A perplexing case report of concomitant multiple sclerosis and myasthenia gravis.

Introduction and importance: The co-occurrence of multiple sclerosis (MS) and myasthenia gravis (MG) within the Middle East and North African Region (MENA) has been scarcely reported in current literature. This rare case report explores the pathophysiological mechanisms and potential avenues of treatment modalities. Such insights can potentially facilitate the development of more efficacious and targeted treatment modalities and perhaps pave the way for disease prevention. Case description: Twenty-nine-year-old female patient presented with diplopia of two weeks duration associated with occasional blurred vision in the left eye. On physical examination, she was discovered to have marked left eye ptosis. A visual evoked potential (VEP) test was performed, which revealed asymmetrical delay. MRI imaging revealed a few white matter hyperintense foci noted at both periventricular regions and the corpus callosum with the characteristic appearance of Dawson's fingers, and thus MS was diagnosed. An anti-acetylcholine receptor antibody test returned positive, confirming the diagnosis of concurrent MG. Clinical discussion: Proposed pathophysiological mechanisms underlying the concurrent manifestation of both diseases include, among others, the involvement of HLA haplotype and non-HLA genotypes, as well as the immunogenetic influence of specific transcription factors. Notable HLA haplotype genes include DRB1 and HLA-DQ5 genes. In contrast, non-HLA genes include the interleukin-4 receptor (IL4RA) and factor forkhead box P3 (FOXP3). Conclusion: Considering the similar immunological background of the two diseases, ideally, a single therapeutic modality could be used for management. This will hopefully simplify the patient's treatment regimen and may ultimately reduce the treatment cost and patient burden.

Think Melkersson-Rosenthal Syndrome: A Fissured Tongue With Facial Paralysis.

Melkersson-Rosenthal syndrome (MRS) was first described and named after E. Melkersson in 1928 and C. Rosenthal in 1931. MRS is a rare cause of recurrent facial nerve palsy and can manifest as facial paralysis, orofacial edema, and/or tongue fissuring. Presenting with the complete triad, it was scarcely reported in literature. However, the patient reported here had the complete triad. MRS should be considered when facial paralysis is recurrent or when it presents with orofacial edema, and/or tongue fissuring.

Knowledge and awareness among patients with epilepsy observed at Jordan University Hospital.

The objective of this study was to assess the level of knowledge and awareness about epilepsy among patients with epilepsy (PWE) and to determine the correlation with sociodemographic and disease-related factors. A prospective cross-sectional study was set, and it included PWE attending the adult neurology clinic at Jordan University Hospital (JUH), Amman, Jordan. A structured questionnaire was utilized, which consisted of 3 parts: sociodemographic factors, disease characteristics, and an epilepsy knowledge scale - the Epilepsy Knowledge Profile-General (E.K.P-G) scale. There was a total of 108 patients, 43 males and 65 females with an age range from 16 to 63 years. The average score of the subjects in the E.K.P-G scale was 16.4/34 (48%). Twenty out of the 34 questions were answered correctly by less than 50% of the respondents. There was an overall poor understanding of the etiology of epilepsy. A higher E.K.P-G score was significantly correlated with higher levels of education, higher household income, controlled seizures for more than 2 years, and living in urban areas. On the other hand, there was no significant correlation between the level of knowledge and age, gender, marital status, occupational status, type of seizure, duration of epilepsy, source of information, number of antiepileptic drugs (AEDs), and family history of epilepsy. In conclusion, the study showed a significant lack of knowledge about epilepsy among PWE at JUH. A public educational program is necessary in Jordan to educate PWE about their disorder.

Mesenchymal stem cells and conditioned media in the treatment of multiple sclerosis patients: Clinical, ophthalmological and radiological assessments of safety and efficacy.

AIMS: This open-label prospective phase I/IIa clinical study used autologous bone marrow-derived mesenchymal stromal cells (BM-MSCs) followed by mesenchymal stromal cells conditioned media (MSC-CM) for the first time to treat multiple sclerosis (MS) patients. The primary goal was to assess the safety and feasibility and the secondary was efficacy. The correlation between the MSC-CM content and treatment outcome was investigated. METHODS: Ten MS patients who failed conventional therapy were enrolled. Adverse events were recorded to assess safety. The Expanded Disability Status Scale (EDSS) was the primary efficacy measurement, the secondary included clinical (25WFT, 9-PHT), cognitive (MMS), ophthalmology (OCT, VEP), and radiological (MRI lesion and volume) tests. The MSCs-CM concentration of 27 inflammatory biomarkers was investigated. RESULTS: The treatment protocol was well tolerated by patients. There was an overall trend of improvement in all the tests, except the lesion volume which increased significantly. A decrease of 4 and 3.5 points on the EDSS was achieved in two patients. We report a correlation between a decreased lesion number at baseline and higher IL-6, IL-8, and VEGF MSC-CM content. CONCLUSION: The used protocol was safe and feasible with possible efficacy. The addition of MSC-CM could be related to the magnitude of EDSS improvement observed.

