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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
Abstract:
The prevalence of pathological laughing and crying in multiple sclerosis (MS) is 10%. It has been speculated that the anatomical lesion responsible for the pathological laughing is located in the pontine base, prefrontal cortex, and cerebellum. We report an 18-year-old male patient presenting with pathological laughing and hypomania. In his neurological examination, he had a euphoric effect with ataxic walking and dysarthria speech. He had a bilateral conjugated gaze limitation, with a prominent bilateral horizontal nystagmus on left gaze, dysmetria, dysdiadokokinesia, and remarkable dysfunction in a heel-to-shin test on the left. The IgG index in cerebrospinal fluid was normal with an oligoclonal band was present. In cranial MRI, there was a lesion on central pons which was hypointense in T1 images with contrast enhancement and hyperintense in T2 and flair images. Also another lesion in right brachium pontis which did not contrast enhancement but was hyperintense on T2 and flair images was present. There was an elevation of myoinositol/creatine ratio and choline and a reduction of NAA in proton MR spectroscopy. MR spectroscopic evaluation of the patient demonstrated the demyelination process. There has been no report of patients in whom pathological laughter was the presenting symptom of clinically isolated brainstem syndrome.
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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