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CLINICAL MANIFESTATIONS OF MULTIPLE SCLEROSIS

The triads and pentads described by classics of MS research are currently not sufficient for the clinical characteristics of MS patients. In the clinical picture of this disease, 2 groups of symptoms can be identified: classic and rare. The first group includes the most common symptoms, which are a direct manifestation of damage to the conducting systems of the brain. This also includes the symptom complexes characteristic of MS, reflecting the clinical features of the multifocal demyelinating process. The second group includes rare clinical manifestations of the disease, which can cause difficulties in the differential diagnosis.

1. Early diagnosis.

The diagnosis of MS is still based on the clinical characteristics of the manifestation of the pathological process in the central nervous system. Various neurophysiological, tomographic and immunological methods can only confirm the clinical diagnosis of MS. These methods are of particular value in atypical cases when neurologists cannot make a final conclusion about the presence of MS in a patient. (Gusev E.I., Demina T.L., 2000).

Table 4.

Initial manifestations of multiple sclerosis

(according to Zhulev N.M., Totolyan N.A., 1998)

For the clinical justification of the diagnosis of MS, it is necessary to identify signs of a wave-like chronic process involving several conductive CNS systems. Currently, two main types of criteria are used: clinical and paraclinical, obtained using neurophysiological methods, tomographic studies, cerebrospinal fluid and peripheral blood tests.

The following Poser's diagnostic criteria have been widely used by practical neurologists. They include data from laboratory methods (cerebrospinal fluid examination), caused by brain potentials and MRI.

Table 5.

Diagnosis Criteria for Multiple Sclerosis



(Poser CM et al., 1983)

Currently, the scale compiled by J. Kurtzke (1983) (FS scale - Functional Systems) is used for the clinical evaluation of conductive systems in the world. The scale contains 7 groups of symptoms, which are characterized by different degrees of disturbances (0 points - no changes).

Table 6.

Damage scale of functional systems according to J. Kurtzke

(1983)

Based on the sections of this scale, it is convenient to briefly list the typical clinical manifestations of the disease that can be established during a medical history or neurological examination, and evaluate the effectiveness of the treatment.

Table 7 shows the frequency of typical clinical manifestations of MS (including history and neurological examination) according to sections of the FS scale according to Kurtzke JF (group 1) and Guseva E.I., Demina T.L., Boyko A.N. (2 group).

Table 7.

Frequency of typical clinical manifestations of MS

.

x - 408 patients residing in the USA (Kurtzke JF, 1961)

Well - 520 patients examined at the Department of Neurology and Neurosurgery of the Moscow State Medical University (Gusev E.I., Demina T.L., Boyko A.N., 1997).

The differences in the frequency of a number of pathological manifestations of MS are most likely associated with different approaches to assessing the norm and pathology and / or a specific clinical peculiarity of MS manifestations, possibly due to ethnic characteristics, differences in treatment methods and socio-economic living conditions.

Symptoms of a pyramidal lesion

From the rapid “demyelinating” symptom of Babinsky without loss of strength in the legs (characteristic clinical and functional dissociation) and loss of superficial abdominal and plantar reflexes to spastic lower paraplegia with upper central paraparesis and pseudobulbar disorders, sometimes with symptoms of violent laughter and crying.

These disorders are most often observed in MS, apparently due to the higher probability of focal lesion of the long axons of the pyramidal cells at different levels. Depending on the localization of the focus, hemiparesis and paraparesis, less often monoparesis, can be observed. The upper limbs, as a rule, suffer less often than the lower limbs or are involved in the process at later stages. According to the Department of Neurology and Neurosurgery, Russian State Medical University, Moscow (Gusev E.I., Demina T.L., Boyko A.N., 1997), only 22% of patients showed a significant decrease in strength in their hands. The change in the degree of paresis during the day is quite characteristic: some patients note an increase in weakness in the evening, others in the morning.

Reduced strength in the defeat of the pyramidal system in patients with MS is always accompanied by pathological pyramidal symptoms, an increase in tendon and periosteal reflexes and a decrease in skin abdominal reflexes. The last symptom is a subtle early manifestation of the interest of the pyramidal pathway, especially with an asymmetric decrease, but, of course, is not a sign specific to MS. Moreover, when evaluating this symptom, false-positive phenomena are highly likely due to obesity, postoperative scars, and stretching of the abdominal muscles after childbirth. In patients with MS, as a rule, not only an increase in the amplitude of tendon, periosteal reflexes and their asymmetry is detected, but also a sharp expansion of the zones of their induction, the presence of clones, cross reflexes, and less often protective ones. Typical dissociation of the amplitude of tendon reflexes vertically, i.e. a more pronounced increase in reflexes from the lower limb than from the upper on the same side.

