Remarkable behavioural signs and progressive non-fluent aphasia in a patient with adult-onset leucoencephalopathy with axonal spheroids and pigmented glia

Michitaka Funayama, Masako Sugihara, Taketo Takata, Masaru Mimura, Takeshi Ikeuchi

Research output: Contribution to journalArticle

Abstract

Adult-onset leucoencephalopathy with axonal spheroids and pigmented glia (ALSP), also known as hereditary diffuse leucoencephalopathy with spheroids (HDLS), is a progressive neurocognitive disorder that predominantly affects the cerebral white matter, mainly the frontal subcortical areas and the corpus callosum. Patients with ALSP are clinically characterized by a gradual onset of cognitive and behavioural dysfunction and personality changes, followed by motor impairments such as gait disturbance and bradykinesia. Given the disease-related degenerative changes of the frontal white matter, it is no wonder that patients with ALSP present with behavioural symptoms and non-fluent aphasia, which are found in patients with frontotemporal lobar degeneration. However, behavioural symptoms and non-fluent aphasia in a patient with ALSP have rarely reported in detail. Here, we describe a patient with ALSP who initially presented with remarkable behavioural signs and non-fluent primary progressive aphasia, which resembled symptoms of frontotemporal lobar degeneration. The present case suggests that ALSP should be included in the differential diagnosis for frontotemporal lobar degeneration.

Original languageEnglish
JournalPsychogeriatrics
DOIs
Publication statusAccepted/In press - 2018 Jan 1

Fingerprint

Leukoencephalopathies
Aphasia
Neuroglia
Frontotemporal Lobar Degeneration
Behavioral Symptoms
Primary Progressive Nonfluent Aphasia
Hypokinesia
Corpus Callosum
Gait
Personality
Differential Diagnosis

Keywords

  • axonal spheroids and pigmented glia (ALSP)
  • behavioural signs
  • frontotemporal lobar degeneration (FTLD)
  • hereditary diffuse leucoencephalopathy with spheroids (HDLS)
  • progressive non-fluent aphasia

ASJC Scopus subject areas

  • Gerontology
  • Geriatrics and Gerontology
  • Psychiatry and Mental health

Cite this

Remarkable behavioural signs and progressive non-fluent aphasia in a patient with adult-onset leucoencephalopathy with axonal spheroids and pigmented glia. / Funayama, Michitaka; Sugihara, Masako; Takata, Taketo; Mimura, Masaru; Ikeuchi, Takeshi.

In: Psychogeriatrics, 01.01.2018.

Research output: Contribution to journalArticle

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