Melkersson–Rosenthal syndrome: A rare cause of recurrent facial palsy – A case report

Background: Melkersson–Rosenthal syndrome is a rare, neuro-mucocutaneous, granulomatous disorder of unknown etiology, clinically characterized by a triad of symptoms: recurrent facial nerve palsy, facial swelling and fissured tongue. Melkersson–Rosenthal syndrome is frequently seen in patients in their second or third decade of life. It is diagnosed based on clinical features, and it is rarely possible to observe all the classic triad symptoms at the same time. The disorder may cause recurring peripheral facial palsy that is wrongly diagnosed as recurrent Bell’s palsy. Case presentation: A 25-year-old female patient, referred from Bishoftu town in Ethiopia, was presented to the neurology clinic of Tikur Anbessa Specialized Hospital in Addis Ababa complaining of a five-day history of recurrent left-side peripheral facial weakness, facial edema and fissured tongue. Her past medical history was positive for similar symptoms, for which she was diagnosed with Bell’s palsy and received oral corticosteroid treatment. Left-side lower facial swelling with flat naso-labial fold and fissured tongue were detected on examination. After excluding other mimickers, she was diagnosed with Melkersson–Rosenthal syndrome and completely recovered with a high dose of steroid treatment. Conclusion: Melkersson–Rosenthal syndrome may present with the classic clinical triads of symptoms, but mostly it shows an oligosymptomatic pattern. So, it is usually under-recognized and often misdiagnosed as Bell’s palsy, as had been done in the past in our case. Therefore, Melkersson–Rosenthal syndrome should be considered in the differential diagnosis of patients presented with recurrent peripheral facial weakness, as early detection and therapy might prevent cosmetic disfigurement from multiple relapses [Ethiop. J. Health Dev. 2020; 34(3): 214-216] Key words: Melkersson–Rosenthal syndrome, facial palsy, fissured tongue, facial swelling, Bell’s pals

DYKE–DAVIDOFF–MASSON SYNDROME-A Rare Cause of Cerebral Hemiatrophy in a 17-Years-Old Ethiopian Patient: A Case Report

BACKGROUND: Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease with unknown frequency, mainly presented with seizures, facial asymmetry, contralateral hemiparesis and mental retardation. Often, resulting from brain injury due to a multitude of causes, especially in early life, associated with birth asphyxia. Radiological findings include cerebral hemiatrophy/hypoplasia, calvarial thickening, and hyperpneumatization of the frontal sinuses.CASE PRESENTATION: We report the case of a 17-year-old male patient who presented to Neurology Clinic with complaints of left side body weakness, walking difficulty and poorly controlled seizure for the past 6 years. Brain MRI revealed atrophy of the right cerebral hemisphere.CONCLUSION: Dyke-Davidoff-Masson syndrome should be suspected in any patients who present with classical features and brain imaging showing hemiatrophy. Early identification andtreatment is important in such patients, as it can improve patients prognosis and quality of life

The frequency and impact of admission hyperglycemia on short term outcome of acute stroke patients admitted to Tikur Anbessa Specialized hospital, Addis Ababa, Ethiopia: a cross-sectional study

Background: Admission hyperglycemia (HG) has been associated with worse outcomes among acute stroke patients. A better understanding and awareness of the potentially adverse influence of hyperglycemia on the clinical outcome of acute stroke patients would help to provide guidance for acute stroke management and prevention of its adverse outcomes. We aimed to assess the frequency of admission hyperglycemia and its impact on short term (30-days) morbidity and mortality outcomes of stroke in adult Ethiopian patients in an urban setting. Methods: A prospective, cross-sectional study was conducted among acute stroke patients admitted to Tikur Anbessa Specialized Hospital (TASH), within 72 h of symptom onset, from July to December 2016. Socio-demographic data, neuroimaging findings and capillary blood glucose values were obtained on admission. Hyperglycemia was defined as > 140 mg/dl. National Institute of Health Stroke Scale (NIHSS) and modified Rankin Scale (mRS) were used to assess the baseline stroke severity and the 30-days post-stroke outcome, respectively. Results: A total of 103 first-ever acute stroke patients were included (mean age = 55.5 + 15.3 years, 64.1% male and 65% under the age of 65 years) and 51 (49.5%) were hyperglycemic at time of admission. The median admission NIHSS score was worse in the hyperglycemic patients 14 (IQR 10–19) compared to normoglycemic patients 11 (IQR 8–15). Among stroke survivors, patients with hyperglycemia were 3.83 times (95% CI, 1.99–6.19) more likely to be functionally impaired (mRS = 3–5) at 30-days compared to normoglycemic patients (P = 0.041).Older age (≥ 65 years) (P = 0.017) and stroke severity (NIHSS > 14) (P = 0.006) at admission were both significantly associated with poor functional recovery at 30-day. Among patients who died at 30-day, two-third (66.7%) were hyperglycemic but they failed to show any significant association. Conclusions: Hyperglycemia is prevalent among Ethiopian stroke patients at the time of presentation and it is associated with significantly poor functional recovery at 30th-day of follow up. This finding provides a rationale for achieving normal blood glucose in the course of acute stroke management which could have a favorable impact on the neurological outcome and quality of life for patients.publishedVersio

