Update: L-Carnitine

L-Carnitine

Forty patients with severe traumatic brain injury were randomized into 2 groups. The l-carnitine (LCA-) group received standard treatment with placebo while the (LCA+) group received l-Carnitine 2g/day for one week. Neuron specific enolase (NSE) was measured on days 1, 3 and 7 after the initiation of the study. Neurocognitive and neurobehavioral disorders were recorded on the first and third months.

Neurocognitive function and NSE significantly improved within one week in both groups. Patient mortality was similar in LCA+ and LCA- groups (P value: 0.76). Brain edema was present in 7 patients in LCA+ group and 13 patients in LCA-group (P value: 0.044). While there was no difference in NSE levels between the two groups. Neurological function was preserved in the LCA+ group with an exception of attention deficit, which was frequent in the LCA+ group.

Mahmoodpoor et al. concluded that despite improvements in neurobehavioral function and the degree of cerebral edema, 7-days of treatment with l-Carnitine failed to reduce serum NSE levels or improve mortality rate at 90days in patients with TBI 1).


There is evidence in the literature for mitochondrial dysfunction in Parkinson’s disease as well as fatty acid beta-oxidation, involving l-carnitine.

Gill et al. investigated l-carnitine in the context of microglial activation, suggesting a potential new strategy of supplementation for PD patients. Preliminary results from this studies suggest that the treatment of activated microglia with the endogenous antioxidant l-carnitine can reverse the effects of detrimental neuroinflammation in vitro 2).

1)

Mahmoodpoor A, Shokouhi G, Hamishehkar H, Soleimanpour H, Sanaie S, Porhomayon J, Rasouli F, Nader ND. A pilot trial of l-carnitine in patients with traumatic brain injury: Effects on biomarkers of injury. J Crit Care. 2018 Feb 9;45:128-132. doi: 10.1016/j.jcrc.2018.01.029. [Epub ahead of print] PubMed PMID: 29454227.
2)

Gill EL, Raman S, Yost RA, Garrett TJ, Vedam-Mai V. l-Carnitine Inhibits Lipopolysaccharide-Induced Nitric Oxide Production of SIM-A9 Microglia Cells. ACS Chem Neurosci. 2018 Jan 31. doi: 10.1021/acschemneuro.7b00468. [Epub ahead of print] PubMed PMID: 29370524.

Update: White cord syndrome

White cord syndrome

Presence of intramedullary MRI hyperintensity signal on T2 weighted image in a patient with unexplained neurological deficits following a spinal cord decompression.

Epidemiology

“White cord syndrome” is a very rare condition.

Etiology

It is thought to be the result of acute reperfusion of chronically areas of spinal cord ischemia.

Diagnosis

Its hallmark is the presence of intramedullary MRI hyperintensity signal on T2 weighted image in a patient with unexplained neurologic deficits following a spinal cord decompression.

Treatment

In previous reports patients have improved following steroid therapy and acute rehabilitation 1).

Case reports

2018

Antwi et al. report an additional case of this complication in a 68-year-old man who developed acute left-sided hemiparesis after posterior cervical fusion for cervical spondylotic myelopathy. The patient improved with high dose steroid therapy 2).

2017

A 64-years old male patient with severe neck pain irradiated to both arms, gait disorder and urinary incontinence. He showed spastic tetraparesis, grip weakness and positive bilateral Hoffman sign, with a Nurick scale score of 3 and a Japanese Orthopaedic Association scale (JOA) of 13, Grade I. MRI imaging documented multiple cervical stenosis with voluminous C3–C4 and C5–C6 disc herniations associated to T2-hyperintense myelomalacic area at C3–C4 level.

Patient underwent double-level ACDF with microsurgical discectomy according to Smith Robinson technique and following anterior arthrodesis, first in C5–C6 with the placement of a titanium cage with intrabody screws (Zero P®, Depuy Synthes – Johnson & Johnson – US), then in C3–C4 level with a stand-alone titanium cage (Cervios®, Depuy Synthes – Johnson & Johnson – US). A diamond drill was used to remove osteophythes in both interbody spaces so to increase spinal cord decompression. An autologous fibrin glue was used to ameliorate haemostasis and fusion.

No surgical, nor anaesthesiological complications were observed, all neural structures were respected and intra-operative x-ray showed the correct placement of both cages. During the closure time of the superficial planes, somatosensory and motor evoked potentials suddenly decreased in voltage. When awakened, the patient showed a severe tetraparesis with complete paraplegia and severe motor weakness to upper limbs with diffuse spastic hypertonia.

A neck collar was then placed and an immediate cervical-spine CT imaging confirmed the correct execution of ACDF.

