Zoe Hatzimarkou MSc
Clinical Neuropsychologist
ANAPLASI MEDICAL REHABILITATION CENTER
May 30th is World Multiple Sclerosis Awareness Day. Multiple Sclerosis (MS) is a chronic, autoimmune, inflammatory disease that affects the central nervous system (CNS). MS is the most common, non-traumatic, demyelinating disease of the CNS, causing significant impact on functions such as movement, sensation, cognition, and emotion.
The disease begins when the patient’s own immune system “attacks” the cells of the brain and spinal cord, that is, the neurons. Specifically, the autoimmune attack targets the myelin sheath, the lipid-rich substance surrounding the axons of myelinated nerve fibers. This insulating, protective coating of nerve fibers is vital for the normal functioning of the CNS, enhancing the speed of transmission between neurons and letting us perform a variety of activities, such as walking, talking, and mentally planning these actions.
Based on data from the World Health Organization, it is estimated that there are over 1.8 million patients with MS worldwide. In a study published in the journal Neurology in 2019, it is estimated that in 2017, the prevalence of adult patients in the United States was 362 patients per 100,000 people, i.e., approximately 900,000 patients. In addition, women are affected 3 times more often than men. In Greece, in a study carried out in 2017-2019, it was estimated that the total number of patients was approximately 20,000, of which 65% were women, with a higher frequency in the 45-49 year age group. On average, the age at diagnosis of MS is 32 years. Finally, it has been observed that the prevalence of MS increases with latitude, i.e., it is greater in areas further north of the equator.
The causes of the disease are not entirely clear. However, factors such as infection with the Epstein-Barr virus (EBV), limited exposure to sunlight, smoking, and low vitamin D levels, combined with the patient’s genetic background, play an important role in the onset of the disease.
MS is divided into four subtypes: the relapsing (or intermittent) form, the progressive relapsing (or isolated clinical syndrome), the secondary progressive form, and the primary progressive form. The relapsing form is the most common manifestation of the disease (affecting 85% of patients), with a key feature being periods of extreme relapses (flares) followed by periods of almost complete remission. In the progressive relapsing form, the patient experiences an episode of isolated neurological symptoms (such as visual disturbance, urination problems, or difficulty with coordination and balance), which, with timely treatment, may delay the relapse of MS. In the secondary progressive form, patients who have already been diagnosed with the intermittent form go on to have progressively worse symptoms and are unable to return to their previous functional state. Finally, in the primary progressive form (affecting 15% of patients), there are no periods of exacerbation and remission, but a slow and continuous worsening of symptoms, often manifesting as weakness and spasticity of the lower extremities.
Patients with MS may develop different symptom constellations, including motor dysfunction (weakness, spasticity, ataxia, dysarthria) and sensory impairment (blurred or double vision, numbness, tingling, burning), as well as severe fatigue, pain (neurogenic, musculoskeletal, and mixed type) and incontinence (urge incontinence ). Behavioral, emotional, and cognitive symptoms, which can appear before or after the time of diagnosis, also have a serious impact on the quality of life of patients.
The term “cognitive functions” is an umbrella term for functions that allow us to maintain our attention, learn and remember new information, plan, execute, and monitor our activities, solve problems, understand and use language, recognize objects, and process data. Disorders of cognitive functions have been reported in 45-70% of patients with MS, with no clear gender differences. It is estimated that 40-50% of patients have mild to moderate difficulties, while 10-20% are more severely affected.
Cognitive impairments are a consequence of the devastating effects of demyelination and damage to both white and gray matter. In particular, damage to gray matter (for example, in the thalamus) has been suggested to play a key role in the early impairment of cognitive functions. However, in addition to structural changes, metabolic and functional changes in the CNS are also associated with cognitive dysfunction. It seems that there is no direct correlation between cognitive and motor difficulties; therefore, cognitive difficulties can appear in the early stages of the disease, even in the isolated clinical syndrome, in patients with moderate and severe difficulty, but also in each subtype of the disease.
Patients with MS may develop different constellations of symptoms, including motor (weakness, spasticity, ataxia, dysarthria) and sensory (blurred or double vision, numbness, tingling, burning), as well as severe fatigue, pain (neurogenic, musculoskeletal, and mixed type), and incontinence (urinary urgency). Behavioral, emotional, and cognitive symptoms that may appear before or after the time of diagnosis also have a serious impact on the quality of life of patients.
The term “cognitive functions” is an umbrella term and represents those functions that allow us to maintain our attention, learn and remember new information, plan, execute, and monitor the execution of our activities, solve problems, understand and use language, recognize objects, and combine data. Cognitive function disorders have been reported in 45-70% of patients with MS, with no clear gender differences. It is estimated that 40-50% of patients have mild to moderate difficulties, while 10-20% experience more severe disorders.
Cognitive impairments are a consequence of the devastating effects of demyelination and damage to both the white and gray matter of the CNS. In particular, damage to gray matter (for example, in the thalamus) has been suggested to play a key role in the early impairment of cognitive functions. However, in addition to structural changes, metabolic and functional changes in the CNS are also associated with cognitive dysfunction. It seems that there is no direct correlation between cognitive and motor symptoms; therefore, cognitive symptoms can appear in the early stages of the disease – even in the isolated clinical syndrome – and in patients with moderate and severe symptoms in each subtype of the disease.
