Clinical profile and dynamic magnetic resonance imaging in Hirayama disease: a single-centered cross-sectional study in Nepal.

Hirayama disease (HD) is juvenile monomelic amyotrophy of the distal upper limb first described by Hirayama in 1959 AD. HD is a benign condition with chronic microcirculatory changes. The hallmark of HD is necrosis of the anterior horns of the distal cervical spine. Materials and Methods: Eighteen patients were assessed for clinical and radiological Hirayama disease. Clinical criteria included insidious onset nonprogressive chronic upper limb weakness and atrophy in teens or early twenties without sensory deficits and coarse tremors. MRI was done in a neutral position followed by neck flexion to evaluate cord atrophy and flattening, abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior shifting of the posterior wall of the cervical dural canal, posterior epidural flow voids, and an enhancing epidural component with its dorsal extension. Results: The mean age was 20.33 years, and the majority, 17 (94.4%), were male. Neutral-position MRI revealed loss of cervical lordosis in 5 (27.8%) patients, cord flattening in all patients with asymmetry in 10 (55.5%), and cord atrophy was observed in 13 (72.2%) patients with localized cervical cord atrophy in only 2 (11.1%) and extension of atrophy to dorsal cord in 11 (61.1%) patients. Intramedullary cord signal change was seen in 7 (38.9%) patients. Loss of attachment of posterior dura and subjacent lamina and anterior displacement of dorsal dura was seen in all patients. A crescent-shaped epidural intense enhancement was noted along the posterior aspect of the distal cervical canal in all patients, with dorsal level extension in 16 (88.89%) patients. The mean thickness of this epidural space was 4.38±2.26 (mean±2SD), and the mean extension was 5.5±4.6 vertebral levels (mean±2SD). Conclusion: The high degree of clinical suspicion can guide additional contrast studies in flexion as a set MRI protocol for early detection and avoiding false negative diagnoses of HD.

Clinico-Radiological Profile of Patients With Lateral Medullary Syndrome: A Five Years Observation From a Single-Centered Tertiary Hospital in Nepal.

OBJECTIVE: We aim to correlate the prevalence of symptoms of the lateral medullary syndrome (LMS) based on radiological classification. METHODS: A five-year record of 41 patients diagnosed with LMS and admitted to a tertiary care center in Nepal was reviewed. We used chi-square tests to compare symptoms between rostral and caudal groups and different horizontal subtypes. RESULTS: The subtype prevalence in the horizontal classification of LMS was large (31.7%), lateral (22%), dorsal (19.5%), typical (14.6%), and ventral (12.2%). The most common symptoms in the typical subtype of the horizontal classification were: pain/temperature loss in the contralateral body (7.3%) and dysphagia (7.3%); in the ventral subtype, swaying on the Romberg test (12.2%), dysarthria (9.8%) and dizziness (9.8%); in the dorsal subtype, headache (12.2%) and vomiting (12.2%). Whereas headache (22.2%) and lateropulsion on standing (14.6%), swaying on the Romberg test (14.6%), nausea/vomiting (14.6%) were common in the large subtype, and nausea/vomiting (19.5%) and headache (17.1%) in the lateral subtypes. Whereas, in rostrocaudal classification, the rostral subtype (61%) was more common than the caudal subtype (31%). There was no significant variation in symptoms based on the rostrocaudal classification of LMS. CONCLUSION: The common clinical manifestations are different for different radiological subtypes of LMS. Further comprehensive studies are essential to understand the prevalence of symptoms in different radiological subtypes and the clinical-radiologic correlation in LMS.

Peripheral Leukocytosis and Clinical Outcomes After Aneurysmal Subarachnoid Hemorrhage.