A following cervical MRI showed an enlarged T2-hyperintense area in C5–C6 level

This ischemic-edematous lesion was supposed to be a case of “white cord syndrome” imputable to a mechanism of improper cord reperfusion. A two-days NASCIS III protocol was then performed.

Three days after, a partial recovery in prehensile strength on the right hand (3/5 Medical Research Council Scale, MRC), a partial recovery in flexion of right arm (2/5 MRC), and in flexion of both legs on thighs (2/5 MRC) were observed.

Seven days after the procedure the patient was transferred to a high specialized Rehabilitation Unit with a Nurick score of 4 and a JOA of 6 3).

2013

Chin et al. report a case of complete loss of somatosensory evoked potentials (SSEPs) during elective ACDF at C4-5 and C5-6 followed by postoperative C6 incomplete tetraplegia without any discernible technical cause. A postoperative MRI demonstrated a large area of high signal changes on T2-weighted MRI intrinsic to the cord “white cord syndrome” but no residual compression. This was considered consistent with spinal cord gliosis with possible acute edema. The acute decompression of the herniated disc resulted in cord expansion and rush-in reperfusion. We postulate that this may have led to disruption in the blood brain barrier (BBB) and triggered a cascade of reperfusion injuries resulting in acute neurologic dysfunction. At 16 months postoperatively our patient is recovering slowly and is now a Nurick Grade 4 4).

References

1) , 2)

Antwi P, Grant R, Kuzmik G, Abbed K. “White Cord Syndrome” of Acute Hemiparesis after Posterior Cervical Decompression and Fusion for Chronic Cervical Stenosis. World Neurosurg. 2018 Feb 13. pii: S1878-8750(18)30296-1. doi: 10.1016/j.wneu.2018.02.026. [Epub ahead of print] PubMed PMID: 29452319.
4)

Chin KR, Seale J, Cumming V. “White cord syndrome” of acute tetraplegia after anterior cervical decompression and fusion for chronic spinal cord compression: a case report. Case Rep Orthop. 2013;2013:697918. doi: 10.1155/2013/697918. Epub 2013 Mar 4. PubMed PMID: 23533882; PubMed Central PMCID: PMC3603640.

12th Asian Congress of Neurological Surgeons Conference

Dear Friends & Colleagues,

On behalf of the organizing and scientific committee, it is my great pleasure and honor to invite you to attend the 12th Asian Congress of Neurological Surgeons Conference to be held in Dubai in 2018. This will inaugurate the first ACNS Conference to be held in this region of Asia. It reflects the close collaboration with World Federation of Neurosurgical Societies and adjoined with the 2nd Interim Meeting of World Federation of Skull Base Societies.

This comprehensive international academic endeavor will focus on teaching and education of young neurosurgeons toward excellence in patient care with the motto “inspiration & innovation”. It will encompass special lectures by distinguished guest speakers, panel discussion, workshops, and video presentations among other scientific activities that will infuse the contributions of young neurosurgeons with a focus on multidisciplinary approaches and contemporary evidence-based clinical practice. I am proud of and honored by the global collaboration with leading societies and organizations. I would like to extend my sincere appreciation for their anticipated profound scientific contributions.

The conference will provide an excellent milieu for academic inspiration; it will be complemented by a state-of-the art exhibition and sponsored by leading companies in the field of medical and surgical technology. Dubai is a vibrant, cosmopolitan city that offers an attractive cultural experience with unique opportunities to appreciate the fusion of its mystic history and modernism.

Imad N. Kanaan, MD, FACS, FRCS (Edin.)

President, ACNS Conference 2018

Ophthalmic artery aneurysm surgery

Ophthalmic artery aneurysm surgery

The ophthalmic artery aneurysms can treated safe and effective through a frontolateral approach 1).

The most important risk associated with clipping ophthalmic artery aneurysms is a new visual deficit. Meticulous microsurgical technique is necessary during anterior clinoidectomy, aneurysm dissection, and clip application to optimize visual outcomes, and aggressive medical management postoperatively might potentially decrease the incidence of delayed visual deficits. As the results of endovascular therapy and specifically flow diverters become known, they warrant comparison with these surgical benchmarks to determine best practices 2).

For ophthalmic artery aneurysm treatment if necessary, the ophthalmic artery may be sacrificed without worsening of vision in the vast majority.

Surgery is technically demanding because these aneurysms are often large and may extend into the cavernous sinus 3) 4) 5) 6) 7) 8).

Care must be taken to avoid optic nerve injury caused by the retraction and/or the heat of the drill 9).

For unruptured intracranial aneurysm, drill off anterior clinoid process via an extradural approach before opening dura to approach aneurysm neck maybe safe. Not for ruptured.

