Spontaneous resolution of syringomyelia following pregnancy and parturition in a patient with type I chiari malformation: A case and systematic review.

BACKGROUND: Spontaneous resolution of syringomyelia has rarely been reported in the literature. Rarer still are cases wherein this process is associated with pregnancy and parturition. We review theories on syringomyelia development and spontaneous resolution to better understand the role pregnancy and parturition may play in both processes. METHODS: We present a 30-year-old female with MRI-confirmed spontaneous syrinx regression following caesarean delivery of a full-term pregnancy. We additionally review the literature to identify previously reported cases of spontaneous syrinx regression both independent of and associated with pregnancy. RESULTS: Including the present case, 39 cases describing spontaneous regression of syringomyelia have been reported in the literature, of which only four are associated with pregnancy and parturition. 75% of all reported cases were associated with type I Chiari malformation, though several disorders of the craniocervical junction and spinal canal were implicated. Complete syrinx regression was achieved in 33.3% of cases and 5% of cases described recurrence of syringomyelia following the spontaneous resolution. CONCLUSION: Syringomyelia likely develops due to disturbance of the physiologic flow of cerebrospinal fluid around the craniocervical junction and the obex. Several mechanisms including fissuring of the spinal cord parenchyma and reduction of subarachnoid scarring are likely involved in this process. In the setting of pregnancy, additional mechanisms surrounding the increased intraabdominal forces imparted by a growing fetus, Valsalva-like strain experienced during labor, and hemodynamic changes that occur to accommodate gestation are likely implicated. Nevertheless, patients should continue to be monitored periodically for syrinx recurrence.

Classifications of Odontoid Process Fractures: A Systematic Review and Proposal of a New Simplified Classification System Based on Embryology.

Odontoid fractures are the most common cervical spine fractures in the elderly. Although many classification systems have been developed for them, the ambiguity in various definitions can potentially lead to misunderstandings. This paper aims to review the terminologies and current classification systems of odontoid fractures and propose a new, simplified anatomical classification. Given the descriptive variability of odontoid fractures in current classifications, we systematically reviewed the literature using PRISMA guidelines querying the National Library of Medicine PubMed database. The initial literature search yielded 175 publications. A total of seven reports met the inclusion criteria and were ultimately included for a full review. The classification systems previously used to categorize fractures of the odontoid process often need to be more transparent, imprecise, and incongruous. To simplify them, a new embryologically accurate system is proposed. A new embryological and anatomically-based system, combining the former systems' specific attributes, allows a more straightforward and adaptable classification of odontoid fractures.

The Échancrure of the Uncovertebral Joint: A Forgotten Structure of the C3-C7 Cervical Vertebral Bodies.

Introduction The échancrure (a French term meaning "indentation") of the cervical vertebrae is the poorly defined articular part on the inferolateral aspect of the cervical spine body, which, with the uncinate processes of the associated caudal vertebra, makes up the joints of Luschka (uncovertebral joint). With no known previous studies on the échancrure, the present anatomical study aimed to better elucidate this structure, its prevalence, and its relationships to the adjacent intervertebral foramen and uncinate process. Methods We observed 50 adult cervical spines (100 sides) for the presence of an énchancrure. When an énchancrure was identified, its morphometry was documented and photographed. Measurements included the width and height of the énchancrure. The relationship with the adjacent uncinate process was also studied. Any correlation between the size and shape of the adjacent uncinate process and the énchancrure was recorded. Results Anénchancrure was found at all levels of the cervical vertebrae except at C1 and C7 and was clearly visible on 88% of the sides. The énchancrure, more or less, conformed to the reciprocal shape of the uncinate process, which was found on all sides. The shapes were roughly arched, ovoid, or linear. These structures were always in an anterolateral position on the body of the vertebra and just outside the apophyseal ring. The mean height of the énchancrure was 2.1 mm. The length of the uncinate process correlated positively (r=0.8) to the size of the adjacent énchancrure. The height of the énchancrure was inversely related to the diameter of the adjacent intervertebral foramen. The mean width was 8.3 mm. These structures tended to be largest at C3 and C4 vertebral levels and were smallest at C5 and C6 levels. The énchancrure was most in contact with the uncinate process with lateral flexion of the cervical spine and in specimens with a longer uncinate process, e.g., C6. The énchancrure was also found to be wider in cases of cervical spine degeneration involving the body of the cervical vertebrae. Degeneration of the uncovertebral joint was most often seen at the énchancrure and not at the adjacent uncinate process. Conclusions We found that the énchancrure is found in the majority of cervical spines. These structures tended to be largest at C3 and C4 vertebral levels and were smallest at C5 and C6 levels, and they had more prominence when the adjacent uncinate process was enlarged. The énchancrure should be considered a normal feature of the inferolateral aspect of the cervical vertebrae. Future clinical studies are necessary to better elucidate their functional significance.