Non-Motor Symptoms and Associated Factors in Parkinson’s Disease Patients in Addis Ababa, Ethiopia: A Multicenter Cross-Sectional Study

BACKGROUND: Non-motor symptoms (NMSs) of Parkinson’s disease (PD) were often overlooked and less studied. Little is known about NMSs in Ethiopia. The aim of the study was to determine the prevalence of NMSs and associated factors.METHODS: A multi-center cross-sectional observational study was conducted. NMS questionnaire was used to screen for the NMSs. Both descriptive and analytical statistics were used to analyze the data.RESULTS: Total of 123 PD patients with median of 4 years were investigated. The mean age of PD patients was 62.9 years. The mean age of PD onset was 58.3 years. In 23.6% the age of onset was below age 50. Males accounted 72.4%. Majority of the patients were on Levodopa alone and 31.7% were on levodopa plus trihexyphenidyl. Longer duration of illness was associated with frequent occurrence of NMSs. Constipation was the commonest NMS (78%), followed by urinary urgency (67.5%) and nocturia (63.4%). An unexplained pain was reported by 45.5%, cognitive impairment (45.5%), and sleep disturbance was reported by 45.5% of the study participants. Neurophysciatric symptoms were reported by small proportion of the patients. Lower monthly earning was associated with swallowing problem, unexplained weight change, and lighheadness.CONCLUSION: The prevalence of NMS was high among PD patients in Ethiopia. Constipation was the commonest NMS. Longer duration of illness was associated with frequent occurrence of NMSs. Lower monthly earning was associated with swallowing problem, unexplained weight change, and lighheadness

Underrepresented Populations in Parkinson's Genetics Research: Current Landscape and Future Directions

BACKGROUND: Human genetics research lacks diversity; over 80% of genome-wide association studies have been conducted on individuals of European ancestry. In addition to limiting insights regarding disease mechanisms, disproportionate representation can create disparities preventing equitable implementation of personalized medicine. OBJECTIVE: This systematic review provides an overview of research involving Parkinson's disease (PD) genetics in underrepresented populations (URP) and sets a baseline to measure the future impact of current efforts in those populations. METHODS: We searched PubMed and EMBASE until October 2021 using search strings for "PD," "genetics," the main "URP," and and the countries in Latin America, Caribbean, Africa, Asia, and Oceania (excluding Australia and New Zealand). Inclusion criteria were original studies, written in English, reporting genetic results on PD from non-European populations. Two levels of independent reviewers identified and extracted information. RESULTS: We observed imbalances in PD genetic studies among URPs. Asian participants from Greater China were described in the majority of the articles published (57%), but other populations were less well studied; for example, Blacks were represented in just 4.0% of the publications. Also, although idiopathic PD was more studied than monogenic forms of the disease, most studies analyzed a limited number of genetic variants. We identified just nine studies using a genome-wide approach published up to 2021, including URPs. CONCLUSION: This review provides insight into the significant lack of population diversity in PD research highlighting the immediate need for better representation. The Global Parkinson's Genetics Program (GP2) and similar initiatives aim to impact research in URPs, and the early metrics presented here can be used to measure progress in the field of PD genetics in the future. © 2022 International Parkinson and Movement Disorder Society

Structured headache services as the solution to the ill-health burden of headache: 1. Rationale and description

