SUMMARY Eight patients are described with an unusual form of carotid transient ischemic attack, limb shaking. The basic features included a brief, involuntary, coarse, irregular, wavering movement or tremble involving arm-hand alone, or arm-hand and leg together. In 2 patients limb shaking was the initial manifestation of carotid occlusive disease, and all but one patient had other typical carotid transient ischemic attacks.Major atheromatous carotid occlusive disease was present in all patients on the side opposite the limb movements. Four patients had bilateral carotid occlusive disease.Cerebral ischemia from a carotid territory low-perfusion state may be the pathogenesis of these limb movements, an idea supported by the apparent benefit of surgical revascularization in abolishing or reducing the limb shaking in 6 patients. There was no clinical or EEG evidence to document an epileptiform etiology.Recognition of this uncommon form of carotid transient ischemic attack may be important in the early diagnosis and treatment of carotid occlusive disease.Stroke Case Reports Patient 1An 88-year-old man had a three week history of intermittent episodes of right-sided weakness and speaking difficulty. The episodes, lasting only seconds, involved weakness of the right arm and hand, buckling of the right knee, and speaking difficulty. The usual patterns were right arm and leg, sometimes only the right arm, and rarely the right leg alone. Thick and hesitant speech was associated with some of these events. Involuntary trembling movements of the right arm and hand frequently accompanied the right arm weakness, and prompted a trial of phenytoin treatment on the suspicion these were focal seizures. The phenytoin was discontinued, however, when he became toxic. There was no history of transient monocular blindness. A CT scan and EEG were both normal.On admission evaluation, blood pressure was 200/90. The general physical examination was negative except for loud bilateral carotid bifurcation bruits. The neurologic examination was remarkable only for an occasional hesitancy and stammering quality in his speech, and mild right lower face weakness. Strength in arms and legs was normal.During hospitalization four episodes of right arm and hand shaking were observed when the patient was either supine or sitting, all occurring during intravenous heparin anticoagulation. The movements were wavering, shaking excursions of the arm and hand which lasted 4-5 seconds. During one episode, there were 10 second wavering movements of the right hand, at which time he had difficulty holding a fork. He could talk during the shaking, although speech was more hesitant and several verbal paraphasic errors occurred. His right arm could be lifted on its own power to the horizontal during the shaking, but a lateral drift and wavering-shaking movements of a nonrhythmic nature were observed. Cerebral angiography demonstrated severe stenosis of the left internal carotid artery. Technical difficulties prevented further angiographic studies. Repeat CT scan...

We describe two histologically unusual cases of ependymoma of the filum terminale. Both tumors occurred in 14-year-old boys. An intradural encapsulated mass attached to the filum terminale was demonstrated radiologically in both cases and totally resected at surgery. In case 1 the neoplasm was uniformly composed of pleomorphic giant cells and was without perivascular pseudorosettes or myxopapillary changes. Case 2 was a myxopapillary ependymoma with multiple foci of pleomorphic giant cells. Neither tumor had prominent mitotic activity, necrosis, or endothelial proliferation. Both tumors were immunopositive for cytokeratin and glial fibrillary acidic protein. Ultrastructural features included basal laminae, interdigitating cell processes, microvilli, cilia, intercellular junctions, and cytoplasmic intermediate filaments. Cytogenetic analysis in case 1 showed a hypodiploid karyotype with monosomy of chromosomes 1, 10, 14, 16, 20, and 22. We interpret both tumors as most consistent with a variant of ependymoma. Because of the unique gigantocellular light microscopic appearance of the entire tumor in case 1, we propose classifying this tumor as a new morphologic subtype: giant cell ependymoma of the filum terminale. The combination of gigantocellular and myxopapillary features in case 2 supports a histogenetic relationship between giant cell ependymoma and myxopapillary ependymoma.

"Surgery-only" LGG survivors may be more affected by their tumor and its resection than previously appreciated. A prospective study is needed to address this survivor population.

Surgical correction of synostotic frontal plagiocephaly ("unilateral coronal synostosis") focuses on distortions of the forehead and orbits. Technical variations include unilateral versus bilateral fronto-orbital positioning. Surgical alignment of the deviated nasal root was introduced in our unit. Anthropometry was used to assess anatomic outcome, and results were compared in 22 children with synostotic frontal plagiocephaly who had either (1) unilateral fronto-orbital advancement ("canthal advancement") (n = 8), (2) bilateral fronto-orbital advancement/ modeling without nasal straightening (n = 7), or (3) bilateral fronto-orbital advancement/modeling with closing wedge nasal osteotomy (n = 7). Postoperative fronto-orbital asymmetry was most marked in the group I patients wherein the ipsilateral supraorbital rim was retruded 3.9 mm and elevated 2.6 mm, on average relative to the corneal apex, compared with the normal side. Group II children averaged 2-mm orbital retrusion and 2.2-mm elevation. Group III patients averaged 1.4-mm orbital retrusion and 2.9-mm elevation. These differences in orbital rim measurements among the three groups were not statistically significant. Postoperative nasal root angulation of 4 degrees or more was found in more than 50 percent of children who had either a unilateral or a bilateral procedure, without nasal correction. In contrast, primary nasal osteotomy resulted in a nasal cant of 3 degrees or less in all children. This difference in nasal angulation among the three groups was statistically significant (p = 0.035). Group III had a straighter nasal angle than groups II and I (in that order). Measurement of the distances from nasion to inner and to outer canthi also reflected persistent deviation of the nasal root. Group III children had a more central radix than either group I or II (p = 0.05). The data in this study support an operative strategy of bilateral (parallelogrammic) positioning of the forehead/ superior orbits with primary correction of nasal root angulation.