BACKGROUND: Aneurysmal subarachnoid hemorrhage (SAH) has a high morbidity rate. Following SAH, a powerful systemic inflammatory response ensues contributing to delayed neurological deterioration and outcome. The aim of this study is to investigate if peripheral leukocytosis following SAH impacts clinical outcomes. METHODS: This is a retrospective, observational, single tertiary center study of patients with SAH who underwent microsurgical clipping between 2017 and 2020. The study's inclusion criteria were aneurysmal SAH on baseline computerized tomography (CT), age above 18 years, and hospital admission within 72 hours of bleeding. Traumatic SAH, arteriovenous malformations, and mycotic aneurysms were all excluded. On admission, leukocyte counts were recorded. Demographic and clinical variables were compared between the two groups (TLC ≤12,000 and >12,000). The impact of peripheral leukocytes on clinical outcomes in terms of the Glasgow Outcome Score (GOS) was analyzed. Mann-Whitney U test for continuous variable and Fisher exact test or chi-square test for categorical variables were used for calculation of P-value. A P-value of 0.05 or less was considered statistically significant. RESULTS: Among 90 patients who underwent clipping of ruptured aneurysms, 40 (44.4%) were anterior communicating artery (ACOMM) aneurysms, and 21 (23.3%) were middle cerebral artery(MCA), and 16 (17.8%) were posterior communicating artery (PCOMM). Clinically 57 patients (63.3%) had a World Federation of Neurosurgical Societies (WFNS) grade 1, 17 patients (18.9%) had a grade 2, four patients (4.4%) had a grade 3, and two patients (2.2%) had a grade 4. On radiological examination, six patients (6.7%) had fisher grade 1, 23 patients (25.6%) had grade 2, 22 patients (24.4%) had grade 3, and 39 (43.4%) had grade 4 SAH distribution. Clinical results were poor in 30 individuals (33.3 %), but good in 60 patients (66.7 %). On admission leukocytosis (>12,000) was seen among 34 (37.8%). Leukocytosis (>12,000) was associated with poor WFNS grade (>2); however, statistical significance was not seen with clinical outcome in terms of GOS. CONCLUSION: Poor clinical grade of patients following aneurysmal SAH is associated with peripheral leukocytosis; however, peripheral leukocytosis is not associated with poor outcomes.

Pituitary apoplexy presenting as isolated third cranial nerve palsy: case series.

Pituitary apoplexy (PA) is caused by a sudden increase in pressure in the pituitary region due to acute hemorrhage, infarction or necrosis. PA can also be caused by restricting blood supply to the nerve due to compression of the internal carotid artery. Acute third cranial nerve palsy (third CN) secondary to PA is a rare medical emergency caused by bleeding within a growing mass within the sella turcica. We presented two cases of PA with isolated third CN palsy treated with transsphenoidal pituitary decompression. PA is therefore an important differential diagnosis to consider in patients with isolated third nerve palsy. The prognosis for isolated third nerve palsy in PA appeared successful, with variable recovery from medical and surgical intervention.

Post-traumatic Pseudoaneurysm of the Internal Carotid Artery with Intractable Epistaxis: A Case Report.

Epistaxis is a common otorhinolaryngology emergency. There are several treatment modalities for epistaxis, but bleeding from the internal carotid artery necessitates a particular treatment technique. We report a case of a 22-years old man who presented to us recurrent episodes of epistaxis and blurry vision in the right eye for one month. The patient had undergone maxillo-facial surgery following a road traffic accident one year back. Bleeding episodes were occasionally severe with blood loss of up to 800 to 1000ml. These episodes were managed conservatively with posterior nasal packing and frequent blood transfusions. A computed tomography-angiography revealed a pseudoaneurysm arising from the cavernous segment of the right internal carotid artery which was managed successfully by embolization of the aneurysm sac with coils. Despite the rarity of internal carotid artery pseudoaneurysm in individuals with a history of trauma, doctors must be aware of the possibility. Timely identification and treatment of a pseudoaneurysm can save a person's life.

