Cranial Nerve Impairment Research Articles

Continuous Intrathecal Administration of 5-Fluoro-2′-deoxyuridine for the Treatment of Neoplastic Meningitis

Previously, we reported a good clinical treatment effect of intrathecal chemotherapy by repeated bolus administration of 5-fluoro-2'-deoxyuridine (FdUrd) for neoplastic meningitis (NM). Moreover, we detected no side effects or neurotoxicity despite the long-term repetition of intrathecal administration. On the basis of these findings, continuous intrathecal chemotherapy (CIC) with FdUrd for patients with NM was attempted using a simple pump system. We evaluated the usefulness of CIC with FdUrd for the treatment of NM. A total of 25 patients were enrolled in this study. FdUrd (1.0 mg/d) was administered using a balloon pump system. CIC was continued as long as possible. Eight patients received whole-brain irradiation (3 Gy x 10) simultaneously with CIC. The effects of the treatment were analyzed in terms of improvement in neurological signs and symptoms and the findings of ventricular and lumbar cerebrospinal fluid analysis 2 and 4 weeks after CIC was initiated and on magnetic resonance imaging scans 2 months after CIC began. No apparent toxicity has been observed to date. Evidence of a cerebrospinal fluid response was observed in 13 patients. Headache and nausea were improved in all patients, and cranial nerve impairment was improved in 12 patients. A magnetic resonance imaging response was observed in only 5 patients. Overall response was observed in 15 patients when cases of stable disease were excluded from the responding cases. Survival time from the commencement of CIC (mean +/- standard error of the mean) was 255 +/- 30 days in 25 patients. This therapy may be useful, especially as a maintenance therapy for NM.

Imaging and management of head and neck paragangliomas

Paragangliomas of the head and neck are highly vascular lesions originating from paraganglionic tissue located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas). Diagnostic imaging can be considered in two clinical situations: (1) patients who present with clinical symptoms suggestive of a paraganglioma, and (2) individuals from families with hereditary paragangliomas. It is not only necessary to detect and characterize the lesion, but also to study the presence of multiplicity. For these purposes, MR imaging, and especially 3D TOF MRA, is the modality of choice. CT scanning is especially useful to show destruction of the temporal bone. Angiography in combination with embolization will mainly be used prior to surgical resection, but can also be used for diagnostic purposes when the diagnosis is not yet clear. Many parameters play a role in the decision to treat of which multifocality and impairment of cranial nerves are the most important. The primary therapeutic option for paragangliomas is complete excision of tumor with preservation of vital neurovascular structures. Resection however, should be balanced against a more conservative "wait and scan" policy or palliative treatments such as radiotherapy.

P-ANCA cranial pachymeningitis: a case report

Pachymeningitis is an inflammatory process that thickens the dura mater. This disease has various etiologies including infectious, neoplastic, or autoimmune diseases. We present the case of a patient who developed cranial pachymeningitis with a clinical and biological picture suggestive of a neurological form of vasculitis. A 51-year-old woman developed rhinitis, otitis media, headaches, and deterioration of her condition after a course of recombinant hepatitis B vaccine. After a booster dose of the vaccine, she developed unilateral visual loss and impairment of multiple cranial nerves. Blood analysis showed inflammation and presence of antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Cranial magnetic resonance imaging (MRI) showed pachymeningitis. A complete remission was obtained with immunosuppressive therapy. The initial clinical presentation and subsequent remission under immunosuppressive therapy were suggestive of a vasculitis with nervous system involvement. Though vasculitis was not proven histologically in this patient, we believe that MPO-ANCA-related autoimmunity provoked the patient's disease as already reported in similar cases. As pachymeningitis is a fibrosing process, early recognition and treatment of an autoimmune etiology, even in the absence of previous pulmonary or renal involvement, is required to prevent definitive neurological impairment.

Plasma cell myeloma of the skull base: Report of two cases

Plasma cell myeloma (PCM) of the skull base is rarely encountered in neurosurgical practice. PCM has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. We have described two patients with PCM of the skull base, of which one harbored SPC, and the other progressed to MM. In case 1, a 46-year-old man presented with left multiple cranial nerve impairments and had a large skull base tumor. Subtotal removal was performed. The specimen and general examination, including bone marrow aspiration, revealed SPC. Postoperatively 50Gy of external radiotherapy was administered. The patient has no manifestation of MM 24 months after the initial presentation. In case 2, a 53-year-old woman presented with left abducens palsy and had a left petroclival osteolytic mass. Gross total resection was performed. The specimen revealed a plasmablastic tumor, i.e., myeloma. General examination established the diagnosis of MM. She was administrated adjuvant chemotherapy and autologous bone marrow transplantation. She is alive without local recurrence 30 months after the initial presentation.

