A family of von Recklinghausen's disease associated with bilateral acoustic neurinomas and falx meningiomas

A familial or inherited occurrence of von Recklinghausen's disease has been reported hitherto, some of which were associated with intracranial tumors such as gliomas, neurinomas, meningiomas or others. Authers report a family (case 1: father, case 2: son, case 3: daughter) of von Recklinghausen's disease associated with both bilateral acoustic neurinomas and falx meningioma. Case 1. A 41 year-old male was admitted to our hospital with complaints of bilateral hearing disturbance, headache and loss of visual acuity. On admission, neurological examination revealed left papilledema, right cataracta, impairment of bilateral cranial nerves V, IX, X, bilateral deafness, cerebellar ataxia, motor weakness of lower extremities and sensory disturbance of peripheral region of extremities. Twelve subcutaneous nodules were noted over the entire body and Café-au-lait spots were found slightly to be scattered over the chest and the back. Craniogram and vertebral angiography demonstrated bilateral CP angle tumors, and the diagnosis of bilateral acoustic neurinomas was made. Subtotal removal of bilateral tumors and Torkildsen's shunting were carried out. Histological diagnosis was neurinoma. Post-operative course was good. But 6 years later, he complained of gait disturbance. Computed tomography revealed falx meningioma in the frontal lobe and recurrence of bilateral acoustic neurinomas. So meningioma was totally removed. Eight months after surgery he died of aspiration pneumonia aged 47. Case 2. A 15 year-old male, son of case 1, was admitted with complaints of bilateral deafness, gait disturbance and visual disturbance. Neurological examination disclosed left papilledema, right congenital cataracta, impairment of bilateral cranial nerves V, IX, X, ataxic gait and sensory disturbance of right lower extremity.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cell kinetics of the malignant evolution of meningothelial meningioma

Summary A meningotheliomatous meningioma occurred in the right parietal parasagittal region of a 43-year-old woman. A total excision was carried out. Three years and 9 months later, one parasagittal and two falx tumours in close proximity recurred and were excised. The falx tumors showed the features of an anaplastic transitional meningioma with increased mitoses. The falx meningioma recurred locally and repeatedly, despite radiation therapy and chemoimmunotherapy. She died 7 years and 8 months after the onset. At autopsy, the tumor was found to have metastasized via the cerebrospinal fluid to the spinal cord and pituitary stalk. Extracranial metastases were not evident. At the fourth craniotomy, bromodeoxyuridine (BrdU) was administered intravenously to label tumor cells in the S phase of the cell cycle. The BrdU-labeled cells accounted for 9.0%; they are usually less than 1% in benign meningiomas. Meningothelial meningiomas with a high labeling index recur rapidly, even after Simpson's grade 2 removal, and treatment for a malignant meningioma should be instituted.     

Tumor‐to‐tumor metastasis to the central nervous system

Tumor‐to‐tumor metastasis is a well‐recognized phenomenon. Meningioma is the most common intracranial host tumor, with the breast and lung being the most common primary sites. We report herein two such cases of metastasis from pulmonary adenocarcinoma and malignant melanoma (MM) of vulva, respectively. Case 1: a 69‐year‐old female smoker who had a history of right upper lobectomy of lung for adenocarcinoma presented with a headache and altered mental status, and was found to have a left temporal contrast‐enhancing mass with massive surrounding edema on MRI. The resection specimen revealed foci of metastatic adenocarcinoma within a microcystic meningioma. Case 2: a 75‐year‐old woman with a history of radical vulvectomy for MM died of widespread systemic metastasis of MM. At autopsy, a 2.5 × 2 × 2 cm firm nodule attached to the falx was incidentally found, with focal black discoloration at the periphery of the mass. Histologic examination showed a fibroblastic meningioma with a focus of metastatic MM. Case 1 is the first case report describing a microcystic variant of meningioma harboring metastatic carcinoma. Although MM is one of the most common metastatic brain tumors, MM‐to‐meningioma metastasis is reportedly extremely rare, but can occur.     

