Evaluating cerebellar dentatotomy for the treatment of spasticity with or without dystonia.

Spasticity represents a common and very often incapacitating neurologic condition, for which a limited number of treatment options are available. Stereotactic ablation of the dentate cerebellar nuclei (dentatotomy) was widely used in the past with variable results. In the present study, we reviewed 12 consecutive cases operated on at the Midland Centre for Neurosurgery and Neurology at Birmingham University (Smetwick, UK) in the 1980s. The following clinical aspects were analyzed: severity of spasticity, occurrence of abnormal movements (dystonia/athetosis), language fluency, gait, and overall clinical condition. Follow-up ranged from 0.5 to 94 (mean: 31.6) months. Immediate improvement was noted in 10 patients, and five of them had sustained this improvement at the time of the last assessment. A more pronounced improvement was observed for gait, relative to speech and abnormal movements. No significant morbidity related to the procedure was observed. We conclude that dentatotomy is a safe procedure that should still be considered in specific cases. Here, we review the cases reported in the literature, and present a mechanistic hypothesis about how dentatotomy influences motor tonus, in light of the current knowledge about cerebellar physiology. We believe that this issue is critical for the development of alternative surgical approaches targeting the cerebellum, such as chronic electric stimulation.

Preoperative MRI findings and functional outcome after selective dorsal rhizotomy in children with bilateral spasticity.

PURPOSE: To identify MRI characteristics that may predict the functional effect of selective dorsal rhizotomy (SDR) in children with bilateral spastic paresis. METHODS: We performed SDR in a group of 36 patients. The gross motor functioning measure-66 (GMFM-66) was applied before and after SDR. Available cerebral MRIs were retrospectively classified into three diagnostic groups: periventricular leucomalacia (PVL; n = 10), hydrocephalus (n = 2), and normal (n = 6). In patients with PVL, we scored the severity of the MR abnormalities. We compared the changes in the GMFM-66 after SDR in the diagnostic groups. In patients with PVL, we correlated the severity of the MR abnormalities with the changes in the GMFM-66. RESULTS: The mean follow-up period was 5 years and 4 months (range, 1 year and 1 month to 9 years). The best improvement in gross motor function was observed in patients with normal MRI, and the slightest improvement was observed in patients with hydrocephalus. The severity of the PVL did correlate with the GMFM-66 score before SDR but not with the functional effect of SDR. CONCLUSION: We conclude that with respect to gross motor skills, the improvements after SDR are good in patients with no MRI abnormalities. In the patients with hydrocephalus, the improvements after SDR were insignificant. In patients with PVL, the improvements were intermediate and did not correlate with the degree of PVL.

Idiopathic ventral spinal cord herniation: a rare presentation of tethered cord.

Idiopathic ventral spinal cord herniation is a rare condition that has been increasingly reported in the last decade. The natural history and optimal management have yet to be defined. Therefore, debate exists regarding the pathogenesis and surgical management of this condition. The purpose of this review article is to further educate neurosurgeons about the surgical techniques and outcomes associated with treating this rare and often misdiagnosed condition.

Causes of lower extremity weaknesses after posterior lumbar spine fusion surgery and therapeutic effects of active surgical exploration.

BACKGROUND: This study was aimed at investigating the causes of lower extremity weaknesses after posterior lumbar spine fusion surgery and looking at subsequent treatment strategies. METHODS: Patients who underwent posterior lumbar spine fusion surgery in the Peking University First Hospital between January 2009 and December 2018 were counted. Those who needed secondary surgery because of subsequent lower extremity weaknesses were selected. CT scans and MRIs were used to evaluate the reasons for weaknesses before secondary surgery. Muscle strength was evaluated after surgery. RESULTS: Thirty patients (30/4078, 0.74%) required a secondary surgery because of lower extremity weaknesses after posterior lumbar spine fusion surgery. The main causes of weakness were (1) internal fixation malposition and loosening (11 patients, 36%), (2) epidural hematomas (9 patients, 30%), (3) insufficient decompression (5 patients, 17%), and (4) nerve root edemas (5 patients, 17%). Weakness occurred on average 2.9 days after surgery (1-9 days). Twenty-seven patients (90%) got improved muscle strength after their secondary surgery. CONCLUSIONS: Iatrogenic neurologic deficits and lower extremity weaknesses were rare complications after posterior lumbar spine fusion surgeries, but important to recognize and manage. The main causes of weakness were internal fixation malposition and loosening, epidural hematomas, insufficient decompression, or root edemas. There may be positive, therapeutic effects to subsequent, active surgical exploration.

