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Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration. It is caused by a damage at the Guillain-Mollaret triangle (GMT), which is defined by three anatomical structures: the dentate nucleus, the red nucleus, and the inferior olivary nucleus (ION). Clinically, it may be revealed by palatal myoclonus. On MRI, it appears as a unilateral or bilateral enlargement of the inferior olivary nucleus which shows a high signal intensity on T2-weighted images, with sometimes a cerebellar atrophy. Here we report 2 cases of healthy patients which present hemorrhagic brainstem cavernomas, complicated later by the development of palatal myoclonus and cerebellar ataxia, with MRI features corresponding to an (HOD) secondary to a (GMT) cavernoma. The purpose is to explain the mechanism of (HOD) subsequent to lesion in (GMT), and to describe magnetic resonance imaging features.

An 8-year-old boy presenting with left-angle paralysis, tremor in upper and lower extremities, and diplopia was diagnosed with hemorrhage from a mesencephalic cavernous hemangioma. He underwent hemangiomectomy through the occipital transtentorial approach 4 weeks post-hemorrhage, after which Holmes tremor (HT) markedly reduced. A year later, hemangioma has not recurred; he is now independent in his daily activities. Early intervention in the subacute stage allows for the complete removal of brainstem cavernomas (BSCs), with minimal risk of complications or sequelae. Proper timing and surgical approach for BSCs can prevent re-bleeding and improve HT after an initial hemorrhage, without any lasting negative consequences. [ABSTRACT FROM AUTHOR]

Introduction: The resection of brainstem cerebral cavernous malformations (CCM) harbors the risk of damaging the corticospinal tract (CST) and other major tracts. Hence, visualization of eloquent fiber tracts supports pre- and intraoperative planning. However, diffusion tensor imaging fiber tracking at brainstem level suffers from distortion due to field inhomogeneities and eddy currents by steep diffusion gradients. Research question: This study aims to analyze the effect of distortion correction for CST tractography in brainstem CCM patients. Material and methods: 25 patients who underwent resection of brainstem CCM were enrolled, 24 suffered from hemorrhage. We performed an anatomically based tractography of the CST with a mean minimal fractional anisotropy of 0.22 ± 0.04 before and after cranial distortion correction (CDC). Accuracy was measured by anatomical plausibility and aberrant fibers. Results: CDC led to a more precise CST tractography, further approximating its assumed anatomical localization in all cases. CDC resulted in a significantly more ventral location of the CST of 1.5 ± 0.6 mm (6.1 ± 2.7 mm before CDC vs. 4.6 ± 2.1 mm after CDC; p

Background: "Thirteen-and-a-half" is a newly described clinical syndrome characterized by the combination of the one-and-a-half syndrome with fifth and seventh cranial nerve nuclei involvement (11/2 + 5 + 7 = 131/2). To the authors' knowledge, this is the first report of the thirteen-and-a-half syndrome secondary to pontine cavernoma and, overall, only the second reported case of this syndrome in the literature till date. Case Report: A 20-year-old man presented with the clinical features suggestive of the thirteen-and-a-half syndrome, explained radiologically by pontine cavernoma. We operated him using a suboccipital transvermian approach and he is doing well at 2.5 years follow-up. Interestingly, his one-and-a-half syndrome has partially improved to left horizontal gaze palsy. Conclusion: The clinical appreciation of the thirteen-and-a-half syndrome precisely localizes the lesion to ipsilateral dorsal pontine tegmentum. Neurosurgeons must be aware of the newly described "one-and-a-half- plus" syndromes as they help in a better understanding of pathoanatomy caused by different disease processes in the brainstem. [ABSTRACT FROM AUTHOR]

Cluster headaches are characterized by painful unilateral periorbital attacks accompanied by dysautonomic symptoms. Although cluster headaches are classified as a primary headache disorder, a few cases of inflammatory, vascular or neoplastic conditions have been reported to cause or mimic cluster headaches. This raises the question as to the possible causal relationship between symptoms and an underlying structural lesion that may potentially interfere with the trigemino-autonomic reflex.We hereby report the first pediatric case of a brainstem cavernoma causing cluster headache-like symptoms. Of note, the child experienced pain relief after surgery.With regards to the pathophysiology, the observed ptosis observed in our child during the painful crises might be explained by mass effect of the cavernorma on the cisternal portion of the third cranial nerve. In addition, chronic inflammation of mesencephalic and motor nuclei of the trigeminal nerve by thalamo-pedoncular edema surrounding the lesion may have participated to painful pathways.This case illustrates that cluster headaches may occasionally be secondary to an underlying lesion. This advocates the need for MRI screening, especially as neurosurgical intervention may offer significant symptomatic relief and an improvement in the quality of life of these patients.

