Association of early surgery and absence of ataxia with full recovery after spinal intramedullary ependymoma resection.

OBJECTIVE: Spinal intramedullary ependymomas (IEs) represent a well-defined tumor entity usually warranting resection. Factors that determine full long-term neurological recovery after resection are seldomly reported on in larger clinical series. In this study, the authors aimed to highlight the neurological outcome of patients with IEs after resection, with a focus on full neurological recovery, and to explore possible risk factors for the absence of neurological amelioration to an optimal function after surgical treatment. METHODS: A single-center retrospective analysis of all patients undergoing surgery for IEs between 2007 and 2021 was performed. Data collection included patient demographics, symptoms, clinical findings, histopathological diagnosis, surgical procedures, complications, and neurological outcome. Patients harboring a favorable outcome (modified McCormick Scale [mMS] grade of I) were compared with patients with a less favorable outcome (mMS grade ≥ II) at the final follow-up. RESULTS: In total, 72 patients with a histologically diagnosed IE were included. IEs in those patients (41 males, 31 females; median age 51 [IQR 40-59] years) mostly occurred in the cervical (n = 40, 56%) or thoracic (n = 23, 32%) spine. Upon admission, motor deficits or gait deficits (mMS grade ≥ II) were present in 29 patients (40%), with a median mMS grade of II (IQR I-II). Gross-total resection was achieved in 60 patients (90%), and the rate of surgical complications was 7%. Histopathologically, 67 tumors (93%) were classified as WHO grade 2 ependymomas, 3 (4%) as WHO grade 1 subependymomas, and 2 (3%) as WHO grade 3 anaplastic ependymomas. After a mean follow-up of 863 ± 479 days, 37 patients (51%) had a fully preserved neurological function and 62 patients (86%) demonstrated an mMS grade of I or II. Comparison of favorable with unfavorable outcomes revealed an association of early surgery (within a year after symptom onset), the absence of ataxia or gait disorders, and a low mMS grade with full neurological recovery at the final follow-up. A subgroup of patients (n = 15, 21%) had nonresolving deterioration at the final follow-up, with no significant differences in relevant variables compared with the rest of the cohort. CONCLUSIONS: The data presented solidify the role of early surgery in the management of spinal IEs, especially in patients with mild neurological deficits. Furthermore, the presence of gait disturbance or ataxia confers a higher risk of incomplete long-term recovery after spinal ependymoma resection. Because a distinct subgroup of patients had nonresolving deterioration, even when presenting with an uneventful history, further analyses into this subgroup of patients are required.

VIM thalamotomy in the treatment of Holmes' tremor secondary to HIV-associated midbrain lesion: a case report.

Holmes' tremor (rubral tremor, cerebellar outflow tremor) is characterized by rest, intention and postural tremor, often localized to one upper extremity, associated with ipsilateral dysmetria and dysdiadochokinesia. We describe a case of successful treatment of Holmes' tremor with unilateral nucleus ventralis intermedius (VIM) thalamotomy. The subject is a 43-year-old woman with unremarkable previous medical history. She presented with complete left hemiparesis in the context of human immunodeficiency syndrome and the magnetic resonance image disclosed a contrast-enhancing lesion in right brain peduncle, in topography of red nucleus. She developed a progressive rest, intention and postural tremor in left upper limb. She was submitted to a stereotactic biopsy and the tremor became worse. She performed awake right VIM thalamotomy, with immediate complete resolution of tremor. There were no complications after procedure, and the result is stable after six months. We highlight the role of thalamotomy in cases like ours, once patient recovered well and, due to HIV, will need further neuroimage studies to evaluate neurologic complications of HIV. Deep brain stimulation in such cases may interfere with coming neuroimage quality and may act like a foreign body.

Ataxic gait following total gastrectomy for gastric cancer.

A 58-year-old woman, who had undergone total gastrectomy for early gastric cancer 9 years previously, visited the outpatient clinic complaining of progressive difficulty in walking for 15 d. Laboratory examinations showed macrocytic anemia and a decreased serum vitamin B12 concentration and increased serum concentrations of folate, vitamin E and copper. Magnetic resonance imaging showed multifocal high signal intensities along the posterior column of the cervical and thoracic spinal cord. Treatment consisted of intramuscular injections of vitamin B12 for 7 d, which increased her serum level of vitamin B12 to normal. This was followed by weekly intramuscular injections of vitamin B12 for another 2 wk and oral administration of vitamin B12 three times per day. After comprehensive rehabilitation for 4 wk, she showed sufficient improvements in strength and ataxic gait, enabling her to return to her normal daily activities.

