An Irreversible Radiological Finding of Lentiform Fork Sign in a Patient With Uremic Encephalopathy: A Case Report.

Basal ganglia are highly metabolically active deep gray matter structures that are commonly affected by toxins, metabolic abnormalities, and systemic, degenerative, and vascular conditions. Basal ganglion affected by uremic encephalopathy can typically result in a "Lentiform fork sign" on T2-weighted imaging (T2WI) and a fluid-attenuated inversion recovery (FLAIR) sequence of magnetic resonance imaging (MRI). This sign represents bilateral symmetrical hyperintensities in the basal ganglia surrounded by a characteristic hyperintense rim demarcating the lentiform nucleus from surrounding structures. This finding is also reported in other conditions resulting in metabolic acidosis from any cause, e.g., diabetic ketoacidosis, organic acidemias, dialysis disequilibrium syndrome, and drugs like metformin. In an appropriate clinicopathological context, the presence of this sign helps in the accurate diagnosis of uremic encephalopathy. The peculiarity lies in the reversible nature of these lesions and their affective response to treatment. However, sometimes these lesions may not be reversible. We present the case of a 60-year-old female who presented to the ER with chief complaints of fever and altered sensorium. Laboratory workup revealed deranged renal function tests (RFTs) and leukocytosis with pyuria. MRI of the brain showed bilateral basal ganglion hyperintensities on T2WI/FLAIR images characteristic of the lentiform fork sign. Therefore, a diagnosis of uremic encephalopathy due to acute kidney injury (AKI) secondary to septic shock from urosepsis was made. The patient was managed conservatively with IV hydration and antibiotics. Remarkable clinical recovery was seen over three weeks. The patient was stable on a five-month follow-up. However, the repeat MRI did not show resolution of the lesions.

An analysis of breakthrough seizures and related factors in paediatric epilepsy patients.

OBJECTIVE: To determine the frequency of breakthrough seizures among paediatric patients suffering from epilepsy, and factors related to the precipitation of these seizures. METHODS: The cross-sectional study was conducted from July 1, 2018, to July 1, 2020, at the Combined Military Hospital Lahore and the Military Hospital, Rawalpindi, Pakistan, and comprised children of either gender aged 2-12 years diagnosed with any type of epilepsy presenting at the children outpatient department. Diagnosis of epilepsy was established by either a consultant adult neurophysician or a consultant paediatrician. The presence of breakthrough seizures was assessed by obtaining detailed history from the patient and the primary caregiver. Socio-demographic profile, duration of epilepsy and poly-pharmacy were noted. Data was analysed using SPSS 23. RESULTS: Of the 450 subjects, 259(57.6%) were boys and 191(42.4%) were girls. The overall mean age was 6.353±4.732 years. The presence of breakthrough seizures was noted in 227(50.4%) subjects. Children with young age, with low family income, and those in need of poly-pharmacy showed significantly higher odds for breakthrough seizures (p<0.05). CONCLUSIONS: The incidence of breakthrough seizures in epileptic children was high despite the anticonvulsant agents that were previously effective in controlling seizures.

Psychiatric morbidity among informal caregivers of children suffering from intellectual developmental disorders.

OBJECTIVE: To assess the presence of psychiatric morbidity and associated socio-demographic factors among informal caregivers of children suffering from intellectual developmental disorders. METHODS: The analytical cross-sectional study was conducted at the Neurology Department of a tertiary care hospital in Rawalpindi, Pakistan, from January 1, 2018, to December 31, 2019, and comprised informal caregiver of children diagnosed with intellectual developmental disorders presenting at the paediatric or neurology outpatient clinics of the hospital. Intellectual developmental disorder was diagnosed by consultant neurologists or psychiatrists or paediatricians on the basis of International Classification of Diseases-11 criteria. Psychiatric morbidity in the informal caregiver was assessed using the 12-item general health questionnaire. Data was analysed using SPSS 23, and binary logistic regression was applied to assess association. RESULTS: Of the 500 informal caregivers, 323(64.6%) showed psychiatric morbidity. Increasing age and comorbid epilepsy among the patients were significantly related to the presence of psychiatric morbidity among the informal caregivers (p<0.05). CONCLUSIONS: Majority of informal caregivers of children with learning difficulties were found to have psychiatric morbidity.

Need for Swift Diagnosis of Primary Angiitis of Central Nervous System: A Case With Focal Motor Seizures of Hand Progressing to Aphasia.

Primary angiitis of the central nervous system (PACNS) is a rare disorder and difficult to diagnose. The time period from the presentation of the patient until a diagnosis can be protracted, which may result in further progression of the disease and poor patient outcomes. It has immensely varied symptomatology, further confounding swift diagnosis. We present a case of a 33-year-old male who had focal motor seizures with intact awareness of the right upper limb for three weeks prior to presenting to our hospital with acute right hemiparesis. One month into hospital admission, the patient developed complete motor aphasia while being investigated for the cause of multiple ischemic brain infarcts. The patient underwent immunosuppression along with plasmapheresis and pulse steroid therapy started prophylactically on suspicion of PACNS, which was subsequently confirmed by brain biopsy. Disease remission was achieved with rituximab.

