Pathological laughing as a manifestation in a clinically isolated brainstem syndrome: a case report

Belgin Kocer1, Yusuf Oner, Hale Batur

  • 1Department of Neurology, Gazi University School of Medicine, Cankaya-Ankara, Turkey. ebkocer@yahoo.com

Insights

Pathological laughter, a symptom in 10% of multiple sclerosis (MS) patients, can be an early indicator of brainstem lesions. This case highlights a young male with pathological laughter as the initial sign of clinically isolated brainstem syndrome.

Area of Science:

  • Neurology
  • Neuroimaging
  • Neuroimmunology

Background:

  • Pathological laughing and crying (PLC) affects up to 10% of multiple sclerosis (MS) patients.
  • Lesions in the pontine base, prefrontal cortex, and cerebellum are hypothesized to cause pathological laughter.
  • Clinically isolated brainstem syndrome is a neurological condition characterized by sudden onset of symptoms indicating a lesion in the brainstem.

Observation:

  • An 18-year-old male presented with pathological laughter and hypomania.
  • Neurological examination revealed ataxia, dysarthria, gaze limitation, nystagmus, dysmetria, dysdiadokokinesia, and impaired heel-to-shin testing.
  • Cerebrospinal fluid showed a normal IgG index with an oligoclonal band.
  • Cranial MRI revealed lesions in the central pons and right brachium pontis, consistent with demyelination.

Findings:

  • Proton MR spectroscopy indicated elevated myoinositol/creatine and choline ratios, with reduced NAA, confirming a demyelination process.
  • The patient's presentation of pathological laughter as the sole presenting symptom of a clinically isolated brainstem syndrome is unprecedented in reported cases.
  • The MRI findings correlated with the neurological deficits observed, particularly those related to cerebellar and brainstem pathways.

Implications:

  • This case underscores the importance of considering demyelinating lesions, even in the brainstem, when evaluating new-onset pathological laughter.
  • Early diagnosis and intervention for brainstem lesions can potentially mitigate further neurological damage and disability in MS patients.
  • Further research into the neuroanatomical correlates of pathological laughter in MS may refine diagnostic criteria and treatment strategies.

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