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Leucoencephalopathy with progressive cerebral atrophy
  1. Nina Xie1,2,
  2. Qiying Sun1,2,
  3. Mengchuan Luo1,2,
  4. Yafang Zhou1,2
  1. 1 Department of Geriatric Neurology, Central South University, Changsha, China
  2. 2 Department of Geriatric Neurology, National Clinical Research Center for Geriatric Disorders, Changsha, China
  1. Correspondence to Dr Yafang Zhou, Department of Geriatric Neurology, Central South University, Changsha 410078, China; zyf_1981{at}csu.edu.cn

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Case description

A 38-year-old man presented to our centre with subacute cognitive decline and right limb weakness. His memory had been normal until 5 months before, initially having difficulty recalling friends’ names. Over the next 3 months, he became sleepy and lacked interest in social activities. Gradually his right limbs became inflexible, interfering with tooth brushing and walking.

An MR scan of brain at another hospital showed asymmetric confluent white matter hyperintensities, mild temporal lobe atrophy and normal intracranial arteries (figure 1). He had been treated elsewhere with intravenous immunoglobulins and corticosteroids for presumed antibody-negative autoimmune encephalitis, but his symptoms had continued to worsen despite regular immunotherapy, such that he could no longer talk or walk.

Figure 1

MR scan of brain series (A) FLAIR and (B) enhanced MR scan of brain 3 months after onset showed confluent white matter hyperintensities involving left fronto-temporo-insular lobe, basal ganglia and thalamus, with mild temporal lobe atrophy and faint patchy enhancement. Repeat (C) FLAIR and (D) enhanced MR scan of brain 5 months after onset showed prominent left temporal lobe atrophy. FLAIR, fluid-attenuated inversion recovery.

He had been previously well and was immunocompetent. There was no history of substance abuse or toxin exposure and no relevant family history.

On examination, he was alert with mixed aphasia. Cognitive assessment identified impaired temporal and spatial orientation and problems with long-term and short-term memory. He could not complete calculation or the Mini-Mental State Examination. There was no meningism. He had a right facial palsy. The strength in his right arm and leg was rated as 3/5 proximally and 0/5 distally; left-sided limb strength was normal. Deep tendon reflexes were 4+ on the right, with inducible ankle clonus, and 3+ on the left. The right plantar response was extensor. Sensation testing was limited by cognitive deficits. His sensation to painful stimuli …

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Footnotes

  • Contributors YZ conceived the idea for the article. All authors contributed to data collection and analysis. YZ and NX drafted the manuscript. All authors gave final approval of the version to be published.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Provenance and peer review. Not commissioned. Externally peer reviewed by Fiona McKevitt, Sheffield, UK.

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