Giant olfactory groove meningiomas: advantages of the bilateral fronto-orbitonasal approach.

Olfactory groove meningiomas are usually considered surgically curable, but the operation becomes complex when the tumour reaches massive size and involves vital suprasellar structures, extends into the ethmoid sinuses or extensively involves the bony floor of the anterior fossa. The traditional bifrontal approach provides only limited exposure in these situations, and a bilateral fronto-orbitonasal approach may be more effective. We reviewed our experience in five patients with resections of massive olfactory groove meningiomas to assess the advantages and risks of these two approaches. All patients were women (mean age, 45 years; range, 28-59) presenting with symptoms and signs related to increased intracranial pressure, cranial nerve impairment or brain compression. Computed tomography, magnetic resonance imaging and cerebral angiography were used for diagnosis and operative planning. The approach was bifrontal in two patients and bifronto-orbitonasal in three. Total surgical removal (Simpson grade I, three patients; Simpson grade II, one patient) was achieved in four patients (80%) and subtotal tumour removal (Simpson grade III) was performed in one (20%). One patient had two staged operations. No morbidity or mortality occurred in this series. No clinical or radiologic evidence of recurrence developed in the four patients whose tumour was resected totally. The bilateral fronto-orbitonasal approach resulted in safe and successful total removal of giant olfactory groove meningiomas, achieving long-term prevention of recurrence. We highly recommend this approach. Copyright 1999 Harcourt Publishers Ltd.

Blink reflex discloses CNS dysfunction in neurologically asymptomatic patients with systemic sclerosis

Objective: To investigate trigeminal-nerve, brain-stem, and brain function, in order to disclose a possible nervous system involvement in neurologically asymptomatic systemic sclerosis (SSc) patients. Methods: Using a standard electromyographic (EMG) technique, we recorded the early (R1) and late (R2) components of the blink reflex in 35 SSc patients with no history or signs of cranial-nerve impairment and 20 control subjects. SSc patients were classified as limited or diffuse SSc and also evaluated for disease duration, autoantibody pattern (ANA, ACA, Scl70) and skin score (by Rodnan modified method). Results: Whereas no SSc patients had an abnormal R1, six (18%) had delayed R2 responses. We found no correlation between R2 latency and clinical or laboratory data. Conclusions: Whereas previous studies reported both R1 and R2 abnormalities (reasonably due to trigeminal neuropathy) in symptomatic SSc patients, we found selective abnormalities of the R2 components in asymptomatic patients. The selective R2 abnormality is secondary to a central dysfunction, either because of a direct impairment of the polysynaptic circuits in the medulla, or because of a decreased cortico-reticular drive on these circuits. The lack of overt trigeminal symptoms in our patients favours a suprasegmental dysfunction, possibly due to microvascular lesions disseminated in the subcortical white matter.

Continuous intrathecal chemotherapy with 5-fluoro-2'-deoxyuridine (FdUrd) in treatment of leptomeningeal carcinomatosis

1566 Background: Previously, we reported a good clinical treatment effect of intrathecal chemotherapy (ITC) by repeated bolus administration of 5-fluoro-2'-deoxyuridine (FdUrd) for leptomeningeal carcinomatosis (LMC). Moreover, there was no side effects and neurotoxicity detected despite the long-term repetition of intrathecal administration (IT). From these findings, continuous ITC with FdUrd for patients (pts) with LMC was attemted using a simple pump system. We evaluate the usefulness of continuous IT of FdUrd in treatment of LMC. Method: A total of 25 pts were enrolled in this study between January 2000 and April 2003. FdUrd 1.0 mg/day was administered using a balloon pump system. Continuous ITC was continued as long as possible changing the balloon pump containing FdUrd solution once every 7 days. Eight patients received whole brain irradiation (WBI, 3 Gy X 10 times) simultaneously with IT of FdUrd. The effects of the treatment were analyzed in terms of improvement in neurological signs and symptoms, the findings of ventricular and lumbar cerebrospinal fluid (CSF) after 2 and 4 weeks and MR images 2 months after continuous IT of FdUrd was initiated. Survival time was calculated as of August, 31, 2003. Results: No apparent toxicity has been observed to date. Evidence of CSF response was observed in 15 pts. Headache and nausea was improved in all pts and cranial nerve impairment was improved in 12 pts. MR image response was observed in only 2 patients. Overall response was observed in 15 pts when cases of stable disease were excluded from the responding cases. Survival times (mean ±sem days, n) were 251±30, 25. The survival times in pts with and without WBI were 182±37, 8 and 285±39, 17, respectively (p=0.057). Conclusion: This therapy may be useful, especially as a maintenance therapy of LMC, although WBI seems to cause adverse effects. No significant financial relationships to disclose.

