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

Symptomatic Giant Virchow-Robin Spaces: A Rare Cause of Spastic Quadriparesis in 43-Year-old Ethiopian Patient: A Case Report

BACKGROUND: Virchow-Robin Spaces (VRS) are perivascular spaces that surround small arteries and arterioles. These normal anatomical structures are thought to be involved in the drainage of interstitial fluid and also to play an immunomodulatory role by hosting macrophages. Rarely, it becomes giant and symptomatic resulting in mass effect on adjacent neuronal structures and ventricular system causing different neurological disorders.CASE PRESENTATION: We report a 43-year-old, Ethiopian woman who presented with progressive weakness of all her extremity over the period of seven years. She had associated speech difficulty, visual blurring and pseudo-bulbar affect. Neurologic examination revealed spastic quadriparesis with increased deep tendon reflexes and up going plantar bilaterally. She had horizontal nystagmus, dysarthria and reduced bilateral visual acuity, otherwise normal cognition and cranial nerves examination. Brain MRI showed T1 hypointense, T2 hyperintense and non-enhancing multiple cystic lesions of different size, mainly in bilateral basal ganglia area with mass effect on adjacent internal capsule and lateral ventricles. Considering her clinical presentation and typical radiological features, diagnosis of symptomatic dilated Virchow-Robin spaces was made, and the patient was treated symptomatically.CONCLUSION: Commonly, dilation of Virchow-Robin spaces are not symptomatic, but giant Virchow-Robin spaces, as in our patient may result in spastic quadriparesis, causing great disability on the patient. Thus, we recommend considering symptomatic Virchow- Robin spaces as a potential differential diagnosis of progressive quadriparesis, as early neurosurgical intervention may reduce the neurological complications, such as spastic quadriparesis.&nbsp

Giant Pituitary Adenoma Presenting with Foster-Kennedy Syndrome in a 21-Year Old Ethiopian Patient: A Rarely Reported Phenomenon: A Case Report

BACKGROUND: Disorders of the pituitary gland and the sellar region present a wide spectrum of clinical problems. A variety of lesions in this area tend to present with similar problems; namely, headache, hormonal disorders, and loss of vision. Benign adenomas of the pituitary gland are by far the most common disorders of sellar region. Rarely, patients with pituitary adenoma may present with blindness in one eye and visual field cut (temporal hemianopia) in the contralateral eye. This rare clinical entity is called Foster-Kennedy Syndrome (FKS).CASE PRESENTATION: We present a 21-year-old male patient, presented with progressive visual disturbance of the left eye, associated with global headache. The headache was refractory to over-counter medications. In addition, he had history of sleep disturbance and loss of appetite. Neurological examination was pertinent for left side visual loss with optic atrophy and right eye temporal visual field cut with disc edema. Brain MRI showed 4 cm X 3.5 cm sized lobulated intra and supra sellar mass with heterogeneous contrast enhancement and minimal surrounding edema. Serum prolactin level was 6,705 ng/mL. Otherwise, the other pituitary hormones were in normal range.CONCLUSION: This case highlighted that pituitary adenoma may present with the full picture of Foster Kennedy syndrome. Therefore, we recommend considering pituitary adenoma as a possible differential diagnosis of intracranial lesions presenting as Foster Kennedy syndrome, as early detection and management could potentially salvage patients’ vision and quality of life

Sellar tuberculoma: a rare presentation in a 30-year-old Ethiopian woman: case report

Background: Tuberculosis rarely involves the hypothalamo-pitutary axis. Only 1% of CNS Tuberculoma involves sellar and suprasellar structures. It mainly presents with symptoms of chiasmal compression, hypopituitarism and diabetes insipidus due to destruction of pituitary itself. Definitive diagnosis is made by tissue analysis collected by trans-sphenoidal biopsy.Case presentation: We report a 30-year-old female patient from Addis Ababa, who presented with a global headache, blurring of vision, excessive polyuria, constitutional symptoms of tuberculosis and multiple axillary lymph node enlargement. Biopsy from the axillary lymph node showed caseous granulomatous lesions and brain MRI showed mild thickening of the pituitary stalk and mildly enlarged posterior piturary lobe with hypo-sellar extension and mild communicating hydrocephalus.Conclusion: Considering the available evidence a diagnosis of systemic tuberculosis involving pituitary, lung and lymph node was made and a patient was started on anti-tuberculosis medication together with dexamethasone and carbamazepine. Following treatment initiation patient showed significant clinical improvement. After nine months of anti-tuberculosis treatment all brain MRI findings were resolved and patient become symptom free and discharged from care after one year completion of anti-tuberculosis medication.Keywords: Tuberculoma, Polyuria, Pituitary gland, Diabetes Insipidu

