Guillain-Barré Syndrome Following the BNT162b2 mRNA COVID-19 Vaccine.

PURPOSE: The onset of the COVID-19 (SARS-CoV-2) pandemic in December 2019 created the need for multiple scientific research activities and clinical trials in an attempt to find solutions to mitigate the impact of the virus. One of the important tools to combat the virus is the development of vaccination programs. All types of vaccines have been associated with a mild to severe risk of neurological adverse events. One of these severe adverse events is Guillain-Barré syndrome. CASE REPORT: Here, we describe a case of Guillain-Barré syndrome after the first dose of the BNT162b2 mRNA COVID-19 vaccine and review the literature to increase the current knowledge regarding this complication. CONCLUSION: Guillain-Barré syndrome after COVID-19 vaccination is responsive to treatment. The benefits of administering the vaccine outweigh the risks. Due to the negative impact of COVID-19, it is essential to recognize the development of neurological complications that are potentially associated with vaccination, including Guillain-Barré syndrome.

Cerebral fat embolism syndrome: diagnostic challenges and catastrophic outcomes: a case series.

Fat embolism syndrome is a rare but alarming, life-threatening clinical condition attributed to fat emboli entering the circulation. It usually occurs as a complication of long-bone fractures and joint reconstruction surgery. Neurological manifestations usually occur 12 to 72 hours after the initial insult. These neurological complications include cerebral infarction, spinal cord ischemia, hemorrhagic stroke, seizures, and coma. Other features include an acute confusional state, autonomic dysfunction, and retinal ischemia. In this case series, we describe three patients with fat embolism syndrome who presented with atypical symptoms and signs and with unusual neuroimaging findings. Cerebral fat embolism may occur without any respiratory or dermatological signs. In these cases, diagnosis is established after excluding other differential diagnoses. Neuroimaging using brain magnetic resonance imaging is of paramount importance in establishing a diagnosis. Aggressive hemodynamic and respiratory support from the beginning and consideration of orthopedic surgical intervention within the first 24 hours after trauma are critical to decreased morbidity and mortality.

Epilepsy and Hearing Loss in a Patient with a Rare Heterozygous Variant in the CACNA1H Gene.

The calcium voltage-gated channel subunit alpha 1 H (CACNA1H) is a gene present in eukaryotic cells located on chromosome 16 that encodes the T-type calcium channels, which are important for calcium influx and depolarization of cells. Pathogenic variants in CACNA1H cause autosomal dominant susceptibility to idiopathic generalized epilepsy 6 (OMIM: 611942), which is a broad term that encompasses several common seizure phenotypes. In this article, we reported a Saudi female with a heterozygous variant in the CACNA1H gene (OMIM: 607904) who had epilepsy and hearing loss. This is the first case to report the association of epilepsy and hearing loss with a variant in CACNA1H. We believe that variant may be the reason for developing both epilepsy and sensorineural hearing loss. Further studies are needed to identify the role of CACNA1H in the physiology of the ear to allow for a better understanding of the effects of mutations. In children who present with early childhood hearing loss, genetic studies in highly selected cases including those with a strong family history of hearing loss and epilepsy may help detect a CACNA1H variant early and help with the early screening and diagnosis of other associated disorders including subclinical epilepsy.

Acute cervical dystonia following the BNT162b2 mRNA COVID-19 vaccine.

The coronavirus disease of 2019 (COVID-19) pandemic is caused by a novel coronavirus SARS-Cov-2. Four major vaccine types are being used to fight against this deadly pandemic and save precious human lives. All types of vaccines have been associated with a risk of neurological complications ranging from mild to severe. Cervical dystonia occurring after a COVID-19 vaccine was not previously reported in the literature. In this article, we describe a case of acute cervical dystonia occurring after the first dose of the BNT162b2 COVID-19 vaccine. We attribute the occurrence of cervical dystonia to the vaccine due to the temporal relationship. This report adds to the literature a possible rare side effect of a COVID-19 vaccine and contributes to the limited literature on potential neurological side effects of mRNA-based vaccines. The likely mechanism is autoimmune. Further research is needed to probe and study the exact mechanism.

Rapid implementation of telemedicine in Neurology during the COVID-19 pandemic: Challenges in King Abdulaziz Medical City-Jeddah.

Telemedicine is defined as the remote medical practice of delivering healthcare services to the underserved using information and communication technology. It encompasses a wide range of medical activities, including diagnosis, treatment, disease prevention, and education. The coronavirus disease of 2019 (COVID-19) pandemic has caused significant social dislocation, negative economic impact, and a major change in medical practice in Saudi Arabia. Telemedicine has rapidly moved to the frontline of healthcare practice due to the demand for prevention and mitigation strategies. It has been encouraged and facilitated with huge government support. Herein, we describe the virtual clinical practice of the neurology department at King Abdulaziz Medical City-Jeddah in response to the COVID-19 pandemic. This narrative review is an urgent call to improve the perception and knowledge of both medical personnel and patients concerning telemedicine and to support the utilization of advanced information and communication technology.