Continuous intrathecal chemotherapy with 5-fluoro-2'-deoxyuridine (FdUrd) in treatment of leptomeningeal carcinomatosis

1566 Background: Previously, we reported a good clinical treatment effect of intrathecal chemotherapy (ITC) by repeated bolus administration of 5-fluoro-2'-deoxyuridine (FdUrd) for leptomeningeal carcinomatosis (LMC). Moreover, there was no side effects and neurotoxicity detected despite the long-term repetition of intrathecal administration (IT). From these findings, continuous ITC with FdUrd for patients (pts) with LMC was attemted using a simple pump system. We evaluate the usefulness of continuous IT of FdUrd in treatment of LMC. Method: A total of 25 pts were enrolled in this study between January 2000 and April 2003. FdUrd 1.0 mg/day was administered using a balloon pump system. Continuous ITC was continued as long as possible changing the balloon pump containing FdUrd solution once every 7 days. Eight patients received whole brain irradiation (WBI, 3 Gy X 10 times) simultaneously with IT of FdUrd. The effects of the treatment were analyzed in terms of improvement in neurological signs and symptoms, the findings of ventricular and lumbar cerebrospinal fluid (CSF) after 2 and 4 weeks and MR images 2 months after continuous IT of FdUrd was initiated. Survival time was calculated as of August, 31, 2003. Results: No apparent toxicity has been observed to date. Evidence of CSF response was observed in 15 pts. Headache and nausea was improved in all pts and cranial nerve impairment was improved in 12 pts. MR image response was observed in only 2 patients. Overall response was observed in 15 pts when cases of stable disease were excluded from the responding cases. Survival times (mean ±sem days, n) were 251±30, 25. The survival times in pts with and without WBI were 182±37, 8 and 285±39, 17, respectively (p=0.057). Conclusion: This therapy may be useful, especially as a maintenance therapy of LMC, although WBI seems to cause adverse effects. No significant financial relationships to disclose.

Meningiomas of the posterior petrous bone: functional outcome after microsurgery.

The aim of this study was to analyze a subgroup of patients harboring cerebellopontine angle meningiomas originating from the posterior petrous bone in regard to clinical presentation, surgical anatomy, complications, and long-term functional postoperative results. Data in a series of 51 patients with meningiomas of the posterior petrous bone who had undergone microsurgical treatment at the authors' institution between 1989 and 2002 were retrospectively reviewed. The patient population consisted of 46 women and five men with a mean age of 53 years (range 22-70 years). The main symptom on first admission was impaired hearing in 41%, dizziness in 20%, and tinnitus in 18% of the patients. Results of physical examination and audiological testing revealed hypacusis in 65% of patients, cerebellar ataxia in 31%, and impairment of the fifth cranial nerve in 26%. All patients underwent surgical treatment via a lateral suboccipital approach. Intraoperatively, the tumor was found to be attached to the postmeatal dura in 37%, the premeatal dura in 27.5%, the suprameatal dura in 19.6%, the inframeatal dura in 7.8%, and centered on the porus acusticus in 5.9% of cases. Tumor extension into the internal acoustic meatus was present in seven patients. Tumor resection was categorized as Grade I in 14 patients, Grade II in 29, Grade III in six, and Grade IV in two patients, according to the Simpson classification system. The site of displacement of the cranial nerves was predictable in up to 84% of patients, depending on the dural origin of the tumor as depicted on preoperative magnetic resonance (MR) imaging studies. Postoperatively, a new and permanent facial paresis was observed in five patients (9.8%). In 38 patients in whom both pre- and postoperative audiological data were available, hearing function deteriorated after surgery in 18.4% and improved in 7.9%. Clinical and MR imaging postsurgical data from a mean period of 5.8 years (range 13 months-13 years) were available in all patients. Forty-four patients (86%) resumed normal daily activity. Tumor recurrence was observed in two patients (3.9%), and both underwent a second surgery. Preoperative detailed analysis of MR imaging data gives the surgeon a clue about the dislocation of critical neurovascular structures, particularly the cranial nerves. Nonetheless, the exact relationship of the cranial nerves to the tumor (dislocation, adherence, infiltration, and splaying of nerves) can only be fully appreciated during surgery.