Cutting the falciform ligament early decompresses the optic nerve, and helps minimize worsening of visual impairment from surgical manipulation.

In most cases, a side angled clip can be placed paralell to the parent artery along the neck of the aneurysm 10).

Contralateral approach

Case series

2018

Kamide et al. retrospectively reviewed results from microsurgical clipping of 208 OphA aneurysms in 198 patients.

Patient demographics, aneurysm morphology, clinical characteristics, and patient outcomes were recorded and analyzed.

Despite 20% of these aneurysms being large or giant in size, complete aneurysm occlusion was accomplished in 91% of 208 cases, with OphA patency preserved in 99.5%. The aneurysm recurrence rate was 3.1% and the retreatment rate was 0%.

Good outcomes (modified Rankin Scale score 0-2) were observed in 96.2% of patients overall and in all 156 patients with unruptured aneurysms. New visual field defects (hemianopsia or quadrantanopsia) were observed in 8 patients (3.8%), decreased visual acuity in 5 (2.4%), and monocular blindness in 9 (4.3%). Vision improved in 9 (52.9%) of the 17 patients with preoperative visual deficits.

The most important risk associated with clipping OphA aneurysms is a new visual deficit. Meticulous microsurgical technique is necessary during anterior clinoidectomy, aneurysm dissection, and clip application to optimize visual outcomes, and aggressive medical management postoperatively might potentially decrease the incidence of delayed visual deficits. As the results of endovascular therapy and specifically flow diverters become known, they warrant comparison with these surgical benchmarks to determine best practices 11).

2017

The clinical data of 95 patients with carotid ophthalmic artery aneurysms treated via frontolateral approach in the last 1.5 years in Beijing Tiantan Hospital and Beijing Anzhen Hospital were analyzed retrospectively.Before the operation, digital subtraction angiogram (DSA) was performed among all patients.The patients were divided into two groups by the lateral approach.According to preoperative classification, surgical characteristics and prognosis were summarized.

Ninety-five cases of ophthalmic aneurysms were divided into type Ⅰ of 44 cases (46.3%), type Ⅱ of 34 cases (35.7%) and type Ⅲ of 17cases (17.9%), according to the results of DSA.The diameter of aneurysm was <10 mm (35 cases), 10-25 mm (34 cases), and >25 mm (26 cases). In the 17 cases of subarachnoid hemorrhage (SAH), 8 cases were ruptured carotid-ophthalmic artery aneurysms.Among those 95 patients, 93 were clipped successfully, 2 was trapped.Multiple aneurysms in 5 cases were treated in one surgical session through the same approach.No aneurysm residual was found after postoperative CTA review.Ipsilateral vision of 3 cases were decline.Cerebral infarction was appeared in 9 cases.All the others had a good recovery.

The carotid-ophthalmic artery aneurysms could be well exposed. Microsurgery through frontolateral approach has the advantages such as minimal invasion, less effect on the patients’ look and simple procedure.The frontolateral approach is safe and effective in surgery for ophthalmic segment of the internal carotid artery aneurysms 12).

Case reports

Rustemi et al. illustrated the first case of indocyanine green videoangiography (ICG-VA) application in an optic penetrating ophthalmic artery aneurysm treatment. A 57-year-old woman presented with temporal hemianopsia, slight right visual acuity deficit, and new onset of headache. The cerebral angiography detected a right ophthalmic artery aneurysm medially and superiorly projecting. The A1 tract of the ipsilateral anterior cerebral artery was elevated and curved, being suspicious for an under optic aneurysm growth. Surgery was performed. Initially the aneurysm was not visible. ICG-VA permitted the transoptic aneurysm visualization. After optic canal opening, the aneurysm was clipped and transoptic ICG-VA confirmed the aneurysm occlusion. ICG-VA showed also the slight improvement of the optic nerve pial vascularization. Postoperatively, the visual acuity was 10/10 and the hemianopsia did not worsen.

The elevation and curve of the A1 tract in medially and superiorly projecting ophthalmic aneurysms may be an indirect sign of under optic growth, or optic splitting aneurysms. ICG-VA transoptic aneurysm detection and occlusion confirmation reduces the surgical maneuvers on the optic nerve, contributing to function preservation 13).