Hoffmann reflex from foreleg flexor nerves in cats: lateralization, picrotoxin, strychnine, crossed flexor reflex

Hoffmann (H) reflexes from foreleg flexor nerves were studied in cats. The right and left flexor nerves were stimulated and H reflexes were recorded from the same nerves. Paw preference was assessed by a food reaching test. Stimulation of the right median nerve elicited mono- and polysynaptic reflexes from the left ulnar nerve (crossed flexor nerve). Picrotoxin depressed, and strychnine increased H reflexes from both sides without affecting the spinal motor asymmetry. H reflexes were found to be larger on the left than the right side in right-preferent cats and vice versa in left-preferent cats. The right H-reflex recovery curve was higher than left in right-preferent cats. The inhibitory period of the recovery cycle disappeared after picrotoxin and changed to facilitation for the nonpreferred side. Strychinine caused bilateral, nearly-synchronous motoneuronal discharges from the right and left flexor nerves; the discharges originating from the left side preceded those from the right side in a right-preferent cat. These results indicate that spinal motor activity predominates on the nonpreferred side, which would be a prerequisite for postural adjustments during paw use in cats. This asymmetric motor organization in the forelegs of cats having quadrpedal locomotion seems to be similar to asymmetric motor organization in legs in humans.     

Familial syringomyelia: the first Japanese case and review of the literature

A 47-year-old woman with a 3 month's history of progressive visual loss in her right eye presented with right fundus normal but with marked papilledema on the left. Magnetic resonance imaging (MRI) revealed a large right 'en plaque' meningioma which was successfully removed via a pterional craniotomy. The case outlines that unilateral papilledema offers little information concerning tumor localization, and may represent a 'false' localizing sign.     

Syringomyelia associated with intracranial tumors. Case report and literature review

A case of 39 years old woman with two intracranial meningiomas and syringomyelia is presented. Large right-sided tentorial meningioma in cerebellopontine angle and middle cranial fossa and small left sided sphenoid wing meningioma co-existed with secondary tonsillar herniation and large syringomyelic cavity in cervical and thoracic spinal cord. The patient had dissociated sensory loss on trunk and upper left extremity, muscle atrophy, left hand paresis, long tracts signs. After having done atlanto-occipital decompression, the intramedullary cavity collapsed and neurological symptoms resolved. Two months later large tentorial meningioma was successfully removed via occipito-suboccipital craniotomy with tentorial transsection. A review of the literature concerning syringomyelia secondary to intracranial tumours was done. To our knowledge this is the first such case described in which syringomyelia syndrome was the prominent symptom of the disease and two staged surgical procedure, first oriented at treatment of syringomyelia itself, was applied.     

Malignant Nerve Sheath Tumor of the Spinal Accessory Nerve: A Unique Presentation of a Rare Tumor

Background

Malignant peripheral nerve sheath tumors (MPNSTs), sarcomas originating from tissues of mesenchymal origin, are rare in patients without a history of neurofibromatosis.

Case Report

We report a case of an MPNST of the spinal accessory nerve, unassociated with neurofibromatosis, which metastasized to the brain. The tumor, originating in the intrasternomastoid segment of the spinal accessory nerve, was removed. Two years later, the patient presented with focal neurological deficits. Radiographic findings revealed a well-defined 2.2×2.2×2.2 cm, homogeneously enhancing mass in the left parieto-occipital region of the brain surrounded by significant vasogenic edema and mass effect, culminating in a 1-cm midline shift to the right. The mass was surgically removed. The patient had nearly complete recovery of vision, speech, and memory.

Conclusions

To our knowledge, this is the first documented case of an MPNST arising from an extracranial segment of the spinal accessory nerve and metastasizing to the brain.     

A case of amnesia caused by a falx meningioma in the right frontal region]

A 60-year-old right-handed man suffered frontal lobe dysfunction caused by a falx meningioma. He appeared to have amnesia and left hemiparesis. It is note-worthy that he had a lesion of the right frontal region but exhibited both verbal and non-verbal memory impairment, although his intelligence, remote memory and digit span were normal. Computed tomography and magnetic resonance imaging revealed a falx meningioma in the right frontal lobe including the right cingulate gyrus and corpus callosum. Single photon emission CT revealed a wide area of low perfusion surrounding the right frontal lobe including the cingulate gyrus. After surgery, left hemiparesis and amnesia disappeared. SPECT after surgery showed an area of low perfusion limited to the right frontal region. We concluded that the amnesia in this case might be caused by the interruption of hippocampal input into the cingulate gyrus.     