Like other symptoms in MS, cognitive symptoms vary, and there is no single phenotype or cognitive profile. Despite the presence of significant lesions in white and gray matter, a severe neurocognitive disorder (dementia), with amnesia, aphasia, and agnosia, is not characteristic of a patient with MS. The most frequent cognitive difficulties in MS are impairments in the speed of information processing and the ability to learn and recall new information. Memory storage usually remains intact, while working memory (the ability to hold information in the short term and simultaneously process it) and encoding new information (converting information into a form that can be stored in memory) are the main aspects of memory that are affected. Additionally, impairment of complex attention, that is, the ability to control, shift, and alternate our attention between two sets of information, is quite common in MS, in contrast to speech disorders, which occur less commonly.
In daily life, carrying out normal activities is prolonged, concentration becomes more susceptible to external interference, and switching attention between different tasks becomes more difficult. Furthermore, factors such as emotional difficulties (e.g., depressed mood) and increased fatigue may interfere with the smooth functioning of brain networks, leading to transient cognitive dysfunction. It is important to emphasize that there is are extremely complex interactions between cognitive dysfunction, fatigue and depression in MS. Depression reduces the speed of information processing and executive functions (working memory, complex attention, etc.), while increased fatigue (physical and mental), which in turn, worsens mood and leads to greater fatigue. The patient becomes trapped in an extremely unpleasant, vicious circle.
In addition to depression, patients with MS and their loved ones may experience a range of other behavioral and emotional reactions, such as irritability, mental rigidity, passivity, impatience, and apathy. More serious psychiatric manifestations, such as hallucinations and delusions, are less common. Irritability, apathy, adjustment disorder, and reduced empathy are more frequently observed in patients with MS than in the general population.
Neuropsychological rehabilitation aims to limit cognitive deficits, reduce the devastating effects of cognitive deficits, and increase self-awareness about the impact of cognitive deficits on daily life. Specifically, patients are informed and educated about the disease and its effects on cognitive functioning, are trained in the use of compensatory strategies, are empowered in areas such as social skills, and are supported in adapting to new challenges.
In ANAPLASI, the type and severity of cognitive difficulties are determined through an individual neuropsychological assessment. For valid and reliable neuropsychological assessment, specially verified neuropsychological tools are used (MMSE, MOCA, ACE-R, WAIS-R, BOSTON DIAGNOSTIC APHASIA EXAMINATION, FAB, TMT-A, TMT-B, DELIRIUM OBSERATION SCREENING SCALE), always taking into consideration the patient’s clinical picture.
A personalized neurocognitive program is then developed, based on the specific needs of each patient. Neurocognitive therapy is implemented through specific therapeutic exercises, either using the traditional “paper and pencil” method or using electronic applications on a computer or tablet.
As part of the rehabilitation program in ANAPLASI, combined therapy sessions are held with staff from the Departments of Occupational Therapy, Physiotherapy, Speech Therapy, and Clinical Psychology, tailored to the needs of patients with MS, thus integrating the rehabilitation of patients under the auspices of the Trans-Disciplinary Rehabilitation Team.
Although the impact of MS on patients’ abilities is significant, immediate, specialized, holistic care can improve their quality of life.
At ANAPLASI, we believe that prevention and regular monitoring of cognitive functions are of crucial importance in improving the patient’s quality of life. In this context:
At ANAPLASI
“We do the maximum possible, not the minimum necessary.”
“We care for our patients as we would care for our own family.”
Bibliography
- Bakirtzis, C., Grigoriadou, E., Boziki, MK, Kesidou, E., Siafis, S., Moysiadis, T., & Grigoriadis, N. (2020). The Administrative Prevalence of Multiple Sclerosis in Greece on the Basis of a Nationwide Prescription Database. Frontiers in Neurology, 11. doi:10.3389/fneur.2020.01012
- Foley, FW, & Portnoy, JG (2018). Neuropsychology in the Integrated MS Care Setting. Archives of Clinical Neuropsychology, 33( 3 ), 330–338. doi:10.1093/arclin/acy003
- Hämäläinen, P., & Rosti-Otajärvi, E. (2016). Cognitive impairment in MS: rehabilitation approaches. Acta Neurologica Scandinavica, 134, 8–13. doi:10.1111/ane.12650
- Meca-Lallana, V., Gascón-Giménez, F., Ginestal-López, RC, Higueras, Y., Téllez-Lara, N., Carreres-Polo, J., & Pérez-Miralles, F. (2021). Cognitive impairment in multiple sclerosis: diagnosis and monitoring. Neurological Sciences, 42 ( 12 ), 5183-5193. doi:10.1007/s10072-021-05165-7
- Oh, J., Vidal-Jordana, A., & Montalban, X. (2018). Multiple sclerosis. Current Opinion in Neurology, 31 ( 6 ), 752-759. doi:10.1097/wco.0000000000000622.
- Wallin, M., Culpepper, W., Campbell, J., Nelson, L., Langer-Gould, A., Ann Marrie, R., Cutter, G., Kaye, W., Wagner, L., Tremlett, H., Buka, S., Dilokthornsakul, P., Topol, B., Chen, L., & LaRocca, N. (2019). The prevalence of MS in the United States. Neurology, 92, 1-18. https://doi.org/10.1212/WNL.0000000000007035 .
- Walton, C., King, R., Rechtman, L., Kaye, W., Leray, E., Marrie, RA, & Baneke, P. (2020). Rising prevalence of multiple sclerosis worldwide: Insights from the Atlas of MS, third edition. Multiple Sclerosis Journal, 26( 14 ), 1816-1821. doi:10.1177/1352458520970841.