In countries where headache services exist at all, their focus is usually on specialist (tertiary) care. This is clinically and economically inappropriate: most headache disorders can effectively and more efficiently (and at lower cost) be treated in educationally supported primary care. At the same time, compartmentalizing divisions between primary, secondary and tertiary care in many health-care systems create multiple inefficiencies, confronting patients attempting to navigate these levels (the “patient journey”) with perplexing obstacles. High demand for headache care, estimated here in a needs-assessment exercise, is the biggest of the challenges to reform. It is also the principal reason why reform is necessary. The structured headache services model presented here by experts from all world regions on behalf of the Global Campaign against Headache is the suggested health-care solution to headache. It develops and refines previous proposals, responding to the challenge of high demand by basing headache services in primary care, with two supporting arguments. First, only primary care can deliver headache services equitably to the large numbers of people needing it. Second, with educational supports, they can do so effectively to most of these people. The model calls for vertical integration between care levels (primary, secondary and tertiary), and protection of the more advanced levels for the minority of patients who need them. At the same time, it is amenable to horizontal integration with other care services. It is adaptable according to the broader national or regional health services in which headache services should be embedded. It is, according to evidence and argument presented, an efficient and cost-effective model, but these are claims to be tested in formal economic analyses

The frequency and impact of admission hyperglycemia on short term outcome of acute stroke patients admitted to Tikur Anbessa Specialized hospital, Addis Ababa, Ethiopia: a cross-sectional study

Background: Admission hyperglycemia (HG) has been associated with worse outcomes among acute stroke patients. A better understanding and awareness of the potentially adverse influence of hyperglycemia on the clinical outcome of acute stroke patients would help to provide guidance for acute stroke management and prevention of its adverse outcomes. We aimed to assess the frequency of admission hyperglycemia and its impact on short term (30-days) morbidity and mortality outcomes of stroke in adult Ethiopian patients in an urban setting. Methods: A prospective, cross-sectional study was conducted among acute stroke patients admitted to Tikur Anbessa Specialized Hospital (TASH), within 72 h of symptom onset, from July to December 2016. Socio-demographic data, neuroimaging findings and capillary blood glucose values were obtained on admission. Hyperglycemia was defined as > 140 mg/dl. National Institute of Health Stroke Scale (NIHSS) and modified Rankin Scale (mRS) were used to assess the baseline stroke severity and the 30-days post-stroke outcome, respectively. Results: A total of 103 first-ever acute stroke patients were included (mean age = 55.5 + 15.3 years, 64.1% male and 65% under the age of 65 years) and 51 (49.5%) were hyperglycemic at time of admission. The median admission NIHSS score was worse in the hyperglycemic patients 14 (IQR 10–19) compared to normoglycemic patients 11 (IQR 8–15). Among stroke survivors, patients with hyperglycemia were 3.83 times (95% CI, 1.99–6.19) more likely to be functionally impaired (mRS = 3–5) at 30-days compared to normoglycemic patients (P = 0.041).Older age (≥ 65 years) (P = 0.017) and stroke severity (NIHSS > 14) (P = 0.006) at admission were both significantly associated with poor functional recovery at 30-day. Among patients who died at 30-day, two-third (66.7%) were hyperglycemic but they failed to show any significant association. Conclusions: Hyperglycemia is prevalent among Ethiopian stroke patients at the time of presentation and it is associated with significantly poor functional recovery at 30th-day of follow up. This finding provides a rationale for achieving normal blood glucose in the course of acute stroke management which could have a favorable impact on the neurological outcome and quality of life for patients

Neuroimaging Features and Associated Factors in Multiple Sclerosis Patients: A Perspective from a Private Care Center in Addis Ababa, Ethiopia

BACKGROUND፡ Brain and spine magnetic resonance image (MRI) have an invaluable importance in diagnosing multiple sclerosis (MS) in low prevalence countries such as Ethiopia. The objective of our study was to characterize the neuroimaging features and associated factors in Multiple sclerosis patients inAddis Ababa, Ethiopia.METHOD: A cross-sectional observational study was conducted in 30 multiple sclerosis patients at Yehuleshet Specialty Clinic, Addis Ababa, Ethiopia. Both descriptive and analytical statistics were used to analyze the data.RESULTS: We have enrolled 30 patients with confirmed multiple sclerosis and clinically isolate syndrome. The mean age was 34.7 years (1SD=8.9). Female accounted 86.7%. The mean duration of illness was 3.4 years (1SD=3.1) (range: 1 – 11 years). Relapsing and remitting variant was the commonest sub type (66.7%). Alcohol use and head injury were the commonest identified risk factors reported by the patients. Classical radiological features of MS such as white matter lesions involving juxtacortical, U-fiber, corpus callosum (CC), and Dawson’s finger projections pattern were observed in 46.7%, 23.3%, 70%, and 40% respectively. Cervical and thoracic cords were affected in 40% and 6.7% respectively. Global cortical and CC atrophy was observed in 16.7% and 6.7% respectively. Advanced age was associated with lesions of corpus callosum when adjusted for duration of illness and history of head injury (AOR 1.13, 95% CI 1.01-1.28, p=0.04).CONCLUSION: Typical neuroimaging features of MS were prevalent among Ethiopian MS patients. Age was an independent predictor of lesions involving corpus callosum. Global cortical atrophy was common among Ethiopian MS patients