Awake far lateral craniotomy for resection of foramen magnum meningioma in a patient with tenuous motor and somatosensory evoked potentials

We report a patient with an infratentorial lesion resected under a far-lateral approach during awake craniotomy to optimize intraoperative monitoring. A 72-year-old man presented with falls, difficulty walking, and lower extremity weakness. MRI revealed a 2.2 by 2.3 by 2.8 cm mass at the right cervicomedullary junction, with mass effect on the adjacent spinal cord. During two attempts during surgical positioning under general anesthesia, motor evoked potentials were lost. Each time the operation was aborted. During the third operation, the patient underwent monitored, light anaesthesia and was awakened periodically to confirm conscious motor function. The operation proceeded without complication, and postoperatively there was no further decrease in motor function. This is, to our knowledge, the first use of an awake operation for an infratentorial meningioma via the far-lateral approach, demonstrating the technique may be used safely and can be useful in optimizing motor function monitoring.     

Unusual case of multiple cellular and malignant schwannomas of the cranial and spinal nerves

Schwannomas from cranial nerves and spinal roots are most often benign, malignant schwannomas being uncommon in this location. This report describes a unique case of multiple cellular and malignant schwannomas of the cranial and spinal nerves in a patient with features of neurofibromatosis 2. The tumors were arising from left optic, bilateral oculomotor, trochlear, abducent and vestibular nerves, the left facial and the spinal lumbar nerve roots. The tumor arising from the right trochlear nerve was seen excavating into the hippocampus and the left vestibular nerve into the medulla. In addition, the patient had nodular schwannomas adherent to the inferior surface of the optic chiasm and the cerebellum away from the cranial nerves. Hyperplastic schwannosis was noted in perivascular spaces of thalamus, cerebellum and hippocampus on both sides away from the main lesions. The immunohistochemical and ultrastructural profiles of the tumors suggest that neurofibromas and schwannomas are probably not distinct tumors but lie within a spectrum that differs histologically depending on the predominant cell type.     

Malignant disseminated chordoid meningioma in a 12-year-old child: a role for early cranial and spinal radiation treatment after subtotal resection

Case report We present a case of a chordoid meningioma, a tumour sub-type which comprises less than 0.5% of all meningiomas. The patient, a 12-year-old Somalian girl with a history of having had a craniotomy for a brain tumour in her native country 11 months previously, presented with seizures. Imaging revealed an isolated left frontal tumour, which at the time was felt to be a residual mass. She underwent a macroscopically complete resection of this tumour. Histology revealed it to be a grade 2 chordoid meningioma. Recurrence with additional local meningeal lesions was detected on follow-up magnetic resonance imaging (MRI) at 2 months post surgery. These were resected and she was treated with adjuvant cranial radiation treatment that resulted in non-progression of the cranial tumour on imaging 6 and 12 months post surgery. However, at 12 months post initial presentation, she re-presented with disseminated spinal disease refractory to salvage radiation treatment and succumbed to her illness.Outcome This is the first reported case of cranial radiation treatment being used to treat sub-totally resected recurrent chordoid meningioma. Whilst intra-cranial control of the tumour was achieved with this management, it did not prevent spinal progression. The authors advise a high-surveillance management strategy when treating these lesions and to employ cranial and spinal radiation treatment at the first sign of disease progression.     

Cystic schwannoma of the falx cerebri.

Intracranial parenchymal schwannomas unrelated to a major cranial nerve are uncommon and dural schwannomas are rare. We report a 23-year-old woman without neurofibromatosis admitted with a 3-month history of seizures and left hemiparesis. Radiological investigation revealed a huge cystic tumour in the right cerebral hemisphere, attached to the falx cerebri. The solid part of the tumour showed contrast enhancement. The patient underwent excision of the tumour via a right-sided parietal craniotomy. The histological diagnosis was schwannoma. Recognition of these potentially curable tumours is important and they should be excised if possible.     

