The combination of pyramidal and extrapyramidal syndromes

Some diseases of the central nervous system are manifested by a combination of pyramidal and extrapyramidal syndromes. These leading clinical syndromes may be accompanied by other manifestations (dementia, ataxia, apraxia, and others), but often this combination of syndromes is the main clinical nucleus of the disease.

Main reasons:

1. Atrophic processes in the brain
2. Progressive supranuclear palsy
3. Cortico-basal degeneration
4. Multiple systemic atrophy (stria-nigral degeneration, Shaya-Dryger syndrome, OPCA)
5. Craniocerebral trauma
6. Complex parkinsonism - BAS - dementia
7. Creutzfeldt-Jakob disease
8. Vascular Parkinsonism
9. Binswanger disease
10. Dopa-responsive dystonia (Segawa's disease)
11. Encephalitis (including HIV infection)
12. Disease of accumulation
13. Metabolic encephalopathies
14. Houteon Huntington
15. Volumetric education in the brain

Atrophic processes in the brain

In addition to classical Alzheimer's disease or Pick's disease (in which pyramidal and extrapyramidal symptoms may appear), asymmetric cortical degeneration syndromes sometimes occur in clinical practice, the nosological independence and nature of which is very difficult to determine. Depending on the topography of cortical involvement, characteristic neurological and cognitive impairment profiles are observed. They manifest either slowly progressive aphasia, or visual-motor disorders; there may also be a syndrome of frontal or frontotemporal dysfunction, or bilateral temporal (bitemporal) disorders. They usually accompany most atypical cortical dementias. Whether each of these syndromes is a separate degenerative disease or reflects only one of the variants of a single disease remains unclear. An example is motor neuron disease, which can sometimes be accompanied by several types of asymmetric cortical degeneration, including frontal lobe syndrome and progressive aphasia (the "disinhibition-dementia-parkinsonism-amyotrophy" syndrome).

Nonspecific lobar atrophies and Pick's disease have many common manifestations. Each of them can cause focal degeneration of the frontal and (or) temporal lobes and each of them can lead to "primary progressive aphasia" or to frontal-lobar dementia. On this basis, some authors suggest that the primary progressive aphasia and frontal dementia are different manifestations of a single "Peak-lobar atrophy," which is also referred to as "frontotemporal dementia" or "fronto-temporal lobar degeneration." But the primary progressive aphasia occurs in corticobasal degeneration.

Patients with progressive visual disorders (defect of the lower visual fields, reflecting cortical dysfunction over fissura calcarina) with Alzheimer's disease, which have so far been referred to manifestations of various diseases, are described. In the literature, one can find a reference to cases in which the histopathological picture in the brain blocked corticobasal degeneration, Pick's disease and Alzheimer's disease. You can also find descriptions of Alzheimer's disease with progressive spasticity (primary lateral sclerosis) or cases of primary lateral sclerosis with frontal neuropsychological disorders. Some descriptions are devoted to observations in which the histopathological pattern included manifestations of corticobasal degeneration and Pick's disease; Peak diseases and diseases of diffuse Levi bodies; Alzheimer's disease, parkinsonism and ALS; corticobasal degeneration, progressive supranuclear palsy and multisystem atrophy; Parkinson's disease and motor neuron disease.

All these cases of MRI are asymmetric with respect to focal cortical atrophy (in addition to ventricular expansion in some cases).

Further research, including molecular genetic studies, sheds light on the nature of these rare atrophic-degenerative mixed syndromes.

Progressive supranuclear palsy

The disease usually begins on the 5th-6th decade of life, has a progressive course and is not amenable to therapy. Clinical manifestations are characterized mainly by a triad of syndromes:

1. supranuclear surveillance disorders
2. pseudobulbar syndrome (mainly dysarthria)
3. axial rigidity and dystonia of extensor muscles (mainly, neck muscles).

The most characteristic signs are disruption of the gaze when looking down, postural instability with dysbasia and unexplained falls, "straight" posture (rigid neck in the position of extension), mild hypokinesia. Progressive supranuclear palsy only resembles parkinsonism in connection with the presence of hypokinesia, postural instability and dysarthria; it is hardly possible to talk about true parkinsonism in this disease. In some cases, non-structured pyramidal signs are possible. Criteria for diagnosis and criteria for excluding progressive supranuclear palsy have been developed.