Deep brain stimulation or thalamotomy in fragile X-associated tremor/ataxia syndrome? Case report.

We present the case of a 66-year-old man who has been treated for essential tremor since the age of 58. He developed mild cerebellar gait ataxia seven years after tremor onset. Moderate, global brain atrophy was identified on MRI scans. At the age of 68, only temporary tremor relief could be achieved by bilateral deep brain stimulation of the ventral intermedius nucleus of the thalamus. Bilateral stimulation of the subthalamic nucleus also resulted only in transient improvement. In the meantime, progressive gait ataxia and tetraataxia developed accompanied by other cerebellar symptoms, such as nystagmus and scanning speech. These correlated with progressive development of bilateral symmetric hyperintensity of the middle cerebellar peduncles on T2 weighted MRI scans. Genetic testing revealed premutation of the FMR1 gene, establishing the diagnosis of fragile X-associated tremor/ataxia syndrome. Although this is a rare disorder, it should be taken into consideration during preoperative evaluation of essential tremor. Postural tremor ceased two years later after thalamotomy on the left side, while kinetic tremor of the right hand also improved.

Quick note on tissue engineering-based surgical measures to treat patients with neurogenic bladder-due detrusor/sphincter dyssynergia.

To treat the neurogenic bladder-due detrusor/urethral rhabdosphincter dyssynergia, early combined clean intermittent catheterization/ pharmacotherapy (anticholinergic-, ß3-adrenoceptor agonist drugs) management may be at times crowned with success of preserving an adequate bladder compliance and renal safe conditions.The persistence, instead, of elevated bladder filling pressure levels with high voiding pressure/uroflow values, together with aberrant urethral rhabdosphincter electromyographic findings, make necessary the resort to surgery strategies, among which - a part from rhabdosphincterotomy or alternatively intrasphincteric botulinum A toxin injection or urethral stent insertion - the bladder augmentation cystoplasty, with either reconfigurated bowel- or gastric segment, is today the most efficacious surgical measure to increase the bladder urinary storage meanwhile lowering bladder filling pressure. Given the enterocistoplasty-dependent both potential systemic metabolic imbalances - such as hyperchloremic acidosis/hypokaliemia, hyperoxaluria, bone demineralization, chologenic diarrhoea/steatorrhoea, vit B12 deficiency - together with bowel prosthetic mucus overproduction-due recurrent stone formation, and, sometimes, malignant complications particularly at the intestinal-urinary tract suture line, tissue engineering techniques have been taken into consideration, more than twenty years ago, as alternative measure for bladder augmentation cystoplasty, until to reach successful clinical validation just in patients suffering from either congenital dysraphism- or acquired spinal cord injury-dependent neurogenic bladder. Nevertheless, also the tissue engineering-made augmentation cistoplasty, as well as that bowel-based one, unfortunately remains influenced by spinal cord neuropathydue dysfunctional effects, hence the tissue engineering research could be today directed to suitably overcome such disadvantageous conditions.

Ataxia induced by a thymic neuroblastoma in the elderly patient.

Thymic neuroblastoma is a rare tumor with only few reports in modern literature. Whereas most data is taken from childhood neuroblastoma, little is known about the characteristics of the disease in the adult and elderly population. There are significant differences between adult and childhood neuroblastoma which are reviewed below. We report a case of a 62-year-old male who presented with neurological symptoms of ataxia and opsoclonus and an anterior mediastinal mass. Ultimately, the patient underwent a resection of the mass and pathologic review identified a thymic neuroblastoma. This is the first case of thymic neuroblastoma associated with symptomatic central nervous system disease; it is presented with an up-to-date review of the previous cases in the field as well with a review of the literature of post adolescent neuroblastoma.

Dorsal laminectomy for treatment of cervical vertebral stenotic myelopathy in an alpaca.