Central paralysis in MS is accompanied by various changes in muscle tone - spasticity, hypotension, or dystonia. A big problem for patients is an increase in spastic tone. This symptom is observed, as a rule, in patients with lower paraparesis. From a clinical point of view, spasticity can be characterized as an increase in muscle tone during passive movements (in the classical version, more at the beginning and at the end of movement), restriction of active movements due to stiffness, involuntary reflex muscle spasms as a hyperreaction to external stimuli and involuntary leg extension when walking. With this definition of spasticity, up to 80% of patients had this symptom in varying degrees of severity (Gusev E.I., Demina T.L., Boyko A.N., 1997). Often, increased tone prevails over the severity of paresis and is the main cause of difficulty walking.

With simultaneous damage to the conductors of the pyramidal and extrapyramidal tract, muscular dystonia can be observed. A peculiar clinical manifestation of a multi-focal demyelinating process is a combination of central paralysis with hyperreaction and clonuses, pathological pyramidal signs and at the same time pronounced hypotension due to damage to the conductors of deep sensitivity and / or cerebellum. This combination is one example of the syndrome of "clinical dissociation" or "clinical cleavage" typical of multiple sclerosis (Markov DA, Leonovich A.L., 1976). With prolonged severe course of the disease, the development of flexion contractures and severe muscle hypotension is typical. Moreover, against the background of spastic tone, tendon reflexes decrease. In such patients, manifestations of demyelinating or secondary polyneuropathy can also join.

Impaired coordination (cerebellar involvement)

From episodic dizziness, unevenness of handwriting, one-sided intentional tremor at the end of the finger-nasal test and subtle shakiness for walking when walking to rough static and dynamic ataxia with pronounced hypermetry, making targeted movements difficult or impossible. Speech chanting and coarse axial tremor sharply limit speech communication with others and independent eating. The Sharko triad (nystagmus, chanting, intentional trembling), which impressed the first researchers of MS, has now disappeared into the diverse clinical picture of the disease, and the debut and progression of the cerebellar syndrome always require a differential diagnosis with primary cerebral atrophies.

Cranial nerve disorders (except for the second pair)

According to the history and neurological examination, symptoms of cranial nerve damage are observed in 58% of MS patients (Gusev E.I., Demina T.L., Boyko A.N., 1997). The most often affected III, V, VI and VII pairs of cranial nerves. Foci of demyelination can form in the extra cerebral and intracerebral parts of the nerves, so the clinical manifestations of lesions can be of central and peripheral origin.
When the facial nerve is affected, the focus of demyelination is more often localized in the “inner” knee of the nerve, therefore, classical symptoms of peripheral facial paralysis are noted. In some patients with lesions of the trigeminal nerve, unilateral sensitivity disorders in the face area can be detected. Rare symptoms are trigeminal neuralgia, hemifascial pain, and fascial myoclonia. Foci in the supranuclear regions of the corticonuclear tract can be the cause of the pseudobulbar symptom complex. Quite often, especially in the early stages of the disease, patients complain of dizziness. The foci of demyelination in the lateral loop can lead to hearing loss, almost always bilateral, but this clinical symptom is quite rare. As a rule, subclinical damage to the conductors of the auditory tract can be detected using audiometry and bone conduction in the Schwabach experiment.

The most common symptom of a brain stem lesion is oculomotor disturbance, which may be accompanied by complaints of double vision. MS is characterized by internuclear ophthalmoplegia syndrome associated with foci of demyelination in the posterior longitudinal beam system. Typical manifestations of MS are discoordinated movements of the eyeballs, their vertical or horizontal spacing (squint), lack of eyeballs when viewed from the side and, especially, the inside when the patients are not in a relatively serious condition. Mild ptosis is sometimes observed. Classical signs of damage to the oculomotor and abducent nerves from one side, paresis of the gaze, are somewhat less common. Rarely there are changes in pupillary reactions associated with the involvement of the optic, and not the oculomotor nerve. The transient nature of the oculomotor symptoms and the fluctuations in their severity during the day are of diagnostic value. In the case of unusual transient oculomotor symptoms in the absence of other signs of multifocal brain damage, myasthenia gravis and atypical aneurysms of the vessels of the base of the brain should be excluded. Rare causes of damage to the posterior longitudinal bundle can be intoxication, systemic lupus erythematosus, metastases to the brain stem and other pathological processes in the central nervous system that are not associated with demyelination.