Are acquired hepatocerebral degeneration and hepatic myelopathy reversible?

BACKGROUND: Acquired hepatocerebral degeneration (AHD) and hepatic myelopathy (HM) are rare complications of chronic liver disease and are usually resistant to medical therapy. MATERIALS AND METHODS: The clinical and laboratory findings of 14 male and 2 female patients with AHD or HM were evaluated. RESULTS: The prevalence of AHD and HM was 2% inpatient case series in the last 10 years. The median age of the patients (5 Child's B and 11 Child's C) was 48.7 years (28 to 66 y), and the mean known duration of the liver disease was 75 months (24 to 194 mo). The median time of onset of neurologic findings after diagnosis of the liver disease was 14.5 months. Eight patients who had marked spastic paraparesis or tetraparesis were included in the HM group and all others had AHD group. Sixty-nine percent of the patients had a spontaneous or surgical portosystemic shunts, and the remaining dense retroperitoneal collaterals. During the follow-up period of median 29 months (4 to 72 mo), 12 patients died while waiting for liver transplantation, and these patients suffered from the several complications of chronic liver disease more than the living patients. A marked improvement was observed in 2 of the patients (1 with AHD and the other with HM) at 6 and 8 months after the liver transplantation, respectively. CONCLUSIONS: Our data suggest that liver transplantation had an important effect on the improvement in these patients.

Intramedullary Capillary Hemangioma Presenting with Hydrocephalus and Spastic Paraparesis in 2-Month-Old Infant.

BACKGROUND: Intramedullary capillary hemangiomas are rare lesions with only 14 cases reported so far and are found in middle-aged persons. Until now only a single case has been reported in the pediatric population. Ours was the second pediatric case and the youngest of all to be diagnosed with an intramedullary capillary hemangioma. CASE DESCRIPTION: We are reporting the case of a 2-month male child who presented with features of hydrocephalus and spastic paraparesis. On magnetic resonance imaging he had features suggestive of a D10-11 intradural extramedullary lesion and hydrocephalus. We did a ventriculoperitoneal shunt followed by excision of the lesion in the same sitting. Intraoperatively it was found to be an intramedullary lesion, and histopathologic examination showed features suggestive of capillary hemangioma. Postoperatively he recovered well and there is no recurrence 18 months after surgery. CONCLUSIONS: Intramedullary capillary hemangiomas are extremely rare lesions in infants but should be kept in mind while treating any patient with the spinal lesion. Further reporting of similar cases will help in understanding the natural history, pathogenesis, and management.

A role for myelography in assessing paraparesis.

Imaging of the spine is a fundamental part of assessment of paraparesis. Since the advent of MRI the indications for myelograms have diminished. However, a myelogram, although an invasive test, should still be considered a useful investigation for localising lesions in the spinal cord and for identifying rare causes of myelopathy. This case illustrates how a CT myelogram identified an arachnoid cyst, which is a potentially treatable cause of paraparesis.

Arachnoiditis ossificans with progressive syringomyelia and spinal arachnoid cyst.

We present a 30-year-old man with progressive spastic paraparesis. Spinal imaging revealed extensive calcification of the thoracic cord and cauda equina arachnoid, an intradural extramedullary cyst and evidence of rapidly progressing syringomyelia. Radiological diagnosis was arachnoiditis ossificans and an attempt at surgical decompression was made because of progressive neurologic deterioration. Due to tenacious adhesion of the calcified plaques to the cord and roots, only cyst drainage was achieved; the patient had no clinical improvement. A literature review revealed only two other cases reported in the literature with co-existence of arachnoiditis ossificans and syringomyelia. In none of the previous cases was there an intradural extramedullary arachnoid cyst, nor did the syrinx progress in such a rapid fashion. An attempt is made to explain possible pathophysiological mechanisms leading to this unusual pathology.