CASE DESCRIPTION: An 11-year-old male breeding alpaca was evaluated for a 2-day history of lowered head carriage and lethargy. CLINICAL FINDINGS: On initial examination, the alpaca had signs of lethargy and lowered carriage of the head and neck, but no specific neurologic deficits. Medical management improved the clinical signs, but 8 months later, the alpaca developed acute, progressive general proprioceptive ataxia affecting all 4 limbs and was referred for further evaluation and treatment. Magnetic resonance imaging and CT identified disruption of the normal osseous architecture of C7 and T1. Medical management was attempted, but because of a lack of improvement, the patient underwent surgery 14 months after initial examination. TREATMENT AND OUTCOME: A dorsal laminectomy of C7 and T1 via a dorsal midline approach was performed, and the spinous processes of both vertebrae were removed prior to removal of the overlying lamina. Free dorsal expansion of the spinal cord was ensured by resection of the ligamentum flavum. Six months after surgery, the alpaca had returned to successful breeding with 7 hembra bred in the first year after surgery, producing 6 crias, and 4 crias in the second year. The patient was eventually euthanized 28 months after surgery because of neurologic deterioration but was still ambulatory at that time. CONCLUSIONS AND CLINICAL RELEVANCE: A good outcome with adequate alleviation of clinical signs and breeding soundness for > 2 years following dorsal laminectomy was achieved in this camelid patient. The surgical approach was similar to that in other species and was associated with mild postoperative morbidity. Veterinarians treating camelids should be aware of the initial clinical signs and treatment options for cervical vertebral stenotic myelopathy. In acute cases, the signs of reduced cervical mobility and pain on manipulation should prompt investigation including appropriate diagnostic imaging. Timely surgical intervention should be considered in patients that respond poorly to medical treatment to avoid irreversible spinal cord injury and optimize outcome.

Intracerebellar malignant nerve sheath tumor in a child: case report and review of literature.

PURPOSE: Intracerebellar malignant nerve sheath tumor (ICMNST) is an extremely rare entity, only two cases have been reported previously, and this is the first case to be reported in a child. The histogenesis, diagnosis, and management of this entity are very ambiguous, and natural history in a child is unknown. METHODS: The authors report a 7-year-old girl who presented with ataxia and signs of raised intracranial pressure and discuss the challenges in diagnosis, surgical strategy, and treatment. RESULTS: Following gross total resection and radiation to tumor bed, the patient had unremarkable recovery and is recurrence free at 1-year follow-up. CONCLUSION: ICMNSTs are extremely rare tumors of the cerebellum. Preoperative radiological diagnosis is not possible due to its close radiological resemblance to other common posterior fossa tumors. Immunohistochemistry plays a pivotal role in clinching the diagnosis. Though the reported adult counterparts have shown dismal prognosis, the pediatric counterparts may fare better with good surgical resection followed by radiotherapy.

Pseudotumor cerebri presenting with ataxia and hyper-reflexia in a non-obese woman treated with sinus stenting.

AIM: To report a successful treatment of an atypical case of pseudotumor cerebri. BACKGROUND: Pseudotumor cerebri is a disorder of raised intracranial pressure. Multiple Case Series have been published about its treatment with sinus stenting. All these cases have had typical presentations. CASE: An atypical fulminant case of pseudotumor cerebri which was treated successfully with venous sinus stenting. CONCLUSION: Venous sinus stenting can be done effectively in atypical cases of pseudotumor cerebri.

Treatment of tarsal joint deformities with hinged transarticular external fixators in three young birds.

Pelvic limb deformities are common in many avian species. Three young birds, including a six-week-old Cockatoo and two three-month-old goslings, were presented with tarsal joint deformities. They were treated with an experimental prototype of a hinged linear external fixator, placed in a transarticular fashion, in order to maintain joint function during treatment. All birds had close to normal leg function at six to ten weeks postoperatively. These results suggest that the hinged external fixator may be a viable treatment option for tarsal joint deformities in young birds.

Olfactory mucosal transplantation after spinal cord injury improves voiding efficiency by suppressing detrusor-sphincter dyssynergia in rats.