Cortico-basal degeneration

This is a sporadic, degenerative, slowly progressing disease of mature age, manifested by an asymmetric akinetic-rigid syndrome accompanied by others ("parkinsonism plus") involuntary movements (myoclonus, dystonia, tremor) and lateralized cortical dysfunction (apraxia of the limb, the syndrome of someone else's hand, sensory disturbances in the form of asteroognosis, disorders of discriminatory feelings, feelings of localization). Cognitive defect develops at later stages of the disease. When the dominant hemisphere is affected, aphasia may occur, which is usually not very roughly expressed. Pyramid signs also occur frequently, but are usually moderately expressed, manifesting themselves only in hyperreflexia of one degree or another.

Progressive slowness of movements, a masklike face, rigidity of muscles, flexor posture, dysbasia postural disorders and falls, awkwardness in one of the extremities (most often in the hand), cortical myoclonus are characteristic. Frontal signs are possible in the form of a grasping reflex and paratonism. Macroscopically with corticobasal degeneration, asymmetric atrophy is observed in the frontal and parietal cortex, especially in the region surrounding the Roland and Sylvian furrows. No less characteristic is the depigmentation of the black substance. In the far-advanced stages of the disease, asymmetric frontal parietal atrophy is confirmed by CT or MRI data. In the initial stages, the disease is easily confused with manifestations of Parkinson's disease. For correct diagnosis, great importance is attached to the identification of asymmetric apraxic disorders. Recall that another name for this disease is progressive apraxic rigidity. Dosage-containing drugs are usually not effective.

Multiple system atrophies

Clinically, all forms of multiple systemic atrophy (stria-nigral degeneration, Shaya-Dryger syndrome, OPCA) are manifested by extrapyramidal and pyramidal signs, as well as symptoms of progressive autonomic failure. Depending on the features of the spread of the degenerative process in the brain with MSA and clinical manifestations, it can be represented either in the form of OPCA (dominated by cerebellar signs), or in the form of strigo-nigral degeneration (Parkinsonism predominates), or under the description of the Shay- on the first place symptoms PVN). In those cases where clinical manifestations are less specific, it is lawful to use "multisite atrophy" as a diagnostic term. In general, among the main motor manifestations Parkinsonism (about 90% of all cases) comes first, followed by cerebellum signs (about 55%) and pyramidal symptoms (about 50% -60%). The majority of patients show some degree of IVF (74%). As a rule, patients do not respond to L-DOPA treatment (with a few exceptions).

Parkinsonism in MSA, in contrast to Parkinson's disease, manifests a symmetric akinetic-rigid syndrome without tremor. Only in the form of an exception, asymmetric parkinsonism with rest tremor can be observed, but for the Parkinson's disease, the cerebellar and pyramidal signs are not characteristic. Cerebellar ataxia in the MCA picture, as a rule, manifests itself when walking (dysbasia) and in speech. Both the dysbasia and dysarthria in ISA are mixed, since they are mainly due to extrapyramidal and cerebellar disorders. Muscle tone in the limbs is a mixed pyramidal-extrapyramidal type, in which it is often difficult to identify and measure elements of spasticity and rigidity.

In most cases, symptoms of prostate cancer precede motor impairment and are manifested by orthostatic hypotension, anhidrosis, vesicidal disorders (the so-called neurogenic bladder), and impotence. Perhaps the emergence of other symptoms of PID (a fixed heart rate, pupillary disorders, sleep apnea, etc.).

Craniocerebral injury

Craniocerebral trauma, especially severe, can lead to any variants of the pyramidal syndrome (mono-, hemi-, para-, tri- and tetraparesis) and their combination with a variety of extrapyramidal disorders (more often in the form of akinetic-rigid syndrome, more rarely other hyperkinetic syndromes).

Complex parkinsonism - amyotrophic lateral sclerosis - dementia

This form is found in endemic areas, mainly on the island of Guam.

