Primary renal lymphoma: a comprehensive review of the pathophysiology, clinical presentation, imaging features, management and prognosis.

Primary renal lymphoma (PRL) is defined as a non-Hodgkin's lymphoma restricted to kidneys with the absence of extensive nodal disease. It is an exceedingly rare clinicopathological entity, accounting for 0.7% of extranodal lymphomas. Published medical literature regarding the natural history and clinical outcomes of PRL remains limited. We describe a case of a young patient who presented with left shoulder pain, continuous fever, and unexplained weight loss as atypical initial manifestations of bilateral PRL, confirmed with the standard set of investigations. Furthermore, this article reviews the literature and discusses various aspects of PRL, including pathophysiology, presentation patterns, imaging and pathological characteristics, management, and prognosis. This paper serves to provide an update and aims to enhance the understanding of PRL. Timely diagnosis and treatment are imperative to achieve improved outcomes. Clinicians should maintain a high index of suspicion in order to prevent morbidity and mortality associated with this serious disease.

Van der Knaap Disease.

Van der Knaap disease or megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare, inherited, autosomal recessive disorder. It is characterised by macrocephaly and slowly progressive ataxia, spasticity, and cognitive decline. The usual age of onset is described from birth to infancy. MLC predominantly occurs in some ethnicities where consanguinity is common. This disease is caused by mutations in the gene, which encodes a novel protein, MLC1. The characteristic MRI findings include leukodystrophy and subcortical cysts that yield diagnostic clue in most of the cases. The diagnosis can be established prenatally and genetic counseling is usually offered for future pregnancies. Herein, we chronicle a case of Van der Knaap disease from Pakistan with the classical MRI features.

Managing Postural Hypotension in Diabetic Autonomic Dysfunction When Adrenergic Drugs are Contraindicated: Case Report and Review of Literature.

Postural hypotension, as a manifestation of autonomic neuropathy is a very sinister long-term debilitating complication of diabetes, is usually irreversible and tough to manage with medications. The treatment of this condition following the standard treatment protocols can be contraindicated in the patients with underlying heart conditions. We report the case of a patient at our hospital who presented with full-blown symptomatic dysautonomia secondary to long-standing diabetes, with bedside testing positive for autonomic dysfunction. Treating this patient with the standard protocol of adrenergic agonist could have worsened his underlying coronary artery disease. So, we moved a step aside to go out of the box and we have a trial of the ß1-selective beta-blocker, with astonishing results and significant improvement in the quality of life and symptoms of postural hypotension. We report here the use of alternative treatment option in managing a patient with severe postural hypotension secondary to diabetes-related autonomic neuropathy when adrenergic drugs are contraindicated.