Towards establishing a multiple sclerosis biobank in Jordan.

Genome-wide association studies (GWAS) have been a promising approach in unraveling genetic associations to multiple sclerosis (MS), a complex, multifactorial disease. Biobanks are repositories of patient biospecimens and information that can promote GWAS research. However, the success of GWAS and biobanking is dependent on the level of participation of MS patients in genetic research. In order to initiate MS-based biobanking and GWAS research in Jordan, the willingness of MS patients to participate in long-term, genetic research in Jordan and their preferred type of a consent form were investigated. MS patients (289) were recruited for genetic studies. Personal and clinical information were collected from those who enrolled in the study. Approximately 96% of MS patients agreed to participate in genetic studies. The female:male ratio among patients was 2:1 with most patients being diagnosed with relapsing-remitting MS (88%). The mean age of onset was 28.3 years, the mean duration of illness was 6 years, and the mean Expanded Disability Status Scale was 2.8. Relatedness of parents was significantly associated with having secondary-progressive MS. Approximately 85% of the patients preferred open consent with 37% of them preferring to renew their consent. All the patients approved to be recontacted and update their information via accessing their medical files or physicians. These observations support the establishment of a specialized MS biobank in Jordan and pave the way to participate in international large-scale genetic initiatives.

Fatal encephalitis due to BK virus in a patient with common variable immunodeficiency: a case report.

Encephalitis due to BK virus is a rare condition. Here, we describe a young male patient with common variable immunodeficiency who developed fatal encephalitis due to BK virus. The patient presented initially with ocular symptoms that were followed by behavioral changes and spastic quadriparesis. Diagnosis was made by the compatible clinical findings and detection of viral DNA by polymerase chain reaction in the cerebrospinal fluid. To the best of our knowledge, this is the first report of BK virus encephalitis in a patient with common variable immunodeficiency. We suggest that BK virus should be suspected in cases of encephalitis; particularly in patients with immunodeficiency.

Quality of life in epilepsy at Jordan University Hospital.

OBJECTIVE: To study the quality of life (QOL) and psychiatric symptoms in epilepsy patients at the Jordan University Hospital (JUH), and to compare these findings with those from Middle Eastern and Western literature. METHODS: Fifty epilepsy patients, aged 18 years, or older, attending the Neurology Clinic at Jordan University Hospital, Amman, Jordan over a 6-month-period from March to September 2009, were requested to complete a questionnaire on QOL in epilepsy, as well as a neuropsychiatric inventory, both translated into Arabic. RESULTS: The patients' mean age was 37.6 years, with 26 males, and 24 females. Only 2 patients were divorced and 3 lost their job due to epilepsy. During the year prior to the study, 40% of patients had less than one attack/month and 32% were seizure-free. Two-thirds of patients had tonic-clonic seizures. Most (84%) did not sustain injuries due to the seizures. Approximately 3/4 of patients (73.4%) stated that their seizures were very well controlled with antiepileptic drugs, which did not lead to major side effects. Forty-two patients (84%) did not have any restriction of daily activities due to epilepsy and substantial proportions (range 52-88%) did not perceive any impact of epilepsy on aspects of daily living. Feelings of stigma were noticed in only 7 patients. Mild depression was noted in 42% of patients. CONCLUSION: Compared with others, this study shows a good QOL in patients with epilepsy, less stigma, and little impact of epilepsy on daily living with, however, similar to other reports, a significant rate of depression, which could have been alleviated by better family and social support.

Intracerebral hemorrhage.