A mixed-methods assessment of Routine Health Information System (RHIS) Data Quality and Factors Affecting it, Addis Ababa City Administration, Ethiopia, 2020

AbstractBackground: Effective and efficient health care services need evidence-based decisions, and these decisions should rely on information from high-quality data. However, despite a lot of efforts, routine health data is still claimed to be not at the required level of quality. Previous studies have primarily focused on organization-related factors while little emphasis was given for perception and knowledge of service providers' gaps. Therefore, this study aims to evaluate the quality of data generated from routine health information systems and factors contributing to data quality from diverse aspects. Objective: This study aims in assessing the quality of routine health information system data generated from health facilities in Addis Ababa city administration, providing the level of data quality of routine health information system, and factors affecting it. Method: A cross-sectional study was conducted on 568 health professionals from 33 health centers selected randomly using a two-stage sampling method. A qualitative study was also conducted using 12 key informants. Result: The overall regional data quality level was 76.22%. Health professionals' motivation towards routine health care data have shown a strong association with data quality, (r (31) =.71, p<.001). Lack of adequate Health information system task competence, non-functional PMT, and lack of supervision was also commonly reported reasons for poor data quality. Conclusion: This review has documented the data quality of routine health information systems from health centers under Addis Ababa city. Overall data quality (76.22%) was found to be below the national expectation level, which is 90%. The study emphasized the role of behavioral factors in improving the quality of routine health care data. [Ethiop. J. Health Dev. 2021; 35(SI-1): 15-24 ] Keywords: RHIS, Accuracy, completeness, timeliness, consistency, Addis Abab

Bilateral subdural hematoma a rare complication of common procedure in 30-year-old female patient: A case report and literature review

Background: Lumbar puncture (LP) is a frequent procedure done for administration of spinal anesthesia or for obtaining cerebrospinal fluid for analysis. The common complications of LP are pain at the local site and post LP headache. Rarely the following complication could occur: infections of central nervous system, brain stem herniation, and subdural hematoma. Post LP hematoma needs to be suspected in those patients whom post LP conservative management failed and headache lasts more than one week.Case presentation: We report a 30-year-old, Ethiopian woman who present with bilateral subdural hematoma following lumbar puncture for spinal anaesthesia. She presented with three weeks history of persistent headache, nausea and vomiting. The symptoms started three days following spinal anaesthesia procedure done for cesarean section.Conclusion: Post-dural puncture headache is the commonest benign complication of lumbar puncture. However, change in character of headache, absence of response to routine measures, development of neurological signs, and persistence of headache beyond one week should prompt physicians to consider rare and fatal complications of post-dural puncture such as subdural hematoma. Moreover, timely diagnosis and management is often associated with good prognosis. French title: Hematome sous dural bilateral secondaire a une ponction lombaire   Introduction: La ponction lombaire (PL) est une procédure fréquente effectuée pour l'administration de l'anesthésie rachidienne ou pour obtenir du liquide céphalo-rachidien pour analyse. Les complications courantes de la PL sont la douleur au site local et la céphalée post PL. Les complications suivantes sont plus rares : infections du système nerveux central, engagement du tronc cérébral et hématome sous-dural. Un hématome post PL doit être suspecté chez les patients dont la prise en charge conservatrice du syndrome post PL a échoué et chez lesquels les maux de tête durent plus d'une semaine. Observation :Nous rapportons le cas d’une femme éthiopienne de 30 ans qui a présenté un hématome sous-dural bilatéral après une ponction lombaire pour une rachianesthésie. Elle avait des antécédents de céphalées persistantes, de nausées et de vomissements pendant trois semaines. Les symptômes ont commencé trois jours après la procédure d'anesthésie rachidienne effectuée pour la césarienne. Conclusion: La céphalée post-PL est la complication bénigne la plus courante de la ponction lombaire. Cependant, la modification du caractère des céphalées, l'absence de réponse aux mesures de routine, le développement de signes neurologiques et la persistance des céphalées au-delà d'une semaine devraient inciter les médecins à envisager des complications rares et mortelles de la PL telles qu'un hématome sous-dural. De plus, un diagnostic et une prise en charge rapides sont souvent associés à un bon pronostic. &nbsp