Many authors include nystagmus as a group of symptoms of cranial nerve damage. There is no doubt that vertical nystagmus, which in patients with MS can be detected when the patient is not in a serious condition, is a typical manifestation of damage to the upper parts of the brain stem. The causes and types of horizontal nystagmus in MS are diverse. For a clinical assessment of the significance of the revealed nystagmus, it is advisable to first exclude all other causes not related to damage to the brain stem (traumatic, toxic or physiological origin).

Sensitivity disorders (damage to the spinal-thalamic tract, lemnisci, thalamus, cords and roots of nerves, muscle and bone-ligamentous apparatus)

At an early (10-15 years) and young age (up to 32-35 years old) disease most often (at 31.9-52.2%) begins with a violation of sensitivity, although "late MS" (after 40 years) also begins with the feeling of "cotton" legs. Sensitive disorders mean a wide range of manifestations. The patient’s complaints are many-sided: from numbness of the cheek, lower leg, toes, or an objective decrease in the vibrational sensation from the ankles to the costal arches detected with a tuning fork or pallesthesiometer to paroxysmal trigeminal neuralgia, proprioceptive ataxia, chronic dysesthesia of the extremities, night cramps and musculoskeletal syndromes pain. Lermitt's sensitive phenomenon is known, which consists in the sensation of an electric shock in the arms or back during sharp turns or forced bending of the head.

Disorders of the pelvic organs

Severe dysfunction of the pelvic organs is such a frequent syndrome in MS that Scheinberg proposed his “triad” of symptoms sufficient to diagnose MS in 30 year old men (urinary incontinence, constipation and impotence). These manifestations of the disease can be a more important cause of disability than paresis and coordination disorders and significantly affect the mental state of patients. The earliest manifestations of urination disorders are of the central type: peremptory urgency, rapid, delayed and, at later stages, urinary incontinence. Pelvic dysfunction in MS is characterized by inconsistency of the clinical picture and fluctuations in the severity of disturbances throughout the day.

Optic nerve damage

From a slight sensation of “fog” in front of the eyes that passes on its own for 7-10 days to complete amaurosis with subsequent determination during neurophalmological examination expressed by cattle, temporal decoloration of the fundus, and one- or two-sided atrophy of the optic nerve.

Usually visual acuity remains at the level of 0.1-0.4. A progressive decrease in vision in both eyes with a gray fundus makes us differentiate MS with a hereditary Leber disease.

Intelligence changes

In connection with the improvement of the diagnosis and treatment of MS, the percentage of lung patients has recently increased, for which moderate intellectual-mnestic and psychoemotional disorders are significant.

Neuropsychological examination reveals neurosis-like, asthenic and obsessive-phobic syndromes, personality anxiety, depression and euphoric dementia. The development of these disorders is associated both with premorbid personality traits, social stresses, and morphological reasons. The severity of euphoria, for example, depends on the degree of internal hydrocephalus and the number of plaques in the basal ganglia.

The ability to form concepts, develop abstract thinking and verbal memory depends on the total area of ​​drain foci and zones of hyperintensity of the periventricular space.

It is important to adequately respond to these features of patients with MS and not to prescribe unnecessary psychotropic medications. It should also be borne in mind that the risk of suicide among MS is 7.5 times higher than the population as a whole.

An interesting syndrome of MS is fatigue, not associated with muscle weakness and depressive moods. This constant fatigue concerns not only physical, but also intellectual work, accompanied by dysphoria and emotional lability. It is precisely because of such "unmotivated" fatigue in the early years of the disease that 80% of patients cannot perform their usual work and family responsibilities, which is often the cause of depression, and not vice versa. Special studies using positron emission tomography using deoxyglucose showed the presence of lesion sites in the hippocampus of such patients and a 10-fold increase in glucose hypometabolism zones compared with foci on MRI. The total decrease in the energy of the ancient cerebral cortex preceded the subsequent focal lesion of white matter. It is important to know that many drugs used to treat MS increase this fatigue, cause depression and impaired attention.

Diagnosis criteria (minimized option)

1. Young age of onset of the disease

2. Multi-focal damage to the central nervous system (mainly a combination of pyramidal and cerebellar disorders with pelvic disorders)

3. Progressive-remitting course

4. Identification of foci of demyelination on MRI of the brain

5. Immunologically detected sensitization to the myelin basic protein.

This pentad is sufficient to diagnose the disease, but not the diagnosis of the patient. The following is the diagnosis of focal, extrafocal and extraneural disorders, the establishment of a cause-effect relationship, which plays a decisive role in the appointment and monitoring of therapeutic measures.

Table 8.

Pathogenetic factors of multiple sclerosis and features of their laboratory diagnosis



With the introduction of PMRS, it became possible to predict the stage of the disease and, together with MRI, create a functional-morphological image of MS.

Table 9.

Functional morphological diagnosis of MS stages



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