Aneurysmal subarachnoid hemorrhage during pregnancy: a comprehensive and systematic review of the literature.

Aneurysmal subarachnoid hemorrhage (aSAH) is an emergent condition requiring rapid intervention and prolonged monitoring. There are few recommendations regarding the management of aSAH in pregnancy. We identified all available literature and compiled management decisions as well as reported outcomes through a systematic literature review without meta-analysis to provide recommendations for management of aSAH during pregnancy. We included a total of 23 articles containing 54 cases of pregnancy-related aSAH in our review. From these reports and other literature, we evaluated information on aSAH pathophysiology, diagnosis, and management with respect to pregnancy. Early transfer to an appropriate facility with neurocritical care, a high-risk obstetric service, and a neurosurgery team available is crucial for the management of aSAH in pregnancy. Intensive monitoring and a multidisciplinary approach remain fundamental to ensure maternal and fetal health.

Cholangiocarcinoma Metastasis to the Spine and Cranium.

Background: Cholangiocarcinoma (CCC), a rare tumor arising from the viscera, has a poor prognosis. Although CCC is prone to metastasis, spread to the cranium and spine is exceedingly rare. Treatment for metastatic disease is palliative, with total resection of the primary lesion the only cure. We describe a case of metastatic CCC to the spine and cranium treated with surgical resection. Case Report: A 61-year-old male with a history of hepatitis C with liver transplant and incidental discovery of CCC presented with gradually increasing back pain. Physical examination revealed a palpable nontender mass in the parieto-occipital area. Computed tomography survey of the spine and head revealed mixed sclerotic and lytic lesions of the T9, T11, L2, and L5 vertebral bodies, a lytic lesion on the T6 vertebral body, and a 1.4-cm lesion in the right occipital calvarium. The patient underwent right occipital craniotomy for excisional biopsy of the calvarial mass with gross total resection and immunohistochemical confirmation of CCC. The patient was started on gemcitabine chemotherapy and radiation therapy for spinal metastases. Three months later, the patient died from metastatic disease complications. Conclusion: To our knowledge, only 6 cases of cranial CCC have been reported, and only 2 reported mixed cranial/spinal involvement. We report a rare case of CCC metastasis to the spine and cranium that was treated with surgery, chemotherapy, and radiotherapy. CCC should be considered an exceedingly rare etiology with treatment options aimed solely at palliation. This case supplements the existing literature to inform medical and surgical decision-making.

Long-term outcomes of deep brain stimulation in severe Parkinson's disease utilizing UPDRS III and modified Hoehn and Yahr as a severity scale.

