Pattern reversal visual evoked potentials (prVEPs) in autosomal recessive hereditary spastic paraplegia with thin corpus callosum (ARHSPTCC) patients with SPG 11 mutations in Saudi Arabia, cross section hospital base study.

OBJECTIVE: To retrospectively report prVEPs in SPG11 ARHSP-TCC. BACKGROUND: ARHSPTCC is characterized by a thin corpus callosum, progressive spastic paraparesis, cognitive decline,and axonal neuropathy by SPG11 mutations. Additionally, seizures, cerebellar ataxia, speech and swallowing problems, extrapyramidal signs, and skeletal deformities may occur. Neuroradiological findings include thinning of the anterior corpus callosum (TCC), periventricular white matter changes, and cortical atrophy. Electromyography and nerve conduction studies may reveal axonal neuropathy or anterior horn involvement. However, optic nerve involvement and prVEPs have not been well described. DESIGN/METHODS: Routine prVEPs were performed in 11 subjects with genetically confirmed (Athena Diagnostic USA) SPG11 ARHSPTCC. Independent stimulation of each eye with a full-field checkerboard pattern reverse stimulation technique was performed. Repetitive waveforms were averaged and the P-100 was recorded. RESULTS: Eleven subjects aged 20 to 37 years were studied, 5 were female. Nine were from consanguineous parents. Nine had a family history and 3 pairs were siblings. Nine had TCC, 8 had diffuse brain atrophy and 1 had cerebellum and brainstem atrophy. Additionally, 9 had bilaterally abnormal prVEPs. The mean P100 latency of the left eye was 129.45 ms±19.47, and a mean amplitude of 7 µV±2.33, while the right had a mean P100 of 127.72 ms±12.69, and mean amplitude of 6.74 µV±2.84. CONCLUSIONS: Abnormal prVEPs occurred in 81.82% of our subjects with significantly prolonged P100 bilateral responses. This indicates that the visual pathway is affected in patients with SPG11 ARHSPTCC. However, no specific mutation was predominant. prVEPs should be considered in the routine evaluation for spastic paraparesis.

The Optimal Dose of Amobarbital in the Wada Test for the Presurgical Evaluation of Patients With Temporal Lobe Epilepsy.

OBJECTIVE: The use of amobarbital in the Wada test varied between epilepsy centers, with no unified dosing or protocols available in the literature to standardize its use. We aimed to determine the dose of amobarbital in the presurgical evaluations of patients with temporal lobe epilepsy. METHODS: A retrospective study of patients with temporal lobe epilepsy seen between January 2004 and December 2018 in King Faisal Specialist Hospital and Research Centre in Jeddah, Saudi Arabia, was conducted, and those who successfully underwent a Wada test were studied. A neuropsychologist or a neurologist will assess the memory and language, using standardized testing. RESULTS: A total of 90 patients were studied. The mean age was 30 years (range, 16-52 years), where 49 (57%) of them were men. All patients had a routine neurological examination, including language and memory. The average dose of amobarbital given was 10.1.1 mg (range, 65.7-150 mg). There was no statistical difference between the dosing given to patients who passed or failed the memory testing (101.4 mg vs 94.7 mg, P = 0.1). Multivariate regression analysis showed that amobarbital dose needed an adjustment to patient's weight only for those older than 30 years, (P < 0.05; 95% confidence interval, 0.1-0.5), where an increase in the dose by 0.3 mg·kg·y was required to execute Wada test successfully. CONCLUSION: It was only the patient's age that could influence the modification of Amobarbital dose in the Wada test, yet establishing a universal protocol is challenging because of the lack of well-defined dose determinants.

Endovascular suction thrombectomy for severe cerebral venous sinus thrombosis: A report of two cases.

Cerebral venous sinus thrombosis (CVST) is a severe clinical condition that manifests with diverse symptoms that can be mild, taking the form of a headache, or can be life-threatening, with raised intracranial pressure and herniation. Despite systemic anticoagulation treatment, CVST still carries a mortality risk of approximately 10% because of the failure of venous recanalization by anticoagulants alone. This paper describes two cases with extensive CVST who presented with progressive neurological deterioration despite adequate systemic anticoagulation treatment. Both patients were successfully treated with endovascular clot removal using suction thrombectomy techniques with immediate recanalization of the sinuses and rapid radiographic improvement. The described therapy appears effective and safe; however, further studies are needed to validate the effectiveness of this treatment.

Outcome of intracranial arterial stenting of symptomatic atherosclerotic disease: A single center experience from Saudi Arabia.

OBJECTIVE: To present our local experience with intracranial angioplasty and stenting used for the treatment of symptomatic intracranial stenosis to assess its safety, efficacy, and outcome. METHODS: This is a retrospective review of all the patients with symptomatic intracranial atherosclerotic disease who underwent endovascular treatment in King Faisal Specialist Hospital and research center, Jeddah, Kingdom of Saudi Arabia from January 2003 to December 2014. Clinical, procedural, and outcome variables were gathered. RESULTS: We identified 22 patients who were referred for stenting of symptomatic intracranial atherosclerotic stenosis. In all but 3, the stents were deployed successfully (86% procedural success rate). The procedure was carried out under conscious sedation in 32%. Excellent flow was restored immediately in all successfully-stented cases. Post procedural strokes occurred in 4 patients (17.4%). One non-neurological death was identified in a patient who suffered a major post procedural stroke (4.3%). CONCLUSION: Intracranial atherosclerotic disease is not uncommon in our population. Angioplasty and stenting might be a valid option for the treatment of patients with recurrent symptoms despite optimal medical treatment.

Onyx Embolization of Ruptured Intracranial Aneurysm Associated with Behçet's Disease.

Introduction. Intracranial aneurysms associated with Behçet's disease (BD) are a rare occurrence. They are fragile, thin-walled pseudoaneurysms, which have high tendency to rupture and present a therapeutic challenge. Case Presentation. We report a 26-year-old male with BD presented with subarachnoid hemorrhage due to ruptured middle cerebral artery aneurysm. Additionally, two unruptured aneurysms were identified. He underwent endovascular embolization using Onyx with successful obliteration of the ruptured aneurysm. Medical therapy resulted in regression of one and resolution of the other aneurysms. Conclusion. We describe the first report of the application of Onyx for obliteration of ruptured cerebral aneurysm in BD as a feasible and safe therapeutic option for patients who are not candidates for other techniques.

Emergency excision of cardiac myxoma and endovascular coiling of intracranial aneurysm after cerebral infarction.

Cardiac myxoma is the most common primary tumor of the heart, located mainly in the left atrium. Cerebral embolization or intracranial aneurysm formation as a consequence of left atrial myxomas has been well documented, whereas myxoma embolization causing the combination of cerebral infarction and intracranial myxomatous aneurysm is rare. We report herein, a 67-year-old female with a cardiac myxoma who experienced a left hemispheric embolic ischemic stroke and in addition was found to have right internal carotid artery aneurysm. The patient underwent emergency surgical excision of left atrial myxoma 2 hours after the stroke onset and endovascular coiling of the aneurysm a week later. Although the timing of cardiac surgery is controversial in patients who have had recent ischemic stroke, we recommend immediate resection of cardiac myxoma, if feasible, and early endovascular treatment of associated intracranial myxomatous aneurysms.