patients, between 5 and 10 min in 12, between 10 and 15 in 11, between 15 and 20 in 5 and more than 20 min in one patient. The last one developed a reversible neurological deficit secondary to ischemia attribuated to TAO. Intracranial aneurysm peroperative rupture was noted in 3 patients, clinical vasospam in 13 patients. These results allow us to recommend the routine use of TAO in the surgery of intracranial aneurysm. When application time is limited and cerebral protection used, TAO is safe. It decreases the risk of intraoperative rupture from a 18% rate in literature to 4.2% in our present experience and the risk of symptomatic vasospasm is not increased.

[Growing fracture of the orbital roof. A case report]. / Fracture évolutive du toit de l'orbite.

We report a case of growing fracture of the orbital roof in a 5-year-old child. The presenting sign was a pulsatile orbital mass. This child had a history of a minor head injury with orbital impact 2 years ago. Cerebral CT scan revealed a diastatic fracture of the right orbital roof. On MRI a leptomeningeal cyst extending in the orbital cavity was shown. Frontal craniotomy with direct repair of the dural and bone defects was performed. The outcome was excellent. In the literature the exact pathophysiology of the growing fractures is still debated but a dural laceration along the fracture line is noted in all the cases. They are mostly located in the cranial convexity, and rarely affect the skull base. Only 5 similar cases were found in the relevant literature. Growing fracture of the orbital roof should be suspected if ocular symptoms appears in childs who have sustained a head injury several months or years ago.

[Giant ependymoma of the cauda equina. Long-term development apropos of 7 cases]. / Les épendymomes géants de la queue de cheval. Evolution à long terme. A propos de 7 cas.

Seven cases of giant ependymomas of cauda equina, extending from the conus medullaris to the sacrum, are reported. Mean age of the patients was 36 years. The initial symptoms were not specific, including low back pain and radiculalgias. However, in two cases, the clinical presentation was related to hydrocephalus. At the time of the operation, 6 patients presented with pain, weakness and sphincter dysfunction. The diagnosis of tumor was made after myelogram in all cases. Metrizamide enhanced CT scan has been performed in one case. None patient had preoperative magnetic resonance imaging (MRI) but MRI allowed the follow up in 5 patients and detection of local and remote recurrences in 4 cases. All the tumors were attached to the filum terminale, and unless extended from L2 to S1. Pathologically, 3 patients had tumors classified as myxopapillary and 4 of the cellular type. Two patients had gross total resection of the tumor at the initial operation. Five patients had initial biopsy to make a diagnosis and required subsequent surgery for radical excision after an average of 16.8 months. Radiation therapy has been performed in 1 case after biopsy, in 1 case after total resection (grade III) and in 2 cases after recurrences. Three patients died 1 year, 5 years, 12 years post-operatively. Among these 3 patients, 2 had recurrences. Four patients are alive. One patient has no recurrence 5 years after initial surgery. Another patient remains symptom free 7 years after surgery but MRI showed a local recurrence. The last two patients present recurrences 7 years and 8 years after surgery. These recurrences have been treated by surgical removal.(ABSTRACT TRUNCATED AT 250 WORDS)

[Are calcium channel blockers really useful in cerebral aneurysm surgery?]. / Les anticalciques sont-ils réellement utiles en chirurgie anévrysmale cérébrale?

Two retrospective series of early operated cerebral arterial aneurysms are compared. One series involves 74 patients treated from 1983 to 1987 by vascular volume expansion only. The other series involves 75 patients treated from 1987 to 1990 by vascular volume expansion associated by calcium antagonists (nimodipine or nicardipine). The conclusion is that in our experience, calcium antagonists do not improve the outcome (mortality, cognition findings and return to work) obtained by vascular volume expansion alone. Moreover they put forward that there is no difference between the patients treated by nimodipine and those treated by nicardipine.

[Malignant astrocytoma of the cerebellum. Apropos of 10 cases. Review of the literature]. / Astrocytomes malins du cervelet. A propos de dix cas. Revue de la littérature.

While reviewing a series of 138 cerebellar tumors operated upon between 1978 and 1991, the authors could only find 2 glioblastomas and 8 anaplastic astrocytomas, occurring in 4 children (3 to 14 years old) and 6 adults (23 to 48 years old). These 10 cases represent 2% of the all malignant gliomas population observed during the same period of time. Clinically speaking, nothing makes these tumors different from other cerebellar ones. However, with an heterogenous image and an irregular contrast enhancement, the CT (scan) appearance can lead to the diagnosis. 7 lesions develop within the cerebellar vermis (vermis cerebelli) and 3 develop within the cerebellar hemisphere. Total surgical resection is performed in 9 cases and subtotal resection in 1 case (because of the extension to the floor of the fourth ventricle). Adjunctive radiotherapy on their posterior cranial fossa is achieved in 8 cases. The 2 patients with a glioblastoma present with a recurrence of their tumor at 15 months and 6 years respectively, and eventually died. Out of the patients with an anaplastic astrocytoma, 4 are still alive without recurrence and with a median follow-up of 7 years. The pathogenesis of such lesions is discussed. An aggressive therapeutic management is suggested because of the possible prolonged survival rate.