OBJECTIVES: Deep brain stimulation (DBS) is the surgical treatment of choice for moderate to severe Parkinson's Disease (PD). However, few studies have assessed its efficacy in severe PD as defined by the modified Hoehn and Yahr scale (HY). This study evaluates long-term and medication outcomes of DBS in severe PD. PATIENTS AND METHODS: We retrospectively collected the data of 15 patients from 2008 to 2014 with severe PD treated with DBS. Retrospective assessment with the modified Hoehn and Yahr scale and motor subset of the Unified Parkinson's Disease Rating Scale (UPDRS III) were used to objectively track severity and motor function improvement, respectively. Levodopa equivalence daily doses (LEDD), number of anti-PD medications and number of daily medication doses were used to measure improvements in medication burden. Data was evaluated using univariate analyses, one sample paired t-test, two sample paired t-test, and Wilcoxon signed-rank test. RESULTS: The mean post-operative follow-up was 44.63 months, average age at diagnosis and the average age at time of DBS was 51.3 years and 61.5 years, respectively, and the time from diagnosis to treatment was 13.2 years. Significant decreases were seen in UPDRS III scores (pre-op = 44.533; post-op = 26.13; p = 0.0094), LEDD (pre-op = 1679.34 mg; post-op = 837.48 mg; p = 0.0049), and number of daily doses (pre-op = 21.266; post-op 12.2; p = 0.0046). No significant decrease was seen in the number of anti-PD medications (pre-op = 3.8; post-op = 3.2; p = 0.16). CONCLUSION: Following DBS, severe PD patients demonstrated significant improvements in motor function and medication burden during long-term follow-up. We believe our results prove that DBS is efficacious in the management of severe PD, and that further research should follow to expand DBS criteria to include severe disease.

Evaluation and Management of Spinal Subarachnoid Hemorrhage in a Patient with Lupus Vasculitis.

BACKGROUND Isolated spinal artery subarachnoid hemorrhage is a rare occurrence in the general population, but occurs more commonly as one of many neurologic sequela of systemic lupus erythematosus (SLE). The etiology of a neurologic deficit in an SLE patient is often multifactorial. Comorbid conditions, such as antiphospholipid antibody syndrome, predispose to stroke. Other diagnoses, including transverse myelitis, may also be attributed to local inflammation. CASE REPORT A 37-year-old woman with SLE and antiphospholipid antibody syndrome experienced severe back pain followed by sudden paralysis and sensory loss below the T2 level. She remained alert and oriented on examination, with neurologic exam positive for diminished strength in the arms and with total loss of sensation and strength in the legs. Diagnostic workup was limited due to a contrast allergy and severe lupus nephritis; however, initial imaging showed increased cervical-thoracic spinal cord signal and concern for acute blood in the subarachnoid space. No neurosurgical intervention occurred, and the patient was treated with high-dose steroids and plasmapheresis for a possible transverse myelitis and non-aneurysmal subarachnoid hemorrhage. The patient received further neurologic and rheumatologic workup and remained neurologically stable, with improvement in proximal arm strength on physical exam. CONCLUSIONS We highlight the diagnostic challenges in treating a patient with SLE with acute paralysis and sensory loss. In this case, aggressive early treatment of the patient's myelitis and myelopathy were successful in leading to mild neurological improvement.

Repeated restraint stress exposure during early withdrawal accelerates incubation of cue-induced cocaine craving.

A major challenge for treating cocaine addiction is the propensity for abstinent users to relapse. Two important triggers for relapse are cues associated with prior drug use and stressful life events. To study their interaction in promoting relapse during abstinence, we used the incubation model of craving and relapse in which cue-induced drug seeking progressively intensifies ('incubates') during withdrawal from extended-access cocaine self-administration. We tested rats for cue-induced cocaine seeking on withdrawal day (WD) 1. Rats were then subjected to repeated restraint stress or control conditions (seven sessions held between WD6 and WD14). All rats were tested again for cue-induced cocaine seeking on WD15, 1 day after the last stress or control session. Although controls showed a time-dependent increase in cue-induced cocaine seeking (incubation), rats exposed to repeated stress in early withdrawal exhibited a more robust increase in seeking behavior between WD1 and WD15. In separate stressed and control rats, equivalent cocaine seeking was observed on WD48. These results indicate that repeated stress in early withdrawal accelerates incubation of cocaine craving, although craving plateaus at the same level were observed in controls. However, 1 month after the WD48 test, rats subjected to repeated stress in early withdrawal showed enhanced cue-induced cocaine seeking following acute (24 hours) food deprivation stress. Together, these data indicate that chronic stress exposure enhances the initial rate of incubation of craving during early withdrawal, resulting in increased vulnerability to cue-induced relapse during this period, and may lead to a persistent increase in vulnerability to the relapse-promoting effects of stress.

Sequence determinants of the Caenhorhabditis elegans dopamine transporter dictating in vivo axonal export and synaptic localization.