Spinal cord herniation repair with microstaples: case report.

Idiopathic spinal cord herniation (ISCH) is a relatively rare and frequently misdiagnosed condition. It preferentially affects women and causes progressive thoracic myelopathy that presents as a Brown-Séquard syndrome or as spastic paraparesis. Although its etiology and pathogenesis are controversial, ISCH is characterized by the presence of an anterior dural defect that allows the incarceration of a segment of the cord. Typically, a C-shaped ventral displacement and kinking of the cord are visible on sagittal MRI. Surgery aimed at stopping or reversing myelopathic symptoms is usually recommended for symptomatic patients. Surgical options include reduction of the hernia and direct suturing, or enlargement of the dural defect, with or without patching. Suturing under the cord in a very tight space can be troublesome and may lead to neurological deterioration. The authors present the case of a symptomatic ISCH in which nonpenetrating titanium microstaples were used to close the dural defect after cord reduction. The patient experienced a good outcome, and the follow-up MRI study showed adequate cord repositioning and stability of the suture. The use of microstaples, which allows for an easier and faster dural closure than conventional suturing, is a novel technical adjunct that has not been previously reported for this condition. In addition, microstaples produce minimal metallic artifact that does not hinder the quality of follow-up MR images.

Impacted cisterna magna without syringomyelia associated with spastic paraparesis: case report.

We report on a 49 year old man with impacted cisterna magna without the presence of syringohydromyelie (SM). The clinical picture was characterized by spastic paraparesis. Magnetic resonance imaging depicted a cisterna magna filled by the cerebellar tonsils. Six months after osteodural-neural decompression of the posterior fossa there was resolution of neurological symptoms and signs with the exception of hyperactive patellar and Achilles reflexes.

Basilar impression associated with impacted cisterna magna, spastic paraparesis and distress of balance: case report.

We report on a 48 years-old man with basilar impression without syringohydromyelia, in which the cisterna magna was impacted by the cerebellar tonsils. Six months after posterior fossa decompression there was the disappearance of nuchal rigidity, vertigo, spastic paraparesis and improvement of balance. Nevertheless hyperreflexia and diminished pallesthesia of the lower limbs persisted.

Subfascial expansive plasty for intrathecal baclofen pump replacement. A technical note.

PURPOSE: Although intratechal pump replacement is a common technique, it can be sometimes complicated when aiming to replace a 20 ml pump with a bigger one (40 ml). We developed a simple and straightforward technique to relax the wall of the pocket of the pump, preserving its fascial layer. METHODS: A 20-year-old boy with spastic tetraparesia was admitted for pump replacement. After scar opening and pump removal, various lineal incisions were performed in the posterior layer of the subfascial pocket in a cranio-caudal direction. RESULTS: A 40 ml pump was placed without skin incision ampliation and preserving subfascial plane. CONCLUSIONS: This easy, expansive technique for infusion pump replacement preserves subfascial plane and prevents the need for more extensive surgeries.

Giant intraspinal pseudomeningoceles cause delayed neurological dysfunction after brachial plexus injury: report of three cases.

OBJECTIVE AND IMPORTANCE: Delayed neurological dysfunction after a brachial plexus injury is uncommon. We present the cases of three patients with a history of significant brachial plexus trauma and late neurological deterioration secondary to giant intraspinal extradural pseudomeningoceles. CLINICAL PRESENTATION: Three patients, each with a remote history of brachial plexus trauma, presented with slowly progressive upper-limb weakness. An examination revealed bilateral lower motor neuron weakness in the upper extremities in all patients and evidence of spastic paraparesis in one. Magnetic resonance imaging and postmyelogram computed tomographic scans demonstrated large anterior extradural cerebrospinal fluid collections extending from the upper cervical to lower thoracic and lumbar levels in each patient. Myelograms demonstrated a connection with the subarachnoid space in two patients. INTERVENTION: Direct obliteration of the connection between the cyst and the subarachnoid space was completed in two patients, and a cystoperitoneal shunt was placed in the third. Postoperative imaging demonstrated complete resolution of the extradural collections. Arrest of progression of upper-limb deterioration was observed in all patients, and dramatic improvement of long tract symptoms occurred in one. CONCLUSION: Giant intraspinal pseudomeningoceles are a rare complication of brachial plexus root injuries or avulsion, capable of causing significant morbidity. Early intervention can improve symptoms related to long tract involvement and prevent further deterioration of lower motor neuron disease. The pathophysiology of neurological dysfunction caused by these giant collections is unclear; however, vascular and mechanical factors thought to be important in the pathogenesis of cervical myelopathy also may have a role.