1) , 12)

Wang JT, Kan ZS, Wang S. [Surgical management of ophthalmic artery aneurysms via minimally invasive frontolateral approach]. Zhonghua Yi Xue Za Zhi. 2017 Apr 18;97(15):1179-1183. doi: 10.3760/cma.j.issn.0376-2491.2017.15.014. Chinese. PubMed PMID: 28427127.
2) , 11)

Kamide T, Tabani H, Safaee MM, Burkhardt JK, Lawton MT. Microsurgical clipping of ophthalmic artery aneurysms: surgical results and visual outcomes with 208 aneurysms. J Neurosurg. 2018 Jan 26:1-11. doi: 10.3171/2017.7.JNS17673. [Epub ahead of print] PubMed PMID: 29372879.
3)

Hosobuchi Y. Direct surgical treatment of giant intracranial aneurysms. J Neurosurg. 1979;51(6):743–756.
4)

Sundt T M Jr, Piepgras D G. Surgical approach to giant intracranial aneurysms. Operative experience with 80 cases. J Neurosurg. 1979;51(6):731–742.
5)

Almeida G M, Shibata M K, Bianco E. Carotid-ophthalmic aneurysms. Surg Neurol. 1976;5(1):41–45.
6)

Kattner K A, Bailes J, Fukushima T. Direct surgical management of large bulbous and giant aneurysms involving the paraclinoid segment of the internal carotid artery: report of 29 cases. Surg Neurol. 1998;49(5):471–480.
7)

Nutik S L. Ventral paraclinoid carotid aneurysms. J Neurosurg. 1988;69(3):340–344.
8)

Nutik S. Carotid paraclinoid aneurysms with intradural origin and intracavernous location. J Neurosurg. 1978;48(4):526–533
9)

Kumon Y, Sakaki S, Kohno K, Ohta S, Ohue S, Oka Y. Asymptomatic, unruptured carotid-ophthalmic artery aneurysms: angiographical differentiation of each type, operative results, and indications. Surg Neurol. 1997 Nov;48(5):465-72. PubMed PMID: 9352810.
10)

Day AL. Clinicoanatomic features of supraclinoid aneurysms. Clin Neurosurg. 1990;36:256-74. Review. PubMed PMID: 2403885.
13)

Rustemi O, Cester G, Causin F, Scienza R, Della Puppa A. Indocyanine Green Videoangiography Transoptic Visualization and Clipping Confirmation of an Optic Splitting Ophthalmic Artery Aneurysm. World Neurosurg. 2016 Jun;90:705.e5-705.e8. doi: 10.1016/j.wneu.2016.03.010. Epub 2016 Mar 12. PubMed PMID: 26979923.

Encephaloclastic cyst

Poorly circumscribed areas of parenchymal destruction associated with cystic components.

Encephaloclastic cysts provoked by intraventricular chemotherapy are very uncommon.

Rare complication of a malfunctioning methotrexate Ommaya reservoir 1) 2).

Pathogenesis

The pathogenesis may result from alterations in CSF pulsations with retrograde flow of intraventricular chemotherapy into the brain parenchyma and subsequent development of a local chemical encephalopathy.

Mella et al. report two rare cases of encephaloclastic cyst with intraventricular topotecan use. The patients were diagnosed and treated at The University of Texas MD Anderson Cancer Center. They consented to the publication of their laboratory results and imaging studies for educational purposes.

The patients presented with metastatic cancers (breast/lung) complicated by leptomeningeal disease. Ommaya reservoirs were placed in both cases and patients were initiated on intraventricular topotecan at 0.4 mg twice weekly. After approximately 12 intraventricular treatments, both patients developed confusion, seizures and headaches. MRI of the brain demonstrated cystic dilatation of the brain parenchyma around the catheter that connects to the reservoir dome and delivers the drug to the intraventricular space. The catheter was surrounded by vasogenic edema. Catheters were removed and analyzed and were found to be intact. CSF analyses showed no evidence of infection or malignancy. Intraventricular topotecan was discontinued and both patients demonstrated sustained clinical and radiological responses.

These cases highlight an atypical complication of intraventricular use of topotecan with successful management 3).

1)

Chowdhary S, Chalmers LM, Chamberlain PA. Methotrexate-induced encephaloclastic cyst: a complication of intraventricular chemotherapy. Neurology. 2006 Jul 25;67(2):319. PubMed PMID: 16864827.
2)

Lubomski M, Pell M, Lochhead A, Jude M. Encephaloclastic cyst: a rare complication of a malfunctioning methotrexate Ommaya reservoir. Intern Med J. 2018 Feb;48(2):224-226. doi: 10.1111/imj.13704. PubMed PMID: 29415363.
3)

Mella DB, Kamiya-Matsuoka C, Liao B, Tummala S, de Groot J. Recurrent encephaloclastic cyst induced by intraventricular topotecan. J Neurol Sci. 2015 Feb 15;349(1-2):52-3. doi: 10.1016/j.jns.2014.12.024. Epub 2014 Dec 24. PubMed PMID: 25598491.