The monoamine neurotransmitter dopamine (DA) acts across phylogeny to modulate both simple and complex behaviors. The presynaptic DA transporter (DAT) is a major determinant of DA signaling capacity in ensuring efficient extracellular DA clearance. In humans, DAT is also a major target for prescribed and abused psychostimulants. Multiple structural determinants of DAT function and regulation have been defined, though largely these findings have arisen from heterologous expression or ex vivo cell culture studies. Loss of function mutations in the gene encoding the Caenhorhabditis elegans DAT (dat-1) produces rapid immobility when animals are placed in water, a phenotype termed swimming-induced paralysis (Swip). The ability of a DA neuron-expressed, GFP-tagged DAT-1 fusion protein (GFP::DAT-1) to localize to synapses and rescue Swip in these animals provides a facile approach to define sequences supporting DAT somatic export and function in vivo. In prior studies, we found that truncation of the last 25 amino acids of the DAT-1 C-terminus (Δ25) precludes Swip rescue, supported by a deficit in GFP::DAT-1 synaptic localization. Here, we further defined the elements within Δ25 required for DAT-1 export and function in vivo. We identified two conserved motifs (584KW585 and 591PYRKR595) where mutation results in a failure of GFP::DAT-1 to be efficiently exported to synapses and restore DAT-1 function. The 584KW585 motif conforms to a sequence proposed to support SEC24 binding, ER export from the endoplasmic reticulum (ER), and surface expression of mammalian DAT proteins, whereas the 591PYRKR595 sequence conforms to a 3R motif identified as a SEC24 binding site in vertebrate G-protein coupled receptors. Consistent with a potential role of SEC24 orthologs in DAT-1 export, we demonstrated DA neuron-specific expression of a sec-24.2 transcriptional reporter. Mutations of the orthologous C-terminal sequences in human DAT (hDAT) significantly reduced transporter surface expression and DA uptake, despite normal hDAT protein expression. Although, hDAT mutants retained SEC24 interactions, as defined in co-immunoprecipitation studies. However, these mutations disrupted the ability of SEC24D to enhance hDAT surface expression. Our studies document an essential role of conserved DAT C-terminal sequences in transporter somatic export and synaptic localization in vivo, that add further support for important roles for SEC24 family members in efficient transporter trafficking.

Minimally Invasive Transsulcal Resection of Intraventricular and Periventricular Lesions Through a Tubular Retractor System: Multicentric Experience and Results.

BACKGROUND: Conventional approaches to deep-seated cerebral lesions range from biopsy to transcortical or transcallosal resection. Although the former does not reduce tumor burden, the latter are more invasive and associated with greater potential for irreparable injury to normal brain. Disconnection syndrome, hemiparesis, hemianesthesia, or aphasia is not uncommon after such surgery, especially when lesion is large. By contrast, the transsulcal parafascicular approach uses naturally existing corridors and a tubular retractor to minimize brain injury. METHODS: A retrospective review of patients undergoing minimally invasive transsulcal parafascicular resection of ventricular and periventricular lesions, across 5 independent centers, was conducted. RESULTS: Twenty patients with lesions located in the lateral ventricle (n = 9), the third ventricle (n = 6) and periventricular region (n = 4) are described in this report. Average age was 64 years (8 male/12 female). The average depth from cortical surface was 4.37 cm. A 13.5-mm-diameter tubular retractor (BrainPath [NICO Corporation, Indianapolis, Indiana, USA]) of differing lengths was used, aided by neuronavigation. Gross total resection was obtained in 17 patients. Pathologies included colloid cyst, subependymoma, glioma, meningioma, central neurocytoma, lymphoma, and metastasis. Three patients experienced transient morbidity: memory loss (2), hemiparesis (1). One patient died 3 months postoperatively as a result of unrelated pulmonary illness. Follow-up ranged from 6 to 27 months (average, 12 months). CONCLUSIONS: This technique is safe and effective for the treatment of intraventricular and periventricular lesions. Surgery-related morbidity is minimal and often transient. Lesions are satisfactorily resected and residuum occurs only when the neoplasm involves vital structures. The tubular retractor minimizes trauma to brain incident in the surgeon's path.