前下小脳動脈内耳道部動脈りゅうの２治験例と文献的考察

Aneurysms of the distal anterior inferior cerebellar artery (AICA) are very rare, occurring in 0.03-0.5% of cerebral aneurysms. We report 2 cases of distal AICA aneurysms at the internal auditory meatus. In 1 of them, the whole aneurysm was located in the internal acoustic meatus. Such an aneurysm is called an intrameatal aneurysm, which is an extremely rare type of distal AICA aneurysm with only 7 cases reported in the literature. Both of our patients presented with subarachnoid hemorrhage and were treated with early clipping surgery via the lateral suboccipital approach. The clipping was successful and they recovered uneventfully, but continued to have a hearing loss. We provide a review of the literature and a discussion of possible causes of 8th cranial nerve impairment in distal AICA aneurysms.

Ruolo della Neuroradiologia nella diagnostica della malformazione di Chiari di I tipo

Chiari's type I malformation is defined as the caudal displacement of the cerebellar tonsils into the occipital foramen, sometimes associated with displacement of the cerebellar vermis. Syringomyelia is present in 65% of patients with Chiari's type I malformation and in 25% of all cases of tonsillar ectopia detected by MR scan. In contrast, asymptomatic syringomyelia is sporadic. MR investigation of the whole spinal cord is indicated when Chiari's type I malformation is present. Although the incidence of symptomatic patients increases when the ectopia exceeds 5 mm, there are no statistically significant correlations between the size of cerebellar ectopia and the incidence of syringomyelia or the degree of other clinical manifestations such as headache, Ménière's syndrome and inferior cranial nerve impairment. Symptoms and hence prognosis and possible surgical treatment depend not only on the tonsillar displacement, but on its impact on CSF dynamics. Cine-MR scanning offers great potential in the assessment of CSF dynamics in the occipital foramen, revealing asymptomatic subjects at risk of developing syringomyelia. The surgical treatment of this category of patients remains a controversial topic.

Functionally essential neuronal population of the facial motor nucleus

Cranial nerve impairment is one of the more serious complications in neurosurgery. Nevertheless, the important question of how many neurons are required for cranial nerve functions remains unanswered. The VIIth cranial nerve (facial nerve) in mice was subjected to graded crush injuries. After assessment of the facial function, the number of uninjured, healthy facial motor neurons was quantified with a retrograde neuronal tracer. We report that normal facial function is preserved if intact neurons account for more than 56% of the control value, while complete facial paralysis occurs if intact neurons are reduced to less than 32% of the control value.

Paralisia bulbar progressiva juvenil doença de Fazio-Londe: relato de caso

Progressive bulbar palsy, also called Fazio -Londe disease, is characterized by progressive impairment of cranial nerves in children. It was first reported by Fazio in 1892 and until now only 30 cases have been published in the literature. Both sexes can be affected and clinical course can be divided on early (< 6 years age, predominance of respiratory symptoms) and late course (6-20 years of age, predominance of motor symptoms on superior limbs). We report a 4 years old boy that started with intense stridor and respiratory distress, initially being diagnosed as an acute asthma attack. Clinical signs worsened and 12 months latter he already had impairment of cranial nerves V, VII, VIII, IX and X confirmed by clinical examination and neurophysiological evaluation.

Management of Paragangliomas

Head and neck paragangliomas are highly vascular tumors with variable clinical behavior. The goal of this paper is to determine specific clinical and radiological findings and extract from these findings a treatment algorithm. Twenty-three patients with paragangliomas were referred from different surgical centers for angiography and pre-operative embolization. Clinical records were analyzed retrospectively, and focused on impairment of cranial nerves. Angiographic features of paragangliomas, such as arterial supply, tumor flow characteristics, and venous drainage, were evaluated to find characteristic angioarchitectural patterns. All but one patient presented with a single tumor. All eight jugular and four of five vagal paragangliomas caused a lower cranial nerve deficit. Tympanic paragangliomas presented with hearing loss and tinnitus. The ascending pharyngeal artery was the main feeder and contributed to the supply in every tumor. Jugular and vagal paragangliomas typically compromise the patency of the jugular vein with resulting antegrade or retrograde flow through collateral venous systems. Surgical resection of vagal and jugular paragangliomas was especially performed when unifocal paragangliomas were present. In all of these patients, the tumor caused a cranial nerve deficit. The supply from an enlarged ascending pharyngeal artery is typical for paragangliomas. The venous drainage pattern of jugular and vagal paragangliomas allows differentiation from other vascular lesions at the skull base.