Background: Holmes tremor is a rare symptomatic movement disorder, characterized by a combination of resting, postural, and intention tremor. It is usually caused by lesions in the brainstem, thalamus, and cerebellum. Despite pharmacological advances, its treatment remains a challenge; many medications have been used with various degrees of effectiveness. Stereotactic thalamotomy and deep brain stimulation in the ventralis intermedius nucleus have been effective surgical procedures in cases refractory to medical treatment. Case Report: Here we report a young woman with topiramate-responsive Holmes tremor secondary to a brainstem cavernoma. Discussion: Herein we report a Holmes tremor responsive to Topiramate.

We present the case of a 64-year-old female, with a 5-year history of symptomatic multiple cerebral cavernomas, who developed paresthesia in the right leg and arm, followed by vertigo, slurred speech and forward gaze diplopia. Brain investigation revealed two lesions with supra- and infratentorial localization with signs of recent hemorrhage of different ages. The localization of the lesions in the midpoints and the basal ganglia imposed a conservative approach.

Surgical resection of brainstem cavernous malformations (BCMs) is a high-risk procedure and can be challenging to the neurosurgeon. Lateral surgical routes are becoming increasingly used to approach ventrolaterally brainstem cavernoma. Surgical approach decision depends on the location of the cavernoma in the brainstem and a possible association with brainstem developmental venous anomalies (DVAs). DVA can affect the formation and clinical course of cavernous malformation (CM). CMs related to DVAs tend to have more aggressive behavior than isolated CM. In cases of DVAs associated with hemorrhage, CMs are most often the site of bleeding rather than DVAs themselves. In this case report, we present a 24-year-old woman with a pontomedullary CM and associated dorsally located DVA. BCM was operated through a far lateral suboccipital craniotomy. Brainstem entry point was at inferior olive with extension to the pontomedullary sulcus. This approach should be preferred as a safe surgical exposure to the central and paramedian pontomedullary cavernoma, especially in the cases with associated intraparenchymal brainstem DVA. Such surgical exposure allows preservation of the concomitant brainstem DVA. [ABSTRACT FROM AUTHOR]

Brainstem cavernoma is a challenging neurosurgical pathology and microsurgery remains the only treatment option. Although the decision-making between interventional and conservative approach to this disease may be complex, malformations presenting multiple bleedings are usually good candidates for surgery. 1 On the other hand, microsurgical resection of cavernomas can offer an effective resolution with acceptable morbidity. In this video, we present a case of pontine cavernoma with multiple hemorrhages in a young patient. The anatomical characteristic of the lesion defines the best suitable craniotomy for surgery. In this case, an anterior petrosal approach 234 was used to access the peritrigeminal area and safely perform the resection. Anatomical considerations are described on this skull base approach along with the rationale and benefits of this exposure. Electrophysiological neuromonitoring is essential for this kind of procedure and preoperative tractography also enabled the best understanding of the disease. Finally, we also discuss alternative managements and potential complications. 5 With the patient's consent, we also show the excellent clinical evolution after few weeks of recovery and the restoration of the corticospinal tract, previously displaced by the cavernoma, to its original position. [ABSTRACT FROM AUTHOR]

We present the case of a 64-year old female, with a 5 year history of symptomatic multiple cerebral cavernomas, who developed paresthesia in the right leg and arm, followed by vertigo, slurred speech and forward gaze diplopia. Brain investigation revealed two lesions with supra- and infratentorial localization with signs of recent hemorrhage of different ages. The localization of the lesions in the midpons and the basal ganglia imposed a conservative approach. [ABSTRACT FROM AUTHOR]

Abstract Mesencephalic cavernous malformations (MeCMs) account for 4 to 35% of the cavernous malformations of the central nervous system and are generally rare. Surgical resection of brainstem cavernomas are high-risk procedures and can be challenging to the neurosurgeon. Several approaches have been described, but the approach must allow for a straight line of sight in which the surgeon, the pial incision, and the MeCM are all collinear. This alignment provides the best view of the lesion while minimizing the need for brainstem retraction. The pial incision should be chosen to minimize the distance to the lesion while avoiding critical nuclei and tracts. In this case report, we present a 34-year-old woman with a MeCM resected by an anterior transcallosal transchoroidal approach with minimal damage to surrounding brain tissue. Although rarely used, it should be considered a valuable alternative to ventrally located brainstem cavernomas.