The spectrum of muscle pathologies: Three decades of experience from a reference laboratory in Saudi Arabia.

BACKGROUND: When investigating patients with a suspected neuromuscular disorder, a muscle biopsy is considered an instrumental tool to reach a definitive diagnosis. There is a paucity of publications that assess the diagnostic utilization and yield of muscle biopsies. We intend to present our experience in this regard over an extended period of more than three decades. METHODS: This is an observational retrospective cohort study in which we collected pathology reports for muscle biopsies diagnosed at our reference lab between 1986 and 2017. RESULTS: We identified a total of 461 cases of muscle biopsy performed, which fulfilled the inclusion criteria. Pediatric cases defined as ≤14 years of age constituted a significant proportion of cases (n = 275, 60%). Normal biopsies were reported in 27% of cases (n = 124), and in 4%, the biopsies were non-diagnostic. The most common pathologies reported were non-specific myopathy (n = 72, 16%), dystrophy (n = 71, 15%), and neurogenic disorders (n = 60, 13%). CONCLUSION: In conclusion, the muscle biopsy will continue to play a crucial role, as a gold standard or as a complementary investigation, in the diagnosis of certain neuromuscular disorders. Increasing the yield and accuracy of muscle pathology should be the main concern and priority to neuropathologists reporting muscle biopsies. In addition, utilizing next-generation sequencing and other molecular techniques have changed the location of muscle biopsy in the algorithm of the diagnosis of neuromuscular disorders. This paper is an urgent call to establish the Saudi Neuropathology Society and the muscle pathology and neuromuscular disorders registry.

Hashimoto`s Encephalopathy Presenting with Progressive Cerebellar Ataxia.

Hashimoto`s encephalopathy is a rare neurological syndrome occurring in patients with autoimmune thyroid disease. The diagnosis of Hashimoto`s encephalopathy is based on the clinical picture with the presence of serum anti-thyroid antibodies regardless of the thyroid disorder. Acquired cerebellar ataxia associated with Hashimoto`s disease is a rare occurrence. In this article, we present a case who had progressive non-familial autoimmune pancerebellar disease in association with an increased level of thyroid peroxidase and thyroglobulin antibodies. The patient was managed aggressively with both intravenous immunoglobulins and plasma exchange, which stopped the progression of the disease and allowed for slow improvement. Early diagnosis of Hashimoto`s encephalopathy with autoimmune cerebellar ataxia and intervention with immunomodulatory therapy are of paramount importance. Close monitoring after steroid therapy is important since some patients with this rare disease might be resistant to steroid therapy and require aggressive immunomodulatory therapy.

Determination of quality gap for educational services in undergraduate medical education. A cross-sectional study in Saudi Specialized Health Sciences University.

OBJECTIVE: To assess the perception and expectation of students about the quality of educational services and identify the gap in educational services. METHODS: This cross-sectional study was conducted at King Saud bin Abdulaziz University for Health Sciences (KSAU-HS) colleges across all regions (Riyadh, Jeddah, and Al-Ahsa) within one academic year (September 2017- April 2018). The difference between the students' perceptions and expectations was calculated to identify the gap between these 2 opinions. RESULTS:   A total of 416 (83.2%) questionnaires were completed with an almost equal ratio of male to female (51% and 49%). There was a significant difference in mean (gap) between students' expectations and perceptions of educational service quality at KSAU-HS (p less than 0.05). The results showed negative values for most of the items. It revealed that there is a significant difference between male and female in responsiveness, empathy, and tangible. CONCLUSION: The findings of this study showed that all the dimensions were not up to the expectations of students. The results will help the higher management to initiate a protocol to address all weaknesses.

De novo Craniopharyngioma of the Fourth Ventricle: Case Report and Review of Literature.

Craniopharyngiomas usually involve the sella and suprasellar space. Their occurrence in the posterior fossa without extension to the suprasellar region is uncommon with only 16 cases reported in the literature. We report a case of a primary posterior fossa craniopharyngioma that was managed by complete excision with a good recovery. Our case was unique in that the craniopharyngioma occurred in the fourth ventricle and extended downward to the level of C1, a manifestation that was reported only twice in the past. The literature on the topic is reviewed.

Quality of life among multiple sclerosis patients in Saudi Arabia.