Background: We undertook this study to characterize those patients who required reoperations for Chiari malformation and to determine whether modifications in surgical technique at the initial procedure might have obviated the need for repeat surgery. Methods: We reviewed the hospital records, imaging studies, operative reports, and follow-up data of those patients who were undergoing a second operation as part of a consecutive series of Chiari decompressions carried out by one neurosurgeon over a 14-year period. We wished to determine whether type of Chiari malformation, age at initial surgery, the presence of associated bony anomalies, or initial operative technique contributed to the need for reoperation. Results: Twenty-two (17%) of the surgical procedures represented reoperations (16 of 100 Chiari I and 6 of 33 Chiari II decompressions). Of the patients in the series who were operated on at age 5 years or younger, 7 (16%) were reoperations (5 of 33 Chiari I and 2 of 10 Chiari II decompressions); 15 patients (17%) of those older than 5 years at surgery were reoperations (11 of 67 Chiari I and 4 of 23 Chiari II decompressions). 8 of the 16 patients who underwent reoperations for Chiari I malformation and 4 of the 6 patients who underwent reoperations for Chiari II malformation had their initial decompression surgery at age 5 years or younger. Reoperation was required because of failure to treat adequately an associated syrinx (n = 11), because of persisting neurological symptoms or headache (n = 9), or because of a possible faulty fourth ventricular stent placement (n = 2). 5 of 9 patients in the operative series with Chiari malformations associated with craniosynostosis represented reoperations (55%), and all 3 of the patients whose syrinx persisted after reoperation had craniosynostosis syndromes. Conclusions: Approximately 17% of the patients with Chiari I and Chiari II malformations in this operative series were undergoing a second operation. Factors in this series which were associated with reoperation included a young age at initial surgery, the presence of complex bony anatomy at the foramen magnum, syndromic craniosynostosis, and failure of the surgeon at the initial operation either to assess patency of the foramen of Magendie or correctly place a fourth ventricular stent.

We reviewed 26 patients with anorectal or urogenital malformations managed by the neurosurgical service at The Children’s Hospital for tethered spinal cord as diagnosed by spinal MRI. There were 13 patients with cloacal exstrophy, 5 with the Vater association, 3 with imperforate anus, and 5 with other complex anomalies. Tethering spinal cord lesions included myelocystocele, lipomyelomeningocele, and filum lipoma. We review the clinical course, management, correlation of MRI and intraoperative findings, and outcome of the patients, as well as the embryology which underlies the association of these malformations.

Two cases of aneurysmal malformations of the vein of Galen (AVG) with spontaneous thrombosis are reported. Angiogram and MRI before thrombosis demonstrated AVGs with slow arteriovenous shunts and associated stagnation of contrast in the venous sac secondary to severe outflow restriction. Based on these findings, one patient was managed conservatively, and the other underwent placement of a ventriculoperitoneal shunt. Surveillance of the lesions with subsequent MRIs revealed spontaneous thrombosis of the AVGs with excellent clinical outcomes. Proposed mechanisms of spontaneous thrombosis include slow flow shunts, obstruction of the venous outflow or obstruction of the feeding artery. Similar cases in the literature are reviewed with special emphasis on diagnostic tests, symptomatology, mechanisms of thrombosis and therapeutic options.

Myxopapillary ependymomas (MPEs) are rare spinal tumors in children. The natural history and clinical course of pediatric MPEs are largely unknown and the indication for adjuvant therapy remains to be clarified. We performed an IRB-approved, retrospective review of children with MPEs treated at the Dana-Farber/Boston Children's Cancer and Blood Disorder Center between 1982 and 2013. Eighteen children (age range 8-21 years, median age 14 years) met inclusion criteria. We reviewed the histopathology, magnetic resonance imaging, tumor location and stage, surgical management, adjuvant therapy, and clinical outcomes. The median follow-up duration was 9.4 years (range 1-30 years). Children most commonly presented with pain, scoliosis, and urinary symptoms. All primary tumors were located in the lower thoracic or lumbar spine. Nine children (50%) had leptomeningeal tumor seeding at presentation, most commonly located within the distal thecal sac. A gross-total resection was achieved in nine children (50%). Three children were treated with irradiation following initial surgery. No child received adjuvant chemotherapy at diagnosis. The 10-year event-free survival (EFS) was 26% ± 14.8. Children with disseminated disease trended towards inferior EFS compared to those with localized disease (10-year EFS 12.7% ± 12 vs. 57 ± 25%, p value 0.07). The 10-year overall survival was 100%. The efficacy of adjuvant irradiation could not be assessed due to the small sample size. Although children with MPEs frequently present with disseminated tumor and/or develop recurrent or progressive disease, their overall survival is excellent. Treatment should aim to minimize both tumor- and therapy-related morbidity.