The adult form of myotonic dystrophy type 1 is a neuromuscular disorder with multisystem involvement, including the central nervous system (CNS). The presenting clinical features of this condition include distal muscle weakness, myotonia, intellectual decline, cataract, frontal baldness and testicular atrophy. Magnetic resonance (MR) imaging shows characteristic white matter changes in the CNS. The clinical presentation, characteristic white matter changes in the brain on MR imaging and electromyographic findings aid in the diagnosis of this disorder.
Adult ; Atrophy ; complications ; Brain ; pathology ; Cataract ; complications ; Central Nervous System ; pathology ; Electromyography ; methods ; Hearing Disorders ; complications ; Humans ; Lactic Acid ; blood ; Magnetic Resonance Imaging ; methods ; Male ; Muscle Weakness ; complications ; Myotonic Dystrophy ; diagnosis ; pathology ; Neuromuscular Diseases ; diagnosis ; pathology

Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Magnetic resonance (MR) imaging is the modality of choice for determining such changes in white matter. SSPE typically demonstrates bilateral but asymmetric periventricular and subcortical white matter involvement. We herein report a rare case of unilateral white matter involvement in a 13-year-old boy with SSPE that closely simulated Rasmussen's encephalitis. To the best of our knowledge, this is the first report of an atypical presentation on MR imaging in which SSPE was a rare cause of unilateral brain parenchymal involvement in a patient with intractable seizures.
Adolescent ; Brain ; pathology ; Diagnosis, Differential ; Encephalitis ; diagnosis ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Subacute Sclerosing Panencephalitis ; diagnosis ; pathology