Abnormalities of short-latency somatosensory evoked potentials in parkinsonian patients

Twenty-two patients (16 affected by parkinsonian syndromes, 6 by other neurological diseases) and 12 age-matched controls were examined. Short-latency somatosensory evoked potentials were recorded from 30 scalp electrodes in the 45-52 msec following separate left and right median nerve stimulation at the wrist. Bit-colour maps were generated on a 4096 pixel matrix via quadratic interpolation. Peak latencies and amplitudes of the parietal, central and frontal components were evaluated. Moreover, the amplitude ratios between parietal and frontal components on the same hemiscalp and between peaks on homologous right and left scalp districts were taken into account. The unique significant difference between parkinsonians and controls was represented by a depressed frontal N30 wave. This peak was absent in 3 and reduced in 7 out of 16 parkinsonians, with an overall abnormality rate of 47% of the examined arms. Average maps pooling data of parkinsonians and controls confirmed the presence of reduced evoked activity for the whole duration of wave N30 on those mid- and parasagittal frontal districts where this peak is maximally represented in normals. A similar abnormality was found in 1 of the 6 non-parkinsonian neurological patients suffering from a meningioma of the falx compressing the left supplementary motor area. Possible pathophysiology of such wave N30 abnormalities in parkinsonians is discussed.     

Modified skin incision for avoiding the lesser occipital nerve and occipital artery during retrosigmoid craniotomy: potential applications for enhancing operative working distance and angles while minimizing the risk of postoperative neuralgias and intraoperative hemorrhage

Chronic postoperative neuralgias and headache following retrosigmoid craniotomy can be uncomfortable for the patient. We aimed to better elucidate the regional nerve anatomy in an effort to minimize this postoperative complication. Ten adult cadaveric heads (20 sides) were dissected to observe the relationship between the lesser occipital nerve and a traditional linear versus modified U incision during retrosigmoid craniotomy. Additionally, the relationship between these incisions and the occipital artery were observed. The lesser occipital nerve was found to have two types of course. Type I nerves (60%) remained close to the posterior border of the sternocleidomastoid muscle and some crossed anteriorly over the sternocleidomastoid muscle near the mastoid process. Type II nerves (40%) left the posterior border of the sternocleidomastoid muscle and swung medially (up to 4.5 cm posterior to the posterior border of the sternocleidomastoid muscle) as they ascended over the occiput. The lesser occipital nerve was near a midpoint of a line between the external occipital protuberance and mastoid process in all specimens with the type II nerve configuration. Based on our findings, the inverted U incision would be less likely to injure the type II nerves but would necessarily cross over type I nerves, especially more cranially on the nerve at the apex of the incision. As the more traditional linear incision would most likely transect the type I nerves and more so near their trunk, the U incision may be the overall better choice in avoiding neural and occipital artery injury during retrosigmoid approaches.     

微囊型脑膜瘤的MRI表现及临床病理对照研究

目的探讨微囊型脑膜瘤的MRI表现及其诊断价值,并与病理相对照。方法回顾性分析12例经病理证实的微囊型脑膜瘤影像学资料及病理资料。结果额叶6例(左4例,右2例),颞叶2例,顶叶大脑镰旁2例,右侧桥脑小脑角区1例,鞍区1例;12例中9例平扫T1WI呈低信号,T2WI呈明显高信号,增强后病变呈明显不均匀强化;3例平扫时T1WI呈不均匀等低信号,T2WI呈不均匀等高混杂信号,增强后病变呈明显不均匀强化;12例病变中5例可见脑膜尾征;10例可见中、重度瘤周水肿;镜下组织中见瘤细胞排列疏松,呈微囊状。结论微囊型脑膜瘤是颅内少见肿瘤,MRI表现有一定特点:T1WI呈低信号,T2WI呈明亮高信号,瘤周水肿明显,增强后呈明显强化,MRI有助于对其诊断和鉴别诊断。     

Postoperative mutism after removal of an anterior falcine meningioma.

Postoperative mutism is rare. We present a 65-year-old man who had transient mutism after resection of anterior falx meningioma. Mild left hemiparesis and palmomental reflex on the right were the only abnormal signs on neurological examination. CT scan and MRI demonstrated a mass at the anterior one-third portion of the falx just superior to the corpus callosum. The mass enhanced homogenously with administration of gadolinium DTPA. The patient underwent surgical resection of the lesion and adjacent falx cerebri. The operation was uneventful. On the second postoperative day he became mute. He could follow verbal commands, and write and read. Postoperative CT scan revealed a hypodense area in the right frontal lobe including a part of the anterior cingulate cortex and the anterior part of the corpus callosum. Histopathological examination revealed a mixed meningioma. Ten days postoperatively, he began to say simple words, and three weeks later he could talk normally. We consider that lesion of the supplementary motor area (SMA) may be responsible for postoperative mutism.     