Mean Evan's Index among Patients with Normal Computed Tomography Scan visiting Radiology Department in a Tertiary Care Centre of Nepal: A Descriptive Cross-sectional Study.

INTRODUCTION: Evan's index is useful to objectively see if ventricles size is abnormal especially in borderline cases of hydrocephalus. Studying ventricular size in CT scan is essential in every pathology of the brain. Use of objective parameters to define hydrocephalus helps us not only to diagnose a case but also follow up the case following treatment. The aim of this study was to find out the mean even index among patients visiting the department of radiology of a tertiary care hospital. METHODS: A descriptive cross-sectional study was conducted at a tertiary care hospital from 1st january 2020 to 31st December 2020. Ethical clearance was obtained from the Institutional Review Committee of Upendra Devkota Memorial Neurological and Allied Sciences (reference number: 116/2021). Computed tomography scans were done for various reasons in the hospital over a one year period and reported normal by the radiologists were included in the study. Convenient sampling was done. Statistical analysis was done using Statistical Package for the Social Sciences. Point estimate at 95% Confidence Interval was calculated along with mean and standard deviation for continuous data. RESULTS: In this study, among the 216 cases, the mean Evan's index was found to be 0.20±0.04. CONCLUSIONS: The mean evan's index in our study population was lower than the normal cut-off value.

Morphometric Measurement of Cranial Vault Thickness: A Tertiary Hospital Based Study.

INTRODUCTION: The skull's main function is to protect the brain. Total skull bone thickness is the total thickness of diploe and the external and internal tables. The measurement of the human skull based on CT images results are of great practical value in the fields of anatomy, clinical medicine, biomechanics study and head injury analysis. There are few literatures about imaging assisted measurement of the cranial vault thickness while sparse literature among Nepalese population. In this study, we aim to measure the thickness of calvarian bones of and find the difference between gender and ethnic groups. METHODS: This was a descriptive cross-sectional study conducted in our center during a period of 6 months. Patient of age 15 to 50 years with normal CT finding were included in the study. Using the axial view of brain CT, the thickness of cranial vault was measured and recorded in millimeter. RESULTS: Among 100 patients, 51 were male and 49 were female. Mean thickness of frontal bone, parietal, temporal and occipital bone were 8.02±1.97 mm, 7.04±1.43 mm, 4.71±1.34 mm and 7.98±2.47 mm respectively. CONCLUSIONS: There was no significant difference in cranial vault thickness among sex or ethnical groups in patients of a hospital.

Role of MR Morphology and Diffusion-Weighted Imaging in the Evaluation of Meningiomas: Radio-Pathologic Correlation.

INTRODUCTION: Preoperative differentiation of benign, atypical and malignant meningiomas would significantly help in surgical planning and treatment. The aim of this study is to look at radio-morphologic behavior of various histopathological types and grades of meningiomas and their diffusion characteristics. METHODS: We performed an analytical cross-sectional study including all patients operated on for meningiomas at our hospital during January 2016 to July 2018. We studied 38 meningiomas in 38 patients aged 14 to 73 years old. All patients underwent MRI prior to surgery, including diffusion-weighted sequences, in a 1.5T scanner. Signal intensity in T2-weighted images, diffusion-weighted images (b=0, 90 and 1,000), and Apparent Diffusion Coefficient maps within the tumors and in the normal parietal white matter as a reference were evaluated. In the histological study, cellularity, proliferation index, histological grade, and cerebral invasion were evaluated. RESULTS: There was female predilection with male:female ratio of 1:2.4. Most meningiomas were supratentorial with most common origin being parafalcine and convexity. Of the 38 meningiomas, 31 were WHO grade I, 6 were WHO grade II (atypical) and one was WHO grade III (anaplastic). Among various tumors' behaviors, incomplete CSF cleft, pial invasion and parenchymal invasion were significantly high in high-grade tumors. Similarly, tumors showing pial invasion, breached tumor-brain interface, no capsular enhancement and parenchyma invasion showed significantly low NADC. Mean ADC value was 0.722±7.7x10-3 mm2/s (normalized ADC 0.9±0.1) in the atypical group and 0.876±24.56x10-3 mm2/s (normalized ADC 1.11±0.31) in the typical group. No statistically significant differences of ADC/NADC were found between histologic subtypes. Two subtypes of typical meningiomas, metaplastic and angiomatous meningioma had the highest values in the ADC maps. CONCLUSIONS: MR morphology like pial invasion, breached tumors brain interface, parenchymal invasion can predict aggressiveness and atypical nature of meningiomas. Meningioma shows moderately restricted diffusion. The signal on the ADC map is associated with tumors cellularity and aggressiveness suggesting its usefulness for predicting the histological grade.