Creutzfeldt-Jakob disease

The disease belongs to the group of prion diseases, which are caused by a very special infectious particle called a prion. In typical cases, the disease begins at the age of 50-60 years and has a subchronic flow (usually 1-2 years) with a fatal outcome. For Creutzfeldt-Jakob disease, apart from dementia, akinetic-rigid syndrome and other extrapyramidal disorders (myoclonus, dystonia, tremor), pyramidal, cerebellar and anterolone symptoms are characteristic. Approximately one-third of cases develop epileptic seizures.

In the diagnosis of Creutzfeldt-Jakob disease, great importance is attached to combining such manifestations as subacute progressive dementia, myoclonus, typical periodic complexes on the EEG (the three-phase and polyphasic activity of the acute form with an amplitude of up to 200 μV occurring at a frequency of 1.5-2 per second), normal composition cerebrospinal fluid.

Parkinsonism syndrome in Creutzfeldt-Jakob disease is observed in the context of massive neurological (including pyramidal) symptoms, which goes far beyond the semiology of akinetic-rigid syndromes.

Vascular Parkinsonism

The diagnosis of vascular parkinsonism can be made only taking into account the paraclinical examination and the presence of an appropriate clinical picture.

Unfortunately, not always in the neurological status of these patients it is possible to detect clinical signs of discirculatory encephalopathy in the form of microsymptoms or rather crude neurological syndromes, indicative of the lesion of one or several vascular pools of the brain. Only in typical cases, there is pyramidal insufficiency, pseudobulbar syndrome of varying degrees, various residual manifestations of cerebral circulation disorders in the form of cerebellar signs, sensitive disorders, and mnestic and intellektkalnyh disorders. If such signs are revealed, then they may be accompanied by symptoms of vascular myelopathy, which indicates a diffuse vascular insufficiency both at the cerebral and spinal levels. Often, you can find signs of vascular lesions of internal organs (heart, kidneys, eyes) and vessels of the extremities.

It is extremely important to pay attention to the features of neurological manifestations of the parkinsonism itself. In typical cases, the onset of vascular parkinsonism is acute or subacute (but can be chronic) followed by spontaneous improvement or steady flow. In diagnostically difficult cases, the course can be progressive, but in such cases, as a rule, there is a step-like progression, reflecting the fluctuations and the relative reversibility of symptoms.

Neurological manifestations of vascular parkinsonism are also peculiar. Recall that it is characterized by the absence of tremors and, as a rule, non-responsiveness to dopa-containing drugs, often the predominant defeat of the lower extremities on both sides (the so-called "parkinsonism of the lower half of the body") with pronounced dysbasia. At the same time, and described vascular hemiparkinsonism (as a fairly rare syndrome).

Today it is generally accepted that the diagnosis of vascular parkinsonism requires mandatory neuroimaging (preferably with MRI), which reveals in such cases multiple cerebral infarctions (usually) or single lacunar infarctions or hemorrhages (rarely). The absence of changes in MRI should serve as an excuse for further pre-examination and clarification of the possible nature of the disease.

Binswanger disease

Binswanger's disease or subcortical arteriosclerotic encephalopathy is a variant of vascular dementia, associated primarily with hypertensive disease, and manifested by dementia, pseudobulbar syndrome, high tendon reflexes, hypokinesia, Parkinson-like dysbasia and the characteristic changes in the MRI picture (leukoareosis). In the picture of Binswanger's disease, a true syndrome of vascular parkinsonism is described. Thus, a combination of pyramidal and extrapyramidal syndromes can occur in this disease.

Dopa-responsive dystonia

Dystonia, sensitive to levodopa (Segawa's disease) is an independent form of hereditary primary dystonia. It is characterized by the onset in the first decade of life, the first symptoms in the legs, expressed by the daily fluctuation of symptoms (another name for the disease: dystonia with pronounced daily fluctuations), the presence of Parkinsonian symptoms and the dramatic effect of small doses of levodopa. The daily fluctuations in symptoms are sometimes expressed so that the patient, normally moving around in the morning, is no longer able to walk unaided during the day. Sometimes severe dystonia simulates paraplegia. The gait looks spastic with knees bent, crossed, with fluttering feet. Tendon reflexes are alive or high. Sometimes the elements of parkinsonism are revealed: stiffness with the phenomenon of "cogwheel", hypomia, hypokinesia, rarely - a tremor of rest. Such patients are often diagnosed with an erroneous diagnosis of cerebral palsy or Strympel disease, or juvenile parkinsonism.