Objective In this study, we investigated the potential prognostic role of morphologic and quantitative diffusion tensor imaging (DTI) in patients with brainstem cavernoma (BSC) in terms of postoperative outcome. Methods In this retrospective study of 14 brainstem cavernoma patients, we analyzed pre- and postoperative DTI data. White matter tractography of corticospinal tracts (CSTs) was performed with the Fiber Assignment by Continuous Tracking algorithm, and morphologic characteristics of CSTs were compared with clinically assessed motor strength. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were measured in ipsi- and contralesional regions of interest at the lesion level, as well as levels caudal and rostral to the lesion. Correlation analysis was performed between lateral index (LI) of ipsi-/contralateral FA and ADC values and patients' motor function. Results Preoperatively, normal morphologic features of CSTs corresponded to normal motor function in 4 patients. The other 10 morphologically abnormal preoperative CSTs didn't show corresponding motor impairment either in pre- or postoperative follow-up period. The sensitivity, specificity, positive predictive, and negative predictive values of white matter tractographic morphology on preoperative motor function were 100%, 57.1%, 70%, and 100%, respectively. The corresponding values on follow-up motor function were 100%, 33.3%, 20%, and 100%, respectively. The mean FA at lesion level was significantly lower than the corresponding FA at the contralateral hemisphere ( P = 0.009). In areas caudal to the lesion the same trend yet without statistic significance was observed ( P = 0.23). There was no significant laterality difference of mean FAs rostral to the level of the lesion. No correlation in LI of FA or ADC in the 3 anatomic levels with simultaneous and long-term follow up motor function was observed. Restoration of the morphology of the affected CST postoperatively was associated with a trend for decreasing ADC compared with the preoperative measurements. Conclusion Intact CST morphology in DTI predicts a favorable postoperative outcome in patients with BSC. Interrupted CSTs and decreased FA values correlate well within BSC lesion level; nevertheless, morphologic characteristics and diffusion parameter changes at lesion level cannot predict poor prognosis. Caudal and rostral diffusion parameters can provide more information of the integrity of CSTs compared with morphologic study alone. [ABSTRACT FROM AUTHOR]

The spatial complexity of highly vulnerable structures makes surgical resection of brainstem cavernomas (BSC) a challenging procedure. Diffusion tensor imaging (DTI) allows for the visualization of white matter tracts and enables a better understanding of the anatomical location of corticospinal and sensory tracts before and after surgery.We investigated the feasibility and clinical usefulness of DTI-based fiber tractography in patients with BSC.Pre- and postoperative DTI visualization of corticospinal and sensory tracts were retrospectively analyzed in 23 individuals with BSC. Preoperative and postoperative DTI-fiber accuracy were associated to the neurological findings. Preoperatively, the corticospinal tracts were visualized in 90 % of the cases and the sensory tracts were visualized in 74 % of the cases. Postoperatively, the corticospinal tracts were visualized in 97 % of the cases and the sensory tracts could be visualized in 80 % of the cases. In all cases, the BSC had caused displacement, thinning, or interruption of the fiber tracts to various degrees. Tract visualization was associated with pre- and postoperative neurological findings. Postoperative damage of the corticospinal tracts was observed in two patients. On follow-up, the Patzold Rating (PR) improved in 19 out of 23 patients (83 %, p = 0.0002).This study confirms that DTI tractography allows accurate and detailed white matter tract visualization in the brainstem, even when an intraaxial lesion affects this structure. Furthermore, visualizing the tracts adjacent to the lesion adds to our understanding of the distorted intrinsic brainstem anatomy and it may assists in planning the surgical approach in specific cases. [ABSTRACT FROM AUTHOR]