Abducens nerve schwannoma: A case report and review of the literature

BACKGROUND Schwannomas of the abducens nerve are extremely rare tumors; only 10 cases have been reported so far. The tumor may be located within the cavernous sinus (CS) or more commonly at the prepontine region. Patients usually present with VIth cranial nerve paresis. CASE DESCRIPTION We report a case of a 42-year-old male patient who presented with headache, vertigo, and hearing deficit, but no symptoms or signs related to a VIth cranial nerve impairment. Computed tomography and magnetic resonance imaging showed a large mass with a ring-like contrast enhancement in the prepontine area. Surgical treatment, via a simple retrosigmoid suboccipital approach, revealed a schwannoma originating from the VIth cranial nerve at its prepontine portion. Postoperative histological examination confirmed the diagnosis of schwannoma, and the patient had only a VIth cranial nerve palsy, which resolved during long-term follow-up. CONCLUSIONS Abducens schwannomas are rare tumors, representing less than 1% of all intracranial schwannomas in our clinic and about 13% of oculomotor nerve schwannomas reported in the literature. These lesions can be mainly divided into tumors originating from the intracavernous portion of the VIth cranial nerve or more commonly from the cisternal part of the nerve, leading to a lesion in the prepontine region. In such cases, with no invasion of the cavernous sinus, radical tumor resection with preservation of the abducens nerve can be performed via a simple suboccipital retrosigmoid approach.

Long-term oncological results in 47 cases of jugular paraganglioma surgery with special emphasis on the facial nerve issue.

Oncological and functional results were assessed in 47 type C and/or D jugular paraganglioma operated on between 1984 and 1998 using the classical infratemporal fossa type A approach (mean follow-up = 66 months). In 24 instances, however, the facial nerve was not re-routed. Total resection was achieved in 33 cases (70 per cent). In 25 patients available for follow-up this resulted in a 92 per cent cure rate while two patients (eight per cent) developed recurrences that are being followed-up clinically and radiologically. Sub-total resection, leaving infracentimetric tumour remnants after being coagulated, was achieved in 14 cases (30 per cent). In 11 patients available for follow-up, only three cases developed tumour regrowth (27 per cent) that was controlled by salvage irradiation or surgery while in the other cases tumour remnants remained stable (73 per cent). Symptomatic post-operative lower cranial nerve impairment was observed in 23 per cent. When results were analysed depending on whether the facial nerve had been re-routed (n = 18) or not (n = 24), the incidence of facial paralysis HB grade III or more at one year was 33 per cent and eight per cent, respectively. Total resection was achieved in 56 per cent when the facial nerve was re-routed versus 75 per cent when it was not, the difference being due to a higher incidence of large tumours in the first group. The present study suggests that: 1) surgical resection of jugular paraganglioma provides overall satisfactory results, i.e. a 86 per cent rate of either cure or tumour remnant stabilization, but carries a significant risk of iatrogeny; 2) complete tumour removal should not be attempted, especially in patients over 60 years of age with no pre-operative neurological deficits, since leaving infracentimetric tumour remnants has no major detrimental effect on the final outcome; 3) facial nerve transposition carries a significant risk of cosmetic sequelae while it does not provide significant advantages in terms of tumour resection and long-term oncological control.

Surgical management of brainstem cavernomas.