OBJECTIVE: To assess quality of life in multiple sclerosis (MS) patients and determine the factors associated with levels of quality of life in MS patients in a public hospital in Saudi Arabia. METHODS: A cross-sectional study was conducted from June 2016 to April 2017 in King Abdulaziz Medical City, Jeddah, Kingdom of Saudi Arabia. Multiple sclerosis patients attending the outpatient and inpatient services were approached and recruited to participate in the study. The Arabic version of EuroQOL-5 Dimensions instrument (EQ-5D) was utilized for the assessment of MS patients quality of life. RESULTS: Data on quality of life were obtained from 292 patients. The reported quality of life of MS patients as measured by the EQ-5D index value score was 0.31+/-0.51 and the EQ-VAS score was 73.87+/-23.41, respectively. It was found that quality of life determined numerically in the EQ-5D index value and EQ-VAS deteriorates proportionally according to the disease duration. CONCLUSION: Multiple sclerosis is associated with a considerable effect on the patients quality of life. It continues to be challenging to manage both medically and psychosocially. Clinicians should consider the assessment of quality of life as routine practice along with the other important measures including symptomatic evaluation, laboratory tests, and neuroimaging to provide a holistic care of their MS patients.

Chromosomal Micro-aberration in a Saudi Family with Juvenile Myoclonic Epilepsy.

BACKGROUND AND OBJECTIVE: Epilepsy is etiologically and genetically complex neurological disorder affecting millions of people worldwide. Juvenile myoclonic epilepsy (JME) is the most common epilepsy syndrome that starts in the teen age group commonly between ages 12, 18, and lasts till adulthood. One out of fourteen people with epilepsy suffers with JME. Myoclonic seizures and muscle twitching or uncontrolled jerking are the most common type of seizures in the people suffering with JME. METHOD: To observe the novel CNVs involved in JME, we investigated a Saudi family with nine siblings including one male and one female affected members. In this study we used high density whole genome Agilent sure print G3 Hmn CGH 2x 400K array-CGH chips. Our results showed CNVs including the amplifications and deletions in different chromosomal regions in the patients as compared to the normal members of the family. Amplifications were observed in the chromosome 22 cytoband 22q11.23 with LDL receptor related protein 5 like (LRP5L), Immunoglobulin Lambda-Like Polypeptide 3 (IGLL3) and crystallin beta B2 pseudogene (CRYBB2P) genes respectively whereas the deletions were observed in the chromosomal regions 4q22.2 with Glutamate receptor, ionotropic, delta 2 (GRID2) as potential gene cytoband 1p31.1 with potential Neuronal Growth Regulator 1 gene (NEGR1) gene in this region and NME/NM23 family member (NME7) gene cytoband 1q24. Moreover, the array CGH resulting in deletions and duplication were also validated by using primer for simple PCR or also by using quantitative real time PCR analysis. We found deletions and duplication in JME patients in our study for the first time in Saudi population. RESULTS & CONCLUSION: The findings in this study suggest that the array-CGH may be considered as a first line of genetic testing for diagnosis of epilepsy unless strong evidence is presented for a monogenic syndrome. The use of high throughput technique in this study will help to identify novel mechanisms underlying epileptic disorder in order to lower the burden of epilepsy in Saudi Arabia.

Neurological complications of bariatric surgery.

OBJECTIVE: To review and analyze the neurological complications from bariatric surgery in Kingdom of Saudi Arabia. METHODS: This cross sectional study was carried out in King Abdulaziz Medical City, Jeddah, Kingdom of Saudi Arabia from January 2009 to December 2015. Important personal and clinical data were collected from the charts of the patients who underwent bariatric surgery. Data on follow up visit and remote complication if present, was also collected. All patients with neurological complications were reviewed in detail. The significant difference was calculated by using T-test and p-value<0.05 was considered significant. RESULTS: A total of 451 patients underwent bariatric surgery, 15 cases had neurological complications (3%). Axonal polyneuropathy was the most frequent neurological complication, but cases of Wernicke syndrome, vitamin B12 deficiency, Guillain-Barre syndrome and copper deficiency were also identified. Fourteen patients (93.3%) had full recovery from the neurological signs and symptoms; one patient died. CONCLUSION: Bariatric surgery is not free of potential neurological complications. Complications may affect both central and peripheral nervous system and death is a possibility. Multidisciplinary care including consultation of different teams is highly recommended.

Array-comparative genomic hybridization analysis of a cohort of Saudi patients with epilepsy.