Cavernous haemangiomas, or cavernous malformations, have been reported during pregnancy, most of which have been either supratentorial or spinal lesions. We encountered a 15-year old pregnant patient with a rapidly progressive and haemorrhagic brainstem cavernous haemangioma. The case presented here describes the history and findings of this patient, as well as the less-commonly utilized technique we used to access the floor of the fourth ventricle via occipital craniotomy for complete macroscopic resection of this lesion, resulting in the gradual return of most of her neurological deficits. [ABSTRACT FROM PUBLISHER]

Abstract: Objective: Cerebral cavernous malformations (CCMs) and especially cavernous malformations (CMs) in highly eloquent brain areas such as brainstem CMs are rare but possible events during pregnancy. Due to the few published cases in literature clear recommendations regarding the management are rare. In this study we evaluate the proceeding decision in pregnant patients with highly eloquent brainstem CMs. Methods: In our series 43 patients with CMs in highly eloquent brain areas, including 39 patients with brainstem CMs, were surgically treated by the senior author between July 2007 and July 2010. Out of these, 29 patients were female and three of them presented with a symptomatic brainstem CMs during pregnancy and were included in this study. According to our experiences and to the available literature we analyzed demographic and clinical variables to provide recommendations for the management of pregnant patients with highly eloquent brainstem CMs. Results: Only one patient was operated during pregnancy the other two patients were surgically treated after delivery, respectively. A thorough review of the literature revealed 12 patients with brainstem cavernomas during pregnancy there of only two patients were operated during pregnancy. Conclusion: Surgical treatment during pregnancy is rarely required, but needs to be performed right away in life-threatening and rapidly progressive clinical situations. Pregnant women with CMs in highly eloquent brain areas such as brainstem CMs need to be treated in specialized centers to assess the best point of time for surgery. Our study offers a useful tool to support the proceeding decision in this rare but important situation. [Copyright &y& Elsevier]

This study reviewed surgical experience with brainstem cavernomas in an attempt to define optimal surgical approaches and risks associated with surgical management. Clinical courses were retrospectively reviewed for 36 consecutive patients (12 men, 24 women; mean age, 42 years) who underwent microsurgical resection of brainstem cavernomas between 1996 and 2006. Medical records, surgical records, and neuroimaging examinations were evaluated. All 36 patients presented with ≥1 hemorrhage from the cavernomas and preoperatively displayed some neurological symptoms. Surgical approach was midline suboccipital for 16 pontine and/or medullary cavernomas under the floor of the fourth ventricle, retrosigmoid for 10 lateral mesencephalic, pontine, and/or medullary cavernomas, occipital transtentorial for 2 thalamomesencephalic and 3 mesencephalic cavernomas, combined petrosal for 2 pontine cavernomas, and other for 3 cavernomas. Complete resection according to postoperative magnetic resonance imaging was achieved in 33 of 36 patients. No mortality was encountered in this study. New neurological deficit occurred in the early postoperative period for 18 patients, but was transient in 15 of these. Neurological state as of final follow-up was improved in 16 patients (44%), unchanged in 17 (47%), and worsened in 3 (8%) compared with preoperatively. In conclusion, symptomatic brainstem cavernomas should be considered for surgical treatment. Careful selection of the optimal operative approach and a meticulous microsurgical technique are mandatory. [ABSTRACT FROM AUTHOR]

The risk of leaving a remnant after surgery for a cavernous malformation in the brainstem is generally not stressed enough, even though such remnants appear to have a high risk of re-bleeding. At least 40% of known cavernoma remnants after surgery have further bleeding episodes. A retrospective analysis of 30 patients with brainstem cavernoma who underwent surgery is presented, focusing on incidence, risk factors and management of post-surgical residuals. The sites were, medulla in three patients, pons-medulla in four, pons in 16, pons-midbrain in four and midbrain in three. All 30 patients came to our clinical observation with at least one episode of acute-onset neurological deficit and all were operated in the sub-acute phase. Only one patient had a worse stable outcome than the pre-surgical state, and 29 did better or were stable. All patients had a brain MRI scan within 72 h after surgery to confirm that complete removal had been achieved. In three, although the surgical cavity and its border appeared clean at the end of surgery, with no lesion remaining, post-operative MRI detected a residuum. These three patients were re-operated, but one had a further bleed prior to excision. In our series, the surgical finding of a multi-lobular cavernoma (as opposed to the more frequent finding of a discrete lesion with a thick capsule), with a thin wall and satellite nodules separated by a thin layer of apparently intact white matter, was common (seven patients). This group included the three patients with evidence of residuum on post-operative MRI. In our experience, the surgical finding of a multi-lobular cavernoma carries a higher risk of residuum and post-surgical re-bleeding. Immediate post-operative brain MRI scans are therefore strongly recommended for their detection, especially in this group of patients, and if a residual is detected early re-intervention is less risky than the natural history. [ABSTRACT FROM AUTHOR]