Central Nervous System Vasculitis Secondary to Sarcoidosis: A Rare Case of Lupus Pernio With Complete Occlusion of Right Internal Carotid Artery.

Sarcoidosis is a systemic inflammatory disorder resulting from an inappropriate immune response to ubiquitous environmental stimuli. It has a predilection for African Americans and people of Northern European countries. The classic histology is that of a non-caseating granuloma. Central nervous system involvement is a rare occurrence in sarcoidosis and even in this manifestation, the presence of vasculitis is comparatively uncommon. We present a case of a 35-year-old female, who presented with complaints of persistent headache of moderate intensity and had a violaceous plaque on nose, being treated by a dermatologist. The patient on further workup had mildly raised proteins on cerebrospinal fluid analysis. MRI brain showed multiple foci in bilateral frontoparietal regions and centrum semiovale, while digital subtraction angiography brain depicted vasculitis of small vessels of brain and complete occlusion of right internal carotid artery at its origin. Biopsy of lesion on nose was performed that showed chronic granulomatous inflammation. A diagnosis of brain vasculitis secondary to sarcoidosis was made. The patient was treated with plasmapheresis and pulse steroid therapy initially, and later on with cyclophosphamide and azathioprine. This resulted in resolution of headache and nose lesion.

Clinical analysis of reversible splenial lesion syndrome in Chinese adults: A retrospective study of 11 cases.

Reversible splenial lesion syndrome (RESLES) is a clinico-radiological entity that defines a reversible lesion in the splenium of the corpus callosum (SCC) on magnetic resonance imaging (MRI). The clinical and radiological characteristics of RESLES are poorly defined and most RESLES literature is in the form of case reports. We reviewed the clinical and radiological data from 11 RESLES patients in order to more clearly describe the characteristics of this disorder in adults.Patients included in this study were diagnosed with RESLES from May 2012 to March 2018. We collected clinical, imaging, and laboratory data of 11 adult patients from Neurology Department of the Affliated Yantai Yuhuangding Hospital of Qingdao University. After analyzing various clinico-radiological features and laboratory parameters, including serum sodium, pathogen testing, cerebrospinal fluid (CSF) studies, electroencephalography (EEG), and MRI findings, we made a diagnosis of RESLES based on the criteria proposed previously by Garcia-Monco et al.Of the 11 patients, 7 (63.63%) were male and 4 (36.36%) were female, ranging in age from 24 to 62 years with an average age of 31.48 ±â€Š11.47 years. Seven cases occurred in the months of winter and spring (December-March). The primary clinical symptoms were headache, seizure, disturbance of consciousness, mental abnormality, and dizziness. All 11 patients had lesions in the SCC and all the lesions disappeared or significantly improved on follow-up imaging that was done within a month of symptom resolution.We found 5 (45.45%) patients had a CSF opening pressure >180 mmH2O, in addition to elevated protein and(or) leukocytes levels in 3 (27.27%) patients. The serum sodium concentration in 6 (54.55%) patients was low (<137 mmol/L) and EEG showed nonspecific slowing in waves 4 (36.36%) patients.When we encounter clinical manifestations such as headache accompanied with mental symptoms, disturbance of consciousness or epilepsy, and brain MRI finds lesions of the corpus callosum, we should consider whether it is RESLES. In order to find out the possible cause of the disease, we should carefully inquire about the history of the disease, complete etiology examination, and CSF tests. Of course, it is one of the necessary conditions for the diagnosis that the lesions in the corpus callosum are obviously relieved or disappeared.

Transvenous Catheter-Based Thrombolysis With Continuous Tissue Plasminogen Activator Infusion for Refractory Thrombosis in a Patient With Behcet's Disease.

Behcet's disease (BD) classically presents with recurrent oral ulcers, genital ulceration, uveitis and skin manifestations. Middle-aged people are usually affected with the male gender being associated with severe variant of the disease. It can involve any organ system of the body. Although central nervous system and vascular involvement tend to occur less frequently, they are the commonest cause of mortality. We present a case of a 30-year-old man referred with suspicion of cerebral venous sinus thrombosis to our hospital and subsequently diagnosed with BD. The patient developed, despite being on immunosuppression and anticoagulation, extensive arteriovenous thrombi of lower limbs requiring catheter-directed thrombolysis with continuous 24-hour infusion of tissue plasminogen activator for refractory right lower limb venous thrombosis and placement of inferior vena cava filter to prevent pulmonary embolism. Later disease remission was achieved with rituximab.