The prevalence of headache disorders in children and adolescents in Ethiopia: a schools-based study

Background The Global Burden of Disease (GBD) study establishes headache as the second-highest cause of disability worldwide. Because most headache data in GBD are from adults, leading to underestimation of headache-attributed burden, a global schools-based programme within the Global Campaign against Headache is contributing data from children (7–11 years) and adolescents (12–17 years). This national study in Ethiopia is the first in this programme reported from sub-Saharan Africa. Methods A cross-sectional survey following the generic protocol for the global study was conducted in six schools (urban and rural), in Addis Ababa city and three regions of Ethiopia. Structured questionnaires were self-completed under supervision by pupils within their classes. Headache diagnostic questions were based on ICHD-3 beta criteria but for the inclusion of undifferentiated headache (UdH). Results Of 2349 potential participants, 2344 completed the questionnaire (1011 children [43.1%], 1333 adolescents [56.9%]; 1157 males [49.4%], 1187 females [50.6%]), a participation proportion of 99.8%. Gender- and age-adjusted 1-year prevalence of headache was 72.8% (migraine: 38.6%; tension-type headache: 19.9%; UdH: 12.3%; all headache on ≥15 days/month: 1.2%; probable medication-overuse headache: 0.2%). Headache was more prevalent in females (76.2%) than males (71.0%), a finding reflected only in migraine among the headache types. Headache was more prevalent among adolescents (77.6%) than children (68.4%), reflected in all types except migraine, although prevalence of UdH fell sharply after age 14 years to 3.9%. For headache overall, findings matched those in Turkey and Austria, obtained with the same questionnaire, but the high prevalence of migraine, not increasing with age, was surprising. The study highlighted diagnostic difficulties in young people, especially when poorly educated, with migraine diagnoses driven by improbably high proportions reporting nausea (44.8%) and vomiting (28.0%) as usual symptoms accompanying their headaches. Conclusions Headache is very common in children and adolescents in Ethiopia. This has major public-health implications, since half the country’s population are aged under 18 years

The burden attributable to primary headache disorders in children and adolescents in Ethiopia: estimates from a national schools-based study

Abstract Background We previously reported high prevalences of headache disorders among children (6–11 years) and adolescents (12–17 years) in Ethiopia. Here we provide data on headache-attributed burden collected contemporaneously from the same study participants. Part of the global schools-based programme within the Global Campaign against Headache, the study is the first to present such data from sub-Saharan Africa. Methods A cross-sectional survey following the generic protocol for the global study was conducted in six schools (urban and rural), in Addis Ababa city and three regions of Ethiopia. The child or adolescent versions of the Headache-Attributed Restriction, Disability, Social Handicap and Impaired Participation (HARDSHIP) structured questionnaires were self-completed under supervision by pupils in class. Headache diagnostic questions were based on ICHD-3 beta but for the inclusion of undifferentiated headache (UdH). Results Of 2,349 eligible participants, 2,344 completed the questionnaires (1,011 children [43.1%], 1,333 adolescents [56.9%]; 1,157 males [49.4%], 1,187 females [50.6%]; participating proportion 99.8%). Gender- and age-adjusted 1-year prevalence of headache, reported previously, was 72.8% (migraine: 38.6%; tension-type headache [TTH]: 19.9%; UdH: 12.3%; headache on ≥ 15 days/month (H15+): 1.2%). Mean headache frequency was 2.6 days/4 weeks but, with mean duration of 2.7 h, mean proportion of time with headache was only 1.0% (migraine: 1.4%; TTH: 0.7%; H15+: 9.1%). Mean intensity was 1.8 on a scale of 1–3. Symptomatic medication was consumed on about one third of headache days across headache types. Lost school time reportedly averaged 0.7 days over the preceding 4 weeks, representing 3.5% of school time, but was 2.4 days/4 weeks (12.0%) in the important small minority with H15+. However, actual absences with headache the day before indicated averages overall of 9.7% of school time lost, and 13.3% among those with migraine. Emotional impact and quality-of-life scores reflected other measures of burden, with clear adverse impact gradients (H15 + > migraine > TTH > UdH). Conclusions The high prevalence of headache among children and adolescents in Ethiopia, who represent half its population, is associated with substantial burden. Lost school time is probably the most important consequence. Estimates suggest a quite deleterious effect, likely to be reflected in both individual prospects and the prosperity of society