Specific genetic anomalies or non-genetic factors could lead to epilepsy, but in various cases the underlying cause is unknown. Novel technologies, such as array comparative genomic hybridization, may reveal the copy number variants (CNVs), established as significant risk factor for epilepsy. This study carried out a high-density whole genome array- comparative genomic hybridization analysis with blood DNA samples from a cohort of twenty epilepsy patients to search for CNVs associated with epilepsy. Microdeletion of 14q31.1 was observed in four patients including two from the same family with loss of the NRXN3 gene; microdeletion of 15q12 in one patient with loss of the GABRG3 gene, and microduplication of 20q13.33 in three patients with loss of the gene group CHRNA4, KCNQ2, EEF1A2 and PPDPF were also found. These CNV findings were confirmed by real-time quantitative polymerase chain reaction. We have described, for the first time, numerous potential CNVs/genes implicated in epilepsy in the Saudi population. The study presents a better description of the genetic variations in epilepsy, and would eventually enable us to provide a foundation for understanding the critical genome regions which might be involved in the development of epilepsy.

Neurophobia among medical students.

OBJECTIVE: To assess the attitude of medical students and junior physicians toward neurology. METHODS: A self-administered, previously validated, questionnaire was distributed among 422 students and junior physicians at King Abdulaziz University, Jeddah, Saudi Arabia from September to December 2012. In this cross-sectional study, the questionnaire included demographic data and 12 statements to examine attitudes toward neurology using a Likert scale. RESULTS: The response rate among participants was 70.3%. The mean age was 22.35 (SD+/-1.28) years. Males comprised 46.2%. While 31.3% of students had not decided regarding their future career, 11.8% selected neurology as their first possible choice. Whereas 29.6% of students were not satisfied with their neurology teaching experience, 84.4% found neurology difficult, and 42.7% of the whole group thought that their neuroscience knowledge was insufficient. Advanced clinical year students (namely, interns) were less likely to consider neurology as a career choice (p=0.001). CONCLUSION: Most of the students had an unfavorable attitude toward neurology on the Likert scale. New strategies are needed to change students` attitude toward this demanding specialty.

Memory boosting effect of Citrus limon, Pomegranate and their combinations.

Memory is greatly influenced by factors like food, stress and quality of sleep, hence present study was designed to evaluate the effect of Citrus limon and Pomegranate juices on memory of mice using Harvard Panlab Passive Avoidance response apparatus controlled through LE2708 Programmer. Passive avoidance is fear-motivated tests used to assess short or long-term memory of small animals, which measures latency to enter into the black compartment. Animals at MCLD showed highly significant and significant increase in latency to enter into the black compartment after 3 and 24 hours respectively than control, animals at HCLD showed significant increase in latency only after 3hours. Animals both at low and moderate doses of pomegranate showed significant increase in test latency after 3 hours, while animals at high dose showed highly significant and significant increase in latency after 3 and 24 hours respectively. There was highly significant and significant increase in latency in animals at CPJ-1 combination after 3 and 24 hours respectively; however animals received CPJ-2 combination showed significant increase in latency only after 3 hours as compare to control. These results suggest that Citrus limon and Pomegranate has phytochemicals and essential nutrients which boost memory, particularly short term memory. Hence it may be concluded that flavonoids in these juices may be responsible for memory enhancing effects and a synergistic effect is observed by CPJ-1 and CPJ-2 combinations.

A child with leukemia and behavioral changes.

A 12-year-old Saudi girl, known case of T-cell leukemia with CNS relapse. She was diagnosed 2 years ago. Multiple cycles of chemotherapy had been used (Fludarabine, Cytarabine, Methotrexate, Cyclosporine, and Mercaptopurine). She was admitted electively for cord blood transplantation. Afterward, she developed visual, and behavioral change followed by seizure.

Generalized myoclonus and spasticity induced by lamotrigine toxicity: a case report and literature review.

Lamotrigine (LTG) is a well-tolerated broad-spectrum antiepileptic drug, which is chemically unrelated to other existing antiepileptic medications. The drug has also some mood-stabilizing properties and, according to some studies, modest antidepressant effects. The exact mechanism of action is unknown, but some animal studies suggest the inhibition of neuronal glutamate release. Despite being relatively safe, LTG has been demonstrated to have proconvulsant effect especially in certain types of epilepsies like myoclonic status epilepticus. Myoclonic status epilepticus and its variations including generalized myoclonic status epilepticus, status myoclonus, and prolonged myoclonus describe a variety of clinical states, which have continuous, unremitting seizures lasting longer than 5 minutes. It is not a commonly reported treatment-emergent neurological complication, but the treatment is always a medical emergency. We report a case of a 46-year-old man who developed generalized myoclonus status epilepticus a few hours after suicidal ingestion of LTG. He remained hemodynamically stable throughout hospitalization and started to recover and achieved complete recovery 3 days later. This is the first reported case of this de novo complication induced by LTG toxicity. We proposed a subcortical mechanism for this complication induced by the toxic doses of LTG.