OBJECTIVE: To study intracerebral hemorrhage (ICH) at Jordan University Hospital (JUH) with the aim of assessing prevalence, age/gender distribution, causes, clinical manifestations, and location of ICH on CT brain, treatment modalities and outcome, and to compare these findings with those from Middle Eastern and Western literature. METHODS: Among 1498 patients with stroke seen over a 6-year-period, from January 2002 to December 2007 at JUH, Amman, Jordan, 100 patients with spontaneous ICH were studied retrospectively. RESULTS: The prevalence of ICH was 6.7%. The mean age was 61 years, with 71 males, and 29 females. The most common cause was hypertension, alone or combined with the use of aspirin and/or warfarin. The most common clinical manifestations were hemiparesis/plegia, impaired level of consciousness (mean Glasgow coma scale [GCS] score = 7.3) and headache/vomiting. The most common location on CT brain was in the basal ganglia (61 patients). Seventy-nine patients received medical treatment and 21 had surgery. Forty-two patients died after a mean of 7 days (ranging from one day to 6 weeks). The factors related to mortality were old age >68 years, GCS <8, intraventricular extension of ICH on CT brain and the presence of other comorbidities. Among 58 survivors, 50 were left with neurological deficit, predominantly hemiparesis/plegia in 46 patients. Twelve patients were left with chronic epilepsy. CONCLUSION: This study shows a lower prevalence of ICH compared to developed countries, a similar age and gender distribution, and hypertension as the major risk factor, which should be the focus of public health in Jordan and the Arab World.

A clinical and electrodiagnostic study of diabetic neuropathy at Jordan University Hospital.

OBJECTIVE: To study diabetic neuropathy (DN) at Jordan University Hospital (JUH) with the aim of assessing age/gender distribution, risk factors, and other diabetic microvascular complications, clinical manifestations, results of nerve conduction studies (NCS), and treatment modalities, and to compare these findings with those from Western and other Middle/Far Eastern literatures. METHODS: Among 562 patients with diabetes mellitus (DM) seen over a 2-year-period from January 2003 to January 2005, at the Diabetes center at Jordan University Hospital (JUH), 110 patients (10 DM type 1; 100 DM type 2) were studied retrospectively. RESULTS: The prevalence of DN was 20%. The mean age was 55.4 years (range 23-75), with 62 females and 48 males. The most common risk factors for DN were old age, long duration of DM, mean hemoglobin A1c, and hypertension. Almost 50% of patients had additional retinopathy, renal involvement, or both. The most common clinical symptoms were distal numbness/paresthesiae in the limbs (60% of patients) and the neurological examination was normal in 2/3 of patients (only 20% had stocking hypoesthesia to pain and temperature and absent ankle jerks). The NCS showed an axonal neuropathy mainly affecting the lower limbs, especially sensory fibers (abnormal sural sensory action potential in 98% of patients). Anticonvulsants (carbamazepine and gabapentin) were used efficiently in 50% of patients as symptomatic treatment, while tricyclic antidepressants were used as add-on in only 23 patients, due to anticholinergic side effects. CONCLUSION: In comparison with other studies, ours shows a lower prevalence of DN, similar age distribution with however, a predominance of females, similar risk factor profiles, clinical/NCS findings, and treatment modalities.

Ulnar neuropathy at Jordan University Hospital.

OBJECTIVE: To study the causes, clinical and electromyography/nerve conduction study (EMG/NCS) findings and treatment modalities in Jordanian patients with ulnar neuropathy (UN) observed in a tertiary care referral center and compare the findings with those from Western literature. METHODS: The case notes of 20 patients with UN referred to the neurophysiology department at Jordan University Hospital, Amman, Jordan, between January 2002 and January 2004 were reviewed. The clinical presentation, causes, EMG/NCS and treatment modalities were registered. RESULTS: Among the 20 patients, 18 were male and 2 female with a mean age of 39 years (range 14-68 years). Ten cases were traumatic UN while the other 10 were presumably idiopathic cubital tunnel syndrome (CTS). The most common clinical manifestations were paresthesiae of 4th/5th digits and weakness/atrophy of small hand muscles. All 10 cases of traumatic UN were axonal on EMG/NCS while among the other 10 with CTS, 3 diabetics had axonal injury and 6 out of 7 nondiabetics had a demyelinating injury, 3 sensorimotor and 3 pure sensory. Additional carpal tunnel syndrome was found in 5 patients. Needle EMG was abnormal only in cases of abnormal ulnar sensory action potential. Nine out of 10 with traumatic UN had surgery while only 3 out of 10 with CTS had cubital tunnel release. CONCLUSION: Compared to previous studies from Western literature, our study shows a similar male predominance as well as comparable clinical and neurophysiological findings and treatment modalities.

Epilepsy at a university hospital in amman, jordan.