A careful retrospective analysis of 36 cases was performed to evaluate the pre- and postoperative rates of morbidity that occur in patients with brainstem cavernous angiomas. The authors evaluated immediate postoperative and follow-up outcomes with regard to clinical findings, the incidence of preoperative hemorrhage(s), location and size of the lesions, and the timing of the surgical procedure after the last hemorrhagic event. Specifically. the following parameters were analyzed: 1) number of hemorrhages; 2) the precise brainstem location (pontomesencephalic, pons, and medulla oblongata); 3) pre- and postoperative cranial nerve status; 4) pre- and postoperative motor and sensory deficits; 5) size (volume) of the lesions; and 6) pre- and postoperative Karnofsky Performance Scale (KPS) scores. Multiple hemorrhages were observed in 16 patients, particularly in those with pontomesencephalic cavernous angiomas (75%). The mean preoperative KPS score was 70.3 +/- 16.3 (+/- standard deviation). Twenty-six patients (72.2%) presented with cranial nerve impairment, 13 (36.1%) with motor deficits, and 17 (47.2%) with sensory disturbance. Volume of the lesions ranged from 0.18 to 18.18 cm3 (mean 4.75 cm3). Postoperative complications included new cranial nerve deficits in 17 patients, motor deficits in three, and new sensory disturbances in 12 patients. In a mean follow-up period of 21.5 months, KPS scores were 80 to 100 in 22 patients. Timing of surgery (posthemorrhage) and multiple hemorrhages did not influence the long-term results. Higher preoperative KPS scores and smaller-volume lesions, however, were factors associated with a better final outcome (p < 0.05). Major morbidity was related mainly to preoperative status and less to surgical treatment. The incidence of new postoperative cranial nerve deficits was clearly lower than that demonstrated preoperatively because of the brainstem hemorrhages. Based on these findings, resection of brainstem cavernomas is the treatment of choice in the majority of these cases because of the high incidence of morbidity related to one or often several brainstem hemorrhages.

Spontaneous Healing and Complete Disappearance of a Giant Basilar Tip Aneurysm in a Child

We report a rare case of spontaneous total thrombosis of a giant basilar tip aneurysm resulting in compression of the brainstem, diagnosed in a two-year-old child who presented with neurological deficits and third cranial nerve impairment. After conservative treatment, the giant aneurysm was completely thrombosed and the clinical symptoms were remarkably improved. MRI demonstrated dramatic shrinkage and ultimately complete disappearance of the giant aneurysm at seven month follow-up.

Ocular and clinical manifestations of Möbius' syndrome.

To assess ocular and otorhinolaryngologic manifestations and intellectual ability in patients with Möbius' syndrome. Patients with Möbius' syndrome underwent prospective ophthalmic, genetic-clinical, and otorhinolaryngologic examinations as well as psychological evaluation. Sixteen patients with Möbius' syndrome between the ages of 8 months and 10.6 years underwent ocular examination. Esotropia was present in 12 (75%) patients and V-pattern in 8 (50%). Limited abduction was present in 30 (93.8%) eyes, and limited adduction was present in 21 (65.6%) eyes. The most frequent refractive error was compound hyperopic astigmatism (13 [40.6%] eyes). Eleven (68.8%) patients had lagophthalmos and 12 (75%) patients had bilateral epicanthus. Unilateral amblyopia was present in 2 (12.5%) patients. Clubfoot was the most common lower limb defect (7 [43.8%] patients). Cranial nerve impairments included paralysis of 7th nerve in all patients, paralysis of the 12th nerve in 13 patients, and paralysis of the 9th and 10th nerves in 3 patients. Evaluation of intellectual ability showed that 4 (25%) patients had normal intelligence. The mothers of 3 (18.8%) patients used misoprostol during the first trimester of pregnancy. Prominent ophthalmic features of Möbius' syndrome in this series were esotropia, V-pattern, abduction limitation, and compound hyperopic astigmatism. Intellectual assessment showed some degree of mental retardation in 75% of patients. Möbius' syndrome is associated with prenatal exposure to misoprostol.

Cranial chordomas: results of radical excision

The authors analyze their experience with the treatment of 29 patients who underwent radical excision of skull base chordomas. Modern skull base surgical techniques were used in all patients who were treated between August 1991 and July 2000. The degree of tumor resection was gauged according to intraoperative inspection and postoperative high-resolution imaging findings. There were 21 patients with primary disease and eight with recurrent disease. Total resection was accomplished in 18 patients. Five patients had undergone radiotherapy prior to the present surgery, and an additional eight patients underwent postoperative radiotherapy. There were no surgery-related deaths. In five patients who died of the disease, surgery and radiotherapy had failed to effect a cure. Two of the remaining patients are alive with recurrent disease, and there is questionable evidence of recurrence in another patient. All 24 patients are functioning independently. Cranial nerve impairment was the most common postoperative deficit, followed by cerebrospinal fluid (CSF) leakage and infection. The use of skull base techniques in radical surgery provides an opportunity to excise the tumor and the involved bone. In most cases the procedure-related cranial nerve deficits improve over time. The complications of CSF leakage and infection can be minimized and are preventable. Proton beam irradiation is an excellent adjuvant treatment but is reserved for patients with definite tumor recurrence or residual tumor that can be identified on the imaging studies.