The purpose of this study was to present our experience in the management of spontaneous brainstem hematomas (BSH). Records of 58 consecutive patients were reviewed, including demographic data, symptoms, Glasgow Coma Scale, treatment, intraoperative findings (in surgical cases), and outcome according to the Glasgow Outcome Scale. Fifteen patients were comatose (GCS 4 or less): 11/15 patients were treated conservatively. Four patients with accompanying acute occlusive hydrocephalus were treated by placement of an external ventricular drainage. None survived. In nine patients (60%), arteriosclerosis and/or long-standing arterial hypertension were known and arteriopathic BSH was suspected. Forty-three patients were not comatose: 37 patients showed no impairment of consciousness (GCS 15), 6 patients presented with mild disturbance of conscious state (GCS 13), progressing to coma (GCS 8) in 1. In the majority (36/43) of the non-comatose patients (83.7%) cavernoma could be revealed and removed surgically. In six patients (14%), an atypically located arteriopathic BSH was assumed and treated medically. One patient had an underlying brainstem arteriovenous malformation and was treated radiosurgically. Many arteriopathic BSH cause immediate coma indicating direct and irreversible damage of midpontine structures. Thus, we suggest not to proceed to surgery, even if the bleeding is accompanied by acute hydrocephalus. The majority of BSH not resulting in immediate coma are caused by underlying cavernomas. In these cases surgery should be considered. [ABSTRACT FROM AUTHOR]

Cerebellar developmental venous anomalies (CDVA) are benign conditions, although sometimes they are associated with haemorrhages and reported to be symptomatic. This is the largest follow-up study to investigate the symptomatology of CDVAs and their association with other malformations. Thirty-two patients were followed for 2 - 9 years. Twenty-eight had isolated asymptomatic CDVA without any neurological condition during follow-up, which might be linked to the CDVA. Four patients had CDVA and an associated vascular pathology: two pontine cavernomas, one asymptomatic arteriovenous malformation (this is the first published case in the literature) and one cerebellar infarct with a developmental variation of the posterior fossa venous circulation. One patient had two CDVAs, while another had a unique draining vein from the upper part of the brainstem too. In conclusion, CDVAs are benign, asymptomatic conditions, but they are sometimes associated with pathogenic malformations requiring detailed neuroradiological investigations. [ABSTRACT FROM AUTHOR]

Given their highly eloquent location, surgical morbidity of brainstem cavernomas (BSCs) remain significant. Currently, there are no clinical factors or imaging techniques that can reliably predict post-operative motor improvement. Diffusion-tensor imaging (DTI) might have some prognostic value. We present a case supporting the use of DTI as predictor of motor recovery following brainstem surgery. [ABSTRACT FROM AUTHOR]

Background: Safe and complete surgical resection of brainstem cavernoma is difficult without injury surrounding normal structures because the lesions are deep and intra-axial. In this article, the authors describe the “microroll retractor” technique for brainstem cavernoma surgery. Methods: The microroll retractor is made of expanded polytetrafluoroethylene sheet. The sheet is cut in several sizes of square piece and transformed into a tubular shape. We insert this roll retractor via minimal brain incision and keep surgical corridor without usual spatulas during lesionectomy. Results: This technique was adopted for 7 patients with brainstem cavernomas. In all patients, we achieved total excision; improvement was recorded in 6 patients, and no change was recorded in 1 patient. Conclusions: The microroll retractor is extremely simple but provides enough surgical corridors for safe total resection of brainstem cavernomas. [ABSTRACT FROM AUTHOR]