Fahr’s disease or Fahr’s syndrome is a rare, neurological disorder characterized by abnormal calcified deposits in basal ganglia and cerebral cortex. Calcified deposits are made up of calcium carbonate and calcium phosphate, and are commonly located in the Basal Ganglia, Thalamus, Hippocampus, Cerebral cortex, Cerebellar Subcortical white matter and Dentate Nucleus. Molecular genetics of this disease haven’t been studied extensively; hence evidence at the molecular and genetic level is limited. Fahr’s disease commonly affects young to middle aged adults. Etiology of this syndrome does not identify a specific agent but associations with a number of conditions have been noted; most common of which are endocrine disorders, mitochondrial myopathies, dermatological abnormalities and infectious diseases. Clinical manifestations of this disease incorporate a wide variety of symptoms, ranging from neurological symptoms of extrapyramidal system to neuropsychiatric abnormalities of memory and concentration to movement disorders including Parkinsonism, chorea and tremors amongst others. Diagnostic criteria for this disease has been formulated after modifications from previous evidence and can be stated briefly, it consist of bilateral calcification of basal ganglia, progressive neurologic dysfunction, absence of biochemical abnormalities, absence of an infectious, traumatic or toxic cause and a significant family history. Imaging modalities for the diagnosis include CT, MRI, and plain radiography of skull. Other investigations include blood and urine testing for hematologic and biochemical indices. Disease is as yet incurable but management and treatment strategies mainly focus on symptomatic relief and eradication of causative factors; however certain evidence is present to suggest that early diagnosis and treatment can reverse the calcification process leading to complete recovery of mental functions. Families with a known history of Fahr’s disease should be counseled prior to conception so that the birth of affected babies can be prevented. This review was written with the aim to remark on the current substantial evidence surrounding this disease.

Study DesignCross sectional and observational.PurposeTo evaluate the different aspects of lumbar disc degenerative disc disease and relate them with magnetic resonance image (MRI) findings and symptoms.Overview of LiteratureLumbar disc degenerative disease has now been proven as the most common cause of low back pain throughout the world. It may present as disc herniation, lumbar spinal stenosis, facet joint arthropathy or any combination. Presenting symptoms of lumbar disc degeneration are lower back pain and sciatica which may be aggravated by standing, walking, bending, straining and coughing.MethodsThis study was conducted from January 2012 to June 2012. Study was conducted on the diagnosed patients of lumbar disc degeneration. Diagnostic criteria were based upon abnormal findings in MRI. Patients with prior back surgery, spine fractures, sacroiliac arthritis, metabolic bone disease, spinal infection, rheumatoid arthritis, active malignancy, and pregnancy were excluded.ResultsDuring the targeted months, 163 patients of lumbar disc degeneration with mean age of 43.92±11.76 years, came into Neurosurgery department. Disc degeneration was most commonly present at the level of L4/L5 105 (64.4%).Commonest types of disc degeneration were disc herniation 109 (66.9%) and lumbar spinal stenosis 37 (22.7%). Spondylolisthesis was commonly present at L5/S1 10 (6.1%) and associated mostly with lumbar spinal stenosis 7 (18.9%).ConclusionsResults reported the frequent occurrence of lumbar disc degenerative disease in advance age. Research efforts should endeavor to reduce risk factors and improve the quality of life.

BackgroundSpinal tuberculosis presents in various pathological patterns. The clinical presentation and often the management depend on exact pathological findings. Objective of study was to evaluate the Pathology of spinal tuberculosis as depicted by MRI findings in 119 consecutive cases of spinal TB.MethodologyIt was a cross sectional and observational study conducted at Civil Hospital, Karachi from July 2010 to December 2012.Total numbers of participants were 119. Diagnosis was based on positive histopathology results along with the supportive evidence in MRI. A pre-structured questionnaire was constructed to record the data. Study was ethically approved by Institutional Review Board of Dow University of Health Sciences. Sample size was calculated by using Open-EPI software. All the data was entered and analyzed through SPSS 19.ResultThere were 119 patients who participated in this study out of which 52 were males and 67 were females. Most common level was Dorso-lumbar (33.6%) and 87.5% of them had spondylodiscitis while 90% had cord compression. All 6 (100%) patients who had their upper- dorsal region affected had gibbus formation while all those patients having lumbosacral region involved had thecal compression 4 (100%). Most common mode of treatment used in patients having Spinal TB at Lumbar region was conservative (86.2%).ConclusionMRI findings were mostly shadowed with features such as disc destruction and thecal or cord compression. MRI scan could be used for early detection of spinal TB which can reduce disability and deaths in patients. Major clinical findings in spinal TB were fever, Para paresis and back pain.

Introduction: Posterior fossa brain tumor is the most devastating forms of human illness, primarily because of the limited space within the posterior fossa, the potential involvement of vital brain stem nuclei, and the mass effect causes obstructive hydrocephalus. Posterior fossa tumors are more common in children than adults. The Objective of the Study: To find out the satisfactory surgical outcome of posterior fossa brain tumors in children at Civil hospital, Karachi. Materials and Methods and Duration of Study Setting: This prospective observational, case series study was conducted from February 2015 to February 2105 in the Department of neurosurgery, Dow University of Health Sciences, Civil Hospital, Karachi, Karachi. Postoperative patients with the diagnosis of posterior fossa tumor were enrolled in the study. Detailed history, physical examination, anthropometrics, and biochemical measurements were recorded. Magnetic resonance imaging was done to determine the satisfactory surgical outcome. Patients were followed up at the third postoperative month to determine the satisfactory surgical outcome. Results: Seventy-one patients fulfilling the inclusion criteria, the mean ± standard deviation age of the study population was 6.63 ± 3.181 years. 29 (40.8%) were <7 years of age and 42 (59.2%) were of age 7 years and above. 50 (70.4%) were males and 21 (29.6%) were females. 49 (69%) patients presented with vomiting. 34 (47.9%) presented with seizures. (40.8%) had papilledema. (25.4%) presented with hemiparesis. 8 (11.3%) had meningismus. On analysis of the frequency of outcome variables (80.3%) achieved the satisfactory surgical outcome. Conclusions: There has been no major study to determine satisfactory surgical outcome in postoperative patients with posterior fossa brain tumor in our population. The study was to provide local data in our population and compare it to the international data. This may help in proper patient management. Majority of the patients had satisfactory surgical outcome. The absence of papilledema, hemiparesis, and meningismus had more chances of satisfactory surgical outcome.