Venture in 101 Cranial Punctures: A Comparative Study between Frame-based versus Frameless Biopsy of 101 Intracranial Space Occupying Lesion.

BACKGROUND: Presumptive diagnosis based solely on the clinical picture and imaging is not sufficient to provide appropriate treatment with certainty and hence histopathological confirmation of intracranial space occupying lesion (ICSOL) is essential. Needle biopsy via stereotactic frame-based or frameless neuronavigation technique is efficient procedure. The objective of this study is to compare their accuracy and efficacy and safety. METHODS: This is a retrospective comparative study conducted among 101 biopsies of ICSOL. Patients data were retrieved from medical record. Data were analyzed in SPSS ver. 20. P value of <0.05 was considered significant. RESULTS: Out of 101 patients, Frame-based stereotactic biopsy was done among 55 patients (54.4%) while 46 patients (45.6%) underwent frameless stealth neuronavigation guided biopsy. Male to female ration was 2.1:1. Age ranged from 5 to 82 years. 54.5% (55 patients) have deeper location of tumor while 45.5% (46 patients) have lobar location of tumor. Frontal (16.8%) and Thalamic (13.8%) were the common site. Mean size of tumor was 3.09±0.85cms. There was statistically significant difference in operative duration among study groups. Overall Diagnostic yield was 89.1%. Glioma was the most common (50.5%) diagnosis. Glioblastoma WHO Grade IV was 37.6% followed by lymphoma (12.8%). CONCLUSION: Needle biopsy via stereotactic frame-based or neuronavigation frameless technique is a safe and efficient procedure having high diagnostic yield. Reasons for negative biopsy could be missed target or retrieval of gliotic tissue.

Clinical Experience with Frame Based Stereotactic Biopsy for Intracranial Space Occupying Lesion.

INTRODUCTION: Preoperative diagnosis of intracranial space occupying lesion based solely on clinical and neuroimaging evaluation may not be sufficient to institute treatment plan without histopathological certainty. Frame based stereotactic biopsy is a technique of retrieving biopsy specimen to determine the histopathology. The aim of this study is to assess the efficacy and accuracy of frame based technique. METHODS: This is a cross-sectional study conducted among 80 patients who underwent computed tomography guided frame based stereotactic biopsy during a period of 6 years. All operations were performed under local anesthesia. Histopathology reports were retrieved and accuracy of biopsy technique analyzed. RESULTS: Out of 80 patients, 58 were male with male to female ratio of 2.6:1. Median age of patients were 50 years with range from 16 to 75 years. Most lesions were in deeper location 49 (61.3%). Most common location was Parietal, 15 (18.8%) followed by Thalamic, 12 (15%). Mean size of lesion was 2.88±0.71cms ranged from 2 to 5cms. Biopsy was accurate to retrieve target in 74 (92.5%) patients. Histopathology revealed glial tumor in 41 (51.2% ) of cases. Overall morbidity was observed in 3 (5.5%) patients. There is no procedure related mortality in this study during study period. CONCLUSIONS: Frame based biopsy of intracranial space occupying lesion is safe and efficacious procedure with high diagnostic yield.