Case study
When I met her for the first time, she was in her early fifties. She had had multiple sclerosis for over 20 years. Her family now kept her at home, isolated from the wider world. Her behaviour would embarrass them. Why?
She suffered from pathological laughter and occasionally inappropriate crying; her husband and children could not deal with this in public. She was clearly very disabled when I met her in the clinic; she was unsteady on her feet and had slurred speech and dancing eyes from cerebellar problems. She had gross cognitive impairment. When I introduced myself to her, she burst into tears. Within two to three months of starting sertraline, a selective serotonin reuptake inhibitor (SSRI), her husband informed me that her laughing and crying episodes had improved by over 50%, and the family were now taking her out regularly. He was very grateful that I had been able to educate them about her symptoms and, more importantly, help her and them as a family deal with this problem.
Pseudobulbar Affect
Pathological or inappropriate laughing and crying are common in pwMS; it is an emotional expression exaggerated or incongruent with underlying mood. From my experience, it is much more commoner than studies suggest, and if you ask about the symptom, many pwMS suffer from a mild form of it.
In short inappropriate laughing and crying is due to frontal lobe or brainstem damage resulting from MS and, in my experience, is associated with cognitive and mood problems. It is essential to realise that inappropriate laughing and crying are symptoms of MS. These symptoms respond to tricyclic and SSRI antidepressants and a combination pill that includes dextromethorphan/quinidine (Nuedexta®).
The medical or neurological name for inappropriate laughing and crying is a ‘pseudobulbar affect’ and is diagnosed using standardised scales or questionnaires, which can be self-administered (Center for neurological study-lability scale). Some clinicians refer to these symptoms as emotional incontinence; I don’t like the term as it implies that the person is actually experiencing the emotions underpinning the laughter and/or crying, which is not always the case.
I would be interested to know how many of you have inappropriate laughing and crying and how disruptive these symptoms are to your life. Does your MS team ask about these symptoms, and have you been offered a screening questionnaire for this problem? If you have been diagnosed as having a pseudobulbar affect have you been treated for it, what treatments did you receive, and how have you responded to these treatments?
Inappropriate laughing and crying in pwMS are two neglected symptoms that often go undetected and untreated in many pwMS. This doesn’t have to be the case. They are also a sign of significant end-organ damage to the brain and are another reason to diagnose and treat MS early and effectively.
Background: Pathological laughing and crying (PLC) is common in multiple sclerosis (MS), defined as emotional expression that is exaggerated/incongruent with underlying mood. In other neurological disorders, PLC is associated with cognitive impairment (CI). Few studies have examined this relationship in MS.
Objective: To determine the association between PLC and CI in an MS population.
Methods: Retrospective chart review study of 153 MS subjects assessed in an outpatient clinic for CI. Data was collected on the minimal assessment of cognitive function in MS (MACFIMS), the Center for neurological study-lability scale (CNS-LS), a screening measure for PLC symptoms and the hospital anxiety and depression scale (HADS). Analyses of covariance compared performance on the MACFIMS between PLC (CNS-LS score ≥ 17, HADS-D ≤ 7) and non-PLC groups.
Results: MS subjects positive for PLC on the CNS-LS but without depression had lower scores on the controlled oral word association test, a measure of verbal fluency, and the California verbal learning test – 2 immediate recall score, a verbal memory measure.
Conclusions: This study demonstrates a connection between CI, specifically verbal fluency and verbal learning, and PLC in MS subjects. Further studies are warranted to explore the causative relationship between CI and PLC.
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General Disclaimer: Please note that the opinions expressed here are those of Professor Giovannoni and do not necessarily reflect the positions of Barts and The London School of Medicine and Dentistry nor Barts Health NHS Trust. The advice is intended as general and should not be interpreted as personal clinical advice. If you have problems, please tell your healthcare professional, who will be able to help you.
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