Partial tibial neurectomy in 113 Belgian blue calves with spastic paresis.

One hundred and thirteen double-muscled Belgian blue calves (69 males and 44 females) with spastic paresis (52 unilateral and 61 bilateral) were treated surgically by partial tibial neurectomy under caudal epidural anaesthesia. Telephone inquiries made at least three-and-a-half months later established that good results were obtained in 83.2 per cent of the calves; a considerable improvement was reported in 4.4 per cent which still had intermittent spastic contractions; severe hyperflexion of the hock necessitating early slaughtering was recorded in 4.4 per cent of the calves; and in 8 per cent there was little or no improvement.

Long-term results of selective dorsal rhizotomy for hereditary spastic paraparesis.

Pure hereditary spastic paraparesis usually presents with progressive weakness and spasticity of the legs, which is similar to spastic cerebral palsy. In this study selective dorsal rhizotomy (SDR) was performed to improve the spasticity of pure hereditary spastic paraparesis and the long-term results were followed. A series of four patients with pure hereditary spastic paraparesis diagnosed by a multidisciplinary team received SDR. The dorsal rootlets from the L2 to S1 levels were selectively resected under electrophysiological monitoring. The patients were followed up for more than 2 years to evaluate the outcome of surgery. There was a significant reduction in muscular spasm after SDR. Standing and walking stability were improved in all patients which led to improvement in walking posture and longer walking distance without assistance. No urinary retention, cerebrospinal fluid leak, surgical infection or kyphosis occurred. For severe pure hereditary spastic paraparesis, SDR can reduce muscle spasm and improve standing and walking stability. These results were stable throughout follow-up. SDR performed at the level of the conus medullaris through a laminectomy from T12 to L1 or L1 to L2 requires a shorter incision, laminectomy of fewer segments, and has a shorter operation time than the usual method (laminectomy from L2 to S1). Intraoperative electrophysiological monitoring is helpful to discriminate abnormal rootlets and protect sphincter function.

The value of intraoperative motor evoked potential monitoring during surgical intervention for thoracic idiopathic spinal cord herniation.

OBJECT: Thoracic idiopathic spinal cord herniation (TISCH) is a rare neurological disorder characterized by an incarceration of the spinal cord at the site of a ventral dural defect. The disorder is associated with clinical signs of progressive thoracic myelopathy. Surgery can withhold the natural clinical course, but surgical repair of the dural defect bears a significant risk of additional postoperative motor deficits, including permanent paraplegia. Intraoperative online information about the functional integrity of the spinal cord and warning signs about acute functional impairment of motor pathways could contribute to a lower risk of permanent postoperative motor deficit. Motor evoked potential (MEP) monitoring can instantly and reliably detect dysfunction of motor pathways in the spinal cord. The authors have applied MEPs during intraoperative neurophysiological monitoring (IOM) for surgical repair of TISCH and have correlated the results of IOM with its influence on the surgical procedure and with the functional postoperative outcome. METHODS: The authors retrospectively reviewed the intraoperative neurophysiological data and clinical records of 4 patients who underwent surgical treatment for TISCH in 3 institutions where IOM, including somatosensory evoked potentials and MEPs, is routinely used for spinal cord surgery. In all 4 patients the spinal cord was reduced from a posterior approach and the dural defect was repaired using a dural graft. RESULTS: Motor evoked potential monitoring was feasible in all patients. Significant intraoperative changes of MEPs were observed in 2 patients. The changes were detected within seconds after manipulation of the spinal cord. Monitoring of MEPs led to immediate revision of the placement of the dural graft in one case and to temporary cessation of the release of the incarcerated spinal cord in the other. Changes occurred selectively in MEPs and were reversible. In both patients, transient changes in intraoperative MEPs correlated with a reversible postoperative motor deficit. Patients without significant changes in somatosensory evoked potentials and MEPs demonstrated no additional neurological deficit postoperatively and showed improvement of motor function during follow-up. CONCLUSIONS: Surgical repair of the dural defect is effected by release and reduction of the spinal cord and insertion of dural substitute over the dural defect. Careful monitoring of the functional integrity of spinal cord long tracts during surgical manipulation of the cord can detect surgically induced impairment. The authors' documentation of acute loss of MEPs that correlated with reversible postoperative motor deficit substantiates the necessity of IOM including continuous monitoring of MEPs for the surgical treatment of TISCH.