Central Nervous System Tuberculosis With Shower Like Pattern of Intracranial Tuberculomas in an Immunocompetent Patient.

Central nervous system tuberculosis (CNS TB), though comparatively uncommon as compared to other forms of extrapulmonary tuberculosis (TB), results in high morbidity and mortality. The symptoms are non-specific and of a progressive nature resulting in delayed diagnosis. We present a case of CNS TB that presented two months after the onset of symptoms. The patient's condition reached the point of being bedbound. On investigation, the widespread involvement of the brain with shower-like pattern of ring enhancing tuberculomas and associated tuberculous meningitis was found. This was a surprising finding considering the patient was immunocompetent. Contrast-enhanced magnetic resonance imaging showed lesions in midbrain, pons, medulla, thalamus, bilateral cerebellar hemispheres and bilateral cerebral hemispheres. Upon treatment initiation the patient responded well with resolution of all lesions.

Evaluating toxicity impacts of environmental exposed chromium on small Indian mongoose (Urva auropunctatus) hematological, biochemical and histopathological functioning.

Sub-lethal toxic impacts of chromium on hematological, biochemical and histological parameters were analyzed in the female small Indian mongoose (Urva auropuctatus) residing contaminated environment of tannery industry. Chromium bioaccumulation in the blood, liver and kidney tissue of the exposed mongooses was found elevated compared to the control mongooses' tissues. Total body weight (75.7%), liver weight (83.6%) as well as HSI (68.1%), RSI (86.2%) and the platelets counts (59.7%) were found significantly elevated, with significantly reduced RBCs (59.6%), and WBCs (64%). LFT and RFT were also found abnormal, moreover, the histopathological injuries had been distinct inside the kidney (>75%) and hepatic (>75%) tissues of exposed animals. Shrinkage and vacuolization (>75%) inside the hepatocyte expanded sinusoidal spaces and nuclear pyknosis (>75%) was evident within the hepatic tissue. Hypertrophy of epithelial cells of renal tubules and inter-renal cells of the head kidney with a reduction in tubular lumens (>75%) and vacuolization of tubules were witnessed within the kidney section. Atrophy inside the kidney inter-renal cells, glomeruli compression within the Bowman's capsules (>75%) following the necrosis in hematopoietic tissues were found in exposed animals. The present findings indicate that chronic exposure to chromium induces severe anemia, decreased serum protein concentration, hepatic and renal tissue histopathology, impairing the vital capabilities of liver, metabolic regulation, excretion, and stress homeostasis maintenance of which within the long-run may posture a severe risk to animal well-being then distress their inhabitants.

ONION PEEL APPEARANCE IN BALOS CONCENTRIC SCLEROSIS--A VARIANT OF MULTIPLE SCLEROSIS.

Balo's concentric sclerosis (BCS) is a variant of multiple sclerosis (MS). It may present as a lesior clinically and radiologically indistinguishable from brain tumour particularly on computerized tomography (CT) scans. Diagnosis only gets clear when magnetic resonance imaging and spectroscopy (MRI & MRS) and brain biopsy is done. We report a case of 30 year old male with progressive headache and left hemi paresis for 3 weeks. There was upper motor neuron (UMN) facial palsy on the left with bilateral papilledema. CT scan of brain showed large hypo-dense area in right frontoparietal lobe consistent with brain tumour. On MRI the diagnosis of BCS was made on basis of concentric lesions of myelinated and demyelinated rings. Demyelination wa confirmed on brain biopsy.

CRYPTOCOCCAL MENINGITIS IN IMMUNOCOMPETENT PATIENT.

Cryptococcal meningitis (CM) is life threatening fungal infection of central nervous system (CNS). Although it is commonly associated with immunosuppression but rarely it can occur in immune competent patient. We report a case of 21 year old non HIV infected girl. Based on initial diagnoses of tuberculosis Bacillus meningitis (TBM), she was started on anti-tuberculosis treatment (ATT). However failure to respond to treatment prompted a quest for alternative diagnosis. A final Diagnosis of CM was confirmed on latex agglutination antigen detection on cerebrospinal fluid (CSF) analysis. The patient responded well to antifungal treatment. Initially diagnosis was missed due to common occurrence of tuberculosis infection in Pakistan and resemblance of its symptomatology and magnetic resonance imaging (MRI) findings with CNS cryptoccocal infection.

Non-moyamoya bilateral middle cerebral artery agenesis mimicking multiple sclerosis.

We present a case of a 41-year-old woman who carried the misdiagnosis of multiple sclerosis for 13 years while on disease modifying therapy [DMT]. Her neurologic work-up showed an extremely rare type of bilateral middle cerebral arteryocclusive disease, a basilar apex aneurysm and paroxysmal atrial fibrillation. A thorough search for alternative neurologic diagnosis is recommended before patients are given a definitive diagnosis and committed to DMT.