Deep sylvian meningioma: a case report and review of literature

Case report  A 6-year-old boy presented with seizures. Computed tomography and magnetic resonance imaging showed a large enhancing mass in the left temporo-parietal region. Treatment  He underwent left temporo-parietal craniotomy and total excision of the lesion. At surgery, there was no dural attachment, and the tumor was mainly in the posterior part of left sylvian fissure. The biopsy was reported as WHO grade I meningioma. Outcome  At 4-year follow-up, he was asymptomatic, and there was no tumor recurrence.     

Using algometry of pressure measuring the threshold of trigeminal pain perception in normal volunteers: a new protocol of studies

Algometry of pressure is a technique that measures the physiology of the nociceptive system. Acting directly on the responsive peripheral nociceptors to pressure stimuli, this technique allows the study on nociceptive integrity in normal subjects or having different algic syndromes. Utilizing 29 asymptomatic volunteers, the threshold of the painful perception was studied, measuring them in a direct way over the emergence of the supra-orbital, infra-orbital and mental nerves. The following algometric average were recorded: right mental nerve 46.2 Kg/cm2 and left 48.6 Kg/cm2; right supra-orbital nerve 47.7 Kg/cm2 and left 45.2 Kg/cm2; right infra-orbital nerve 53.9 Kg/cm2 and left 55.4 Kg/cm2. After reviewing the principles of the algometry utilization, we have validated this protocol, showing the average values obtained by measuring the trigeminal system, afterwards comparing them with an inervated region by cervical branches (major occipital nerve) and the temporal muscle.     

Surgical treatment of parasagittal and falx meningiomas

BACKGROUND AND PURPOSE: We present our experience with surgery of parasagittal and falx meningiomas with special consideration of surgical outcome and risk of recurrence. MATERIAL AND METHODS: A series of 87 consecutive patients surgically treated for parasagittal and falx meningiomas is reported. 50 patients had parasagittal meningiomas and a further 37 had falx meningiomas. Meningioma invaded the superior sagittal sinus in 21 cases. According to Simpson's scale, 25 procedures were Grade I resection, 55 were Grade II resection and 7 were Grade IV resection. Among 21 patients with parasagittal meningiomas invading the sagittal sinus, radical resection of the tumour and invaded part of sinus was made in 9 cases. RESULTS: Seven patients were severely disabled and 4 patients died after the surgery whereas 76 patients had satisfactory outcome on discharge. At the time of analysis, 14 patients had shown evidence of recurrence. Male gender, partial removal of meningioma (Simpson Grade IV) and bilateral falx meningioma had a statistically significant influence on recurrence. There were no tumour recurrences following radical resection of the tumour and invaded part of sinus, but two postoperative deaths due to haemodynamic complications were noted. In the other 12 patients, meningiomas were removed but sinus infiltration was left in place; the postoperative period was uneventful but the rate of clinically important regrowth in this group of patients was 25% in long-term follow-up. CONCLUSIONS: Rate of recurrence of parasagittal and falx meningioma significantly increases in cases of non-radical resection of tumour. Aggressive surgical treatment presents several hazards and carries an increased risk of unsatisfactory outcome; the risk of recurrence, however, is significantly decreased.     

On the use of fast blue, fluoro-gold and diamidino yellow for retrograde tracing after peripheral nerve injury: uptake, fading, dye interactions, and toxicity

The usefulness of three retrograde fluorescent dyes for tracing injured peripheral axons was investigated. The rat sciatic was transected bilaterally and the proximal end briefly exposed to either Fast Blue (FB), Fluoro-Gold (FG) or to Diamidino Yellow (DY) on the right side, and to saline on the left side, respectively. The nerves were then resutured and allowed to regenerate. Electrophysiological tests 3 months later showed similar latencies and amplitudes of evoked muscle and nerve action potentials between tracer groups. The nerves were then cut distal to the original injury and exposed to a second (different) dye. Five days later, retrogradely labelled neurones were counted in the dorsal root ganglia (DRGs) and spinal cord ventral horn. The number of neurones labelled by the first tracer was similar for all three dyes in the DRG and ventral horn except for FG, which labelled fewer motoneurones. When used as second tracer, DY labelled fewer neurones than FG and FB in some experimental situations. The total number of neurones labelled by the first and/or second tracer was reduced by about 30% compared with controls. The contributions of cell death as well as different optional tracer combinations for studies of nerve regeneration are discussed.