OBJECTIVE: To study the clinical, EEG, neuroimaging, treatment results and outcome in Jordanian patients observed in a tertiary care referral center and compare the findings with those from Western and Middle/Far Eastern literature. METHODS: The case notes of 200 patients with epilepsy examined in an adult neurology clinic at Jordan University Hospital, Amman, Jordan, between January 2000 and December 2002 were reviewed. The findings of the EEG records and CT or MRI of the brain were registered. Seizures were classified according to the International League Against Epilepsy into generalized and partial seizures. The results of the treatment with antiepileptic drugs (AEDs) as well as the outcome were assessed. RESULTS: Among the 200 patients reviewed, 119 were female and 81 were male, with a mean age of onset of 24.6 years, the majority (85%) were below 39 years. The main seizure types were generalized in 128 (64%) patients (92 patients with tonic clonic (TC) seizures) and partial in 72 patients. Interictal EEG was abnormal in 69% of the patients. Neuroimaging showed brain lesions in 33.5% of the patients with a higher yield in partial (56%) than in TC seizures (30%). Sodium valproate and carbamazepine were the most frequently and efficiently used AEDs for generalized and partial seizures. After a 3 year follow-up, 76% were fully controlled with AEDs, outcome being better in generalized than partial seizures (worst in complex partial seizures). CONCLUSION: Compared to the previous population on hospital based reports from Western and Middle/Far Eastern literature, our study showed a higher proportion of females and generalized seizures and a relatively better outcome with relatively similar early age of onset, yield of investigations and treatment modalities.

Ischemic stroke in Jordan 2000 to 2002: a two-year, hospital-based study.

A total of 200 consecutive patients with first ischemic stroke seen at Jordan University Hospital in Amman, Jordan, during a 2-year period were studied retrospectively. Mean age was 61.2 years (range: 29-95 years). The most common stroke subtype was lacunar infarct (51%), but the frequency of cardioembolic strokes was low (8%). Hypertension, diabetes mellitus, coronary artery disease, and smoking were the most common risk factors for atherosclerotic noncardioembolic strokes. Chronic atrial fibrillation was the most common risk factor for cardioembolic strokes. No patient had significant (>50%) extracranial carotid or vertebral artery stenosis. The outcome was relatively good; 85% of patients were discharged home, the majority with only mild neurologic deficit. The favorable outcome is most likely because of the relatively young mean age and the predominance of lacunar infarcts.

Carpal tunnel syndrome: a series observed at Jordan University Hospital (JUH), June 1999-December 2000.

One hundred and eighty five consecutive patients with carpal tunnel syndrome (CTS) seen at Jordan University Hospital (JUH) over an 18-month-period were studied retrospectively. Mean age=45 years (range 19-80); 156 females, 29 males. The most common predisposing factors were stressful manual work, followed by diabetes mellitus and obesity (body mass index, BMI>29). The most frequent symptoms were nocturnal hand paresthesiae. The respective sensitivities of Tinel and Phalen signs were 61 and 46%. The diagnostic yield of nerve conduction studies (NCS) was 80% and the most sensitive NCS technique was the median palm-to-wrist (PW) technique with a yield of 61%. Seventy percent of the hands with electrophysiological CTS were moderate and severe. One hundred and forty patients received conservative treatment and 45 underwent surgical decompression.

Multiple sclerosis at Jordan University Hospital.

OBJECTIVE: A study of multiple sclerosis patients admitted to Jordan University Hospital over a 10-year-period with a comparison with other Western and Middle Eastern series. METHODS: The case notes of 32 patients with multiple sclerosis were reviewed to determine the age and sex distribution, clinical findings and results of investigations, treatment and outcome. RESULTS: There were 32 patients (24 females, 8 males, 3:1), with mean age at onset of 31.4 years, and range 6-50 years. All patients fulfilled Poser`s criteria for multiple sclerosis. The most common clinical manifestation was myelopathy followed by a polysymptomatic onset. Two-thirds of the patients had a relapsing - remitting course. The yield of cerebrospinal fluid oligoclonal banding was low (20%). The sensitivities of visual evoked potential was 62%, magnetic resonance imaging of brain 100%, magnetic resonance imaging of cervical spine was 70% and of dorsal spine 44%. Intravenous methylprednisolone Igr for 3-5 days was efficient in acute relapses for all patients. Beta-interferon was efficient in 2 out of 7 patients. After a mean follow-up of 5 years, two-thirds of the patients had mild to moderate disability (Extended disability status scale = 2 and 3). CONCLUSION: The age and sex distribution, clinical and magnetic resonance imaging findings were almost similar to other reports. However the yield of visual evoked potentials was low and that of cerebrospinal fluid oligoclonal banding was very low, the results of treatment with beta-interferon were modest and the outcome at a mean follow-up of 5 years was relatively good.