Encephalitis

Encephalitis (meningoencephalitis and encephalomyelitis) viral (including HIV infection) or post- and para-infectious can sometimes include in their motor manifestations a variety of hyperkinetic and other extrapyramidal and pyramidal syndromes. In typical cases, accompanying or preceding common infectious symptoms, changes in consciousness and other possible syndromes (cephalgic, epileptic, cerebrospinal) are characteristic.

Disease of accumulation

Diseases of accumulation (lipidosis, leukodystrophy, mucopolysaccharidosis) are hereditary in nature, often begin to manifest themselves in childhood and in most cases are characterized by polysystemic neurological manifestations, including mental development disorders, pyramidal, extrapyramidal, cerebellar disorders, epileptic seizures. Often observed changes in the eyes (retinitis) and internal organs.

Metabolic encephalopathies

Metabolic encephalopathies that can develop in diseases of the liver, kidneys, pancreas, endocrine and other somatic diseases are manifested by acute or slowly progressing cerebral disorders (including disorders of consciousness), asterixis, sometimes epileptic seizures, mental disorders (confusion, cognitive impairment) , slowing of electrical activity on the EEG. In neurological status, in addition, metabolic tremor, myoclonus, tendon hyperexflexia, pathological stop signs are sometimes found. In the diagnosis, the somatic examination of the patient and the screening of metabolic disorders are important.

Houteon Huntington

Some forms of Huntington's chorea, in particular the so-called Westphalian form (akinetic-rigid in the onset of the disease) or the late stages of the classical chorea of Huntington, are also manifested by pyramidal signs, in addition to the choreic and akinetic-rigid syndromes.

Volumetric education in the brain

Volumetric education in the brain often begins to manifest progressive pyramidal syndrome or defeat of the cranial nerves against the background of increasing cerebral disorders; however, there may be a combined pyramidal-extrapyramidal syndrome (eg, hemiparkinsonism syndrome with pyramidal signs). Often the symptoms of parkinsonism dominate the clinical picture. In fact, any hemiparkinsonism requires at a certain stage diagnostic procedures to exclude the volumetric process, especially when combined with other (pyramidal and / or cerebral) manifestations. Diagnosis is most often provided by CT or MRI. Every patient with Parkinson's needs to be examined using neuroimaging methods.

Hyponymy can be observed:

• With parkinsonism syndrome
• With depression (mood changes, behavior, history data)
• As one of the manifestations of pseudobulbar paralysis in bilateral lesions of corticobulbar pathways
• As an isolated violation of voluntary motor activity, with the preservation of the automatic motor activity of the mouth and swallowing muscles due to bilateral damage to the front of the tire, the brainstem is called the Foix-Cavany-Marie syndrome.

The combination of psychogenic and organic hyperkinesis (characteristic clinical features)

• Psychogenic dyskinesia usually complicates the previous organic disease that occurs with a dyskinetic syndrome, that is, psychogenic motor disorders (PDR) in the form of psychogenic hyperkinesis "overlaps" the already existing organic hyperkinesis.
• The new (psychogenic) type of dyskinesia is phenomenologically different from the original (organic) dyskinesia, but it can also be analogous (tremor, parkinsonism, dystonia, etc.).
• Psychogenic dyskinesia usually affects the same part of the body as organic hyperkinesia. Psychogenic dyskinesia is usually the main cause of maladaptation of the patient: it causes more severe maladaptation than the concomitant organic hyperkinesis.
• Suggestion or placebo reduces or stops PDR, "layering" on organic dyskinesia. Motor DAD patterns do not correspond to organic types of hyperkinesis.
• Presence of other PDR (multiple motor disorders). Multiple somatization and obvious mental disorders.