Clinico-pathological Diagnosis of Facioscapulohumeral Dystrophy in a 22-year-old Male

Background: Facioscapulohumeral dystrophy (FSHD) is a rare hereditary disease with a prevalence of 2.03–6.8 per 100,000 individuals. FSHD is the third most common type of muscular dystrophy after the Duchene muscular dystrophy and myotonic dystrophy. To the best of our knowledge, the current case report is the first to report probable FSHD case mainly diagnosed using clinico-pathological evidence from sub-Saharan Africa (SSA). Case Report: A 22-year-old right-handed male college student presented with progressive proximal muscular weakness associated with wasting. The weakness started from the bilateral facial muscles and progressively involved proximal upper and lower limbs muscles associated with scapular winging, waddling gait, and bilateral foot drops. His bulbar, sensory, autonomic, and cognitive systems were spared. Muscles EMG showed myopathic patterns and normal serum CK. Muscle biopsy from affected muscles showed variation in fiber size with groups of angular fibers, preserved fibers, and hypertrophic fibers with marked fibrosis and adipose tissue replacement with no apparent inflammation and necrosis which is consistent with pathological features of muscular dystrophy. Considering the clinical semiology, physical findings, EMG findings, and pathological findings diagnosis of FSHD of scapuloperoneal variant was made. The patient was managed with analgesics, nutritional advice, and ankle prosthesis for foot drops. Currently, the patient is in a similar condition with modest improvement in his musculoskeletal pain complaints. Conclusion: This case highlights the fact that a careful clinical evaluation with thorough utilization of diagnostic investigations available at our disposal may support the diagnosis of FSHD in resource-limited areas where the necessary genetic tests were not available. Keywords: facioscapulohumeral muscular dystrophy, dystrophy, clinico-pathology, sub-Saharan Afric

Authenticity and brain health: a values-based perspective and cultural education approach

This perspective paper discusses the concept of authenticity in relation to brain health and neurodegenerative diseases. We define authenticity as being true to oneself and consider it a social value of relevance to neuroscientists, clinicians, and caregivers. From a biological perspective, behaviors that can be interpreted as expressions of authenticity are produced by distributed brain networks. By understanding it as a dynamic process, we argue that harnessing authenticity across the lifespan can be protective by promoting resilience. We discuss the idea of authentic aging, which appreciates the complexity of human life within the world and can enhance positive views of later life. Authenticity is additionally applicable to caring for people with neurodegenerative diseases, both when understanding the behavior of people with dementia and the response of caregivers. Tailoring care to an individual’s personality and strengths may improve their brain health. Finally, we describe an interdisciplinary learning event, themed around masks, designed to engage participants in identifying authenticity in their own work. For scientists, care professionals, and caregivers, reflecting upon authenticity can aid understanding of the person with dementia and therefore improve care

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

Electroencephalographic Findings, Antiepileptic Drugs and Risk Factors of 433 Individuals Referred to a Tertiary Care Hospital in Ethiopia

Background: Little is known about the characteristics of electroencephalogram (EEG) findings in epileptic patients in Ethiopia. The objective of this  study was to characterize the EEG patterns, indications, antiepileptic drugs (AEDs), and epilepsy risk factors.Methods: A retrospective observational review of EEG test records of 433 patients referred to our electrophysiology unit between July 01, 2020 and  December 31, 2021.Results: The age distribution in the study participants was right skewed unipolar age distribution for both sexes and the mean age of 33.8 (SD=15.7) years. Male accounted for 51.7%. Generalized tonic clonic seizure was the most common seizure type. The commonest indication for EEG was  abnormal body movement with loss of consciousness (35.2%). Abnormal EEG findings were observed in 55.2%; more than half of them were Interictal epileptiform discharges, followed by focal/or generalized slowing. Phenobarbitone was the commonest AEDs. A quarter (20.1%) of the  patients were getting a combination of two AEDs and 5.2% were on 3 different AEDs. Individuals taking the older AEDs and those on 2 or more AEDs  tended to have abnormal EEG findings. A cerebrovascular disorder (27.4%) is the prevalent risk factor identified followed by brain tumor, HIV  infection, and traumatic head injury respectively.Conclusion: High burden of abnormal EEG findings among epileptic patients referred to our unit. The proportion of abnormal EEG patterns was  higher in patients taking older generation AEDs and in those on 2 or more AEDs. Stroke, brain tumor, HIV infection and traumatic head injury were  the commonest identified epilepsy risk factors