Primary intramedullary spinal germ cell tumors.

BACKGROUND: Intramedullary spinal germ cell tumors are rare lesions, with germinomas being the most common variant. METHODS: To date, there have been 23 reports of primary intramedullary germ cell tumors described in the literature, the vast majority occurring in Japanese patients. RESULTS: We present a case of a nonmetastatic intramedullary germ cell tumor in a 28-year-old Caucasian woman. CONCLUSIONS: Characteristics of intramedullary germ cell tumors are summarized, and the current role for surgery and adjuvant radiation and chemotherapy are discussed.

Short- and long-term effects of selective dorsal rhizotomy on gross motor function in ambulatory children with spastic diplegia.

OBJECT: The primary aim of this prospective cohort study was to evaluate the short-term (1 year) and long-term (mean 6 years) effects of selective dorsal rhizotomy (SDR) on gross motor function and spasticity in ambulatory children with spastic diplegia. Secondary aims were to investigate side effects, additional treatment during follow-up (botulinum toxin type A injections or orthopedic surgery), and parental satisfaction. METHODS: Thirty-three children who had undergone SDR at a mean age of 6 years and 7 months (± 2 years) were included. There were 7 children at Gross Motor Function Classification System (GMFCS) Level I, 7 at Level II, and 19 at Level III. Gross motor function was assessed with the Gross Motor Function Measure-66 (GMFM-66). Spasticity was measured according to a modified Tardieu scale. Side effects, additional treatment, and parental satisfaction were recorded using a parental questionnaire and medical records. RESULTS: At 1-year follow-up, mean GMFM-66 scores improved significantly by 4.3 ± 4.1 points. Children at GMFCS Levels I and II showed significantly more improvement (7.2 points) on the GMFM-66 compared with children at GMFCS Level III (2.9 points). On long-term follow-up (mean 6 years ± 22 months), mean GMFM-66 scores improved significantly by 6.5 ± 5.9 points, without a difference between children at GMFCS Levels I and II and Level III. No relapse of spasticity was noted. Ten children (30%) needed orthopedic surgery and 13 children (39%) received botulinum toxin type A treatment after SDR. Twenty (91%) of the 22 parents who answered the questionnaire at long-term follow-up believed that their child's functioning had improved after SDR. CONCLUSIONS: Selective dorsal rhizotomy resulted in short- and long-term improvements in gross motor function, without relapse of spasticity. However, the majority of the children still needed additional surgery or botulinum toxin A treatment.

Chiari malformation type I associated with familial spastic paraplegia: report of a surgically treated case.

CASE REPORT: We describe the case of a 7-year-old girl with Chiari malformation type I (CMI) and concomitant familial spastic paraplegia (FSP), who exhibited spastic gait disturbance. She also showed poor results on the standing tolerance test using thermography, which evaluates autonomic nerve function. The patient was subjected to posterior fossa decompression and cervical 1 (C1) laminectomy for CMI based on the result of the standing tolerance test. After the operation her gait disturbance and autonomic nerve dysfunction improved. DISCUSSION: Although no cases of CMI and concomitant FSP have been reported, due to the fact that autonomic nerve dysfunction is one of CMI symptoms but never FSP, the diagnosis of autonomic nerve dysfunction of CMI using the standing tolerance test played an important role in selecting the surgical procedure for this case.