PURPOSE: Several recent studies showed that olfactory mucosal transplantation after spinal cord injury promotes extensive regeneration of the injured spinal cord. We examined the efficacy of olfactory mucosal transplantation for bladder dysfunction after spinal cord injury in rats. MATERIALS AND METHODS: In adult female rats the Th9-10 spinal cord was completely transected, followed by olfactory mucosal transplantation or gelatin sponge filling as the control. Each group was examined by cystometrogram and external urethral sphincter electromyogram. Calcitonin gene-related peptide and growth associated protein 43 double positive expression in the L6/S1 dorsal horn was evaluated by immunohistochemistry. Transplant sites were examined by immunohistochemistry with antibodies against neurofilament M and neuronal class III beta-tubulin. RESULTS: On cystometrogram voiding efficiency was significantly higher in the transplantation group than in controls. On external urethral sphincter electromyogram with simultaneous cystometrogram the transplantation group showed a larger ratio of interburst silent periods to burst activity duration and a greater number of high frequency oscillations. In the transplantation group calcitonin gene-related peptide and growth associated protein 43 double positive expression in the L6/S1 dorsal horn was less dense than in controls. The transplantation group showed strong neurofilament M and neuronal class III beta-tubulin expression at the transplant site. CONCLUSIONS: Olfactory mucosal transplantation after spinal cord injury weakened external urethral sphincter excessive bursting and increased the urethral opening to improve voiding efficiency. Olfactory mucosal transplantation may modify emergence of the spinal micturition reflex after spinal cord injury. Transplantation resulted in new axons growing at the transplant site, implying the possible existence of interneuron bridging across the injured spinal cord.

A 20-year follow-up of the mesh wallstent in the treatment of detrusor external sphincter dyssynergia in patients with spinal cord injury.

OBJECTIVE: To assess the long-term (20 years) effectiveness of the UroLume wallstent(TM) (Pfizer Inc., UK) in the treatment of detrusor external sphincter dyssynergia (DESD) in patients with spinal cord injury (SCI). PATIENTS AND METHODS: Twelve patients with quadriplegia secondary to SCI underwent external striated sphincter stenting with the UroLume wallstent in place of sphincterotomy for DESD ≈ 20 years ago. The mean (range) age was 41.8 (26-65) years. Eleven patients had cervical level injury whilst one had a thoracic injury. All the patients were shown to have high-pressure neurogenic detrusor overactivity and DESD with incomplete emptying on preoperative video-cystometrograms (VCMG). RESULTS: Six of the 12 patients have now been followed-up for a mean (range) of 20 (19-21) years. Of the remaining six, two were lost to follow-up at 1 and 3 years, but both remained free of complications during that time. Two patients developed encrustation causing obstruction, requiring stent removal within 1 year of insertion. Another patient with an adequately functioning stent died 7 years after stent insertion from a chest infection. The twelfth patient developed bladder cancer 14 years after stent insertion and underwent cystectomy with urinary diversion. VCMG follow-up of the six patients showed a significantly sustained reduction of maximum detrusor pressure and duration of detrusor contraction at the 20-year follow-up. Five of these six patients developed bladder neck dyssynergia of varying degrees as shown on VCMG within the first 9 years of follow-up. All were successfully treated with bladder neck incision (BNI) where the last BNI needed was at 12 years. We did not encounter any problem with stent migration, urethral erosion, erectile dysfunction or autonomic dysreflexia. CONCLUSION: Urethral stenting using the UroLume wallstent is effective in the management of DESD in patients with SCI and provides an acceptable long-term (20-year follow-up) alternative to sphincterotomy. The failures manifest within the first few years and can be managed easily with stent removal without any significant problems. Bladder neck dyssynergia was the long-term complication which was treated successfully with BNI. It has no significant interference with erectile function, being reversible, minimally invasive and has a shorter hospital stay.

Embryonic stem cell rescue of tremor and ataxia in myelin-deficient shiverer mice.

Transplantation of neural precursor cells has been proposed as a possible approach for replacing missing or damaged central nervous system myelin. Neonatal and adult myelin-deficient shiverer (shi) mice, bearing a mutation of the myelin basic protein (MBP) gene, have been used extensively as hosts for testing cell engraftment, migration, and myelination, but relatively little progress has been made in reversing shi motor deficits. Here we describe a prenatal cell replacement strategy, showing that embryonic stem cells injected into shi blastocyst embryos can generate chimeric mice with strong and widespread immunoreactive MBP expression throughout the brain and a behavioral (motor) phenotype that appears essentially rescued.