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We report a 32-year-old male who was admitted with speech disturbance. The patient presented to us with confusion, headache, and urinary incontinence. On examination, patient had no deficits except for aphasia. We proceeded with investigations. Magnetic resonance imaging (MRI) brain revealed extensive white matter ischemia and acute infarcts in both centrum semiovale regions. Fluid-attenuated inversion recovery (FLAIR) images showed anterior temporal lobe and external capsule involvement bilaterally [Figures 1 and 2]. Cerebrospinal fluid analysis done to rule out encephalitis was normal. After the investigations, we reviewed the history and found that the patient had recurrent episodes of migraine for several years, and his mother and maternal uncle had repeated episodes of the stroke leading to dementia. We suspected the inherited cause for stroke. We tested the blood for NOTCH 3 gene mutation. It was sequenced from exons 2, 3, 4, 5, and 6 and patient was found to be heterozygous for p.C144s mutation confirming the diagnosis of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leucoencephalopathy (CADASIL).

journal of neurosciences in rural practice

Anterior temporal lobe involvement: Useful magnetic resonance imaging sign to diagnose Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy