Combination of pyramidal and extrapyramidal syndromes

Some diseases of the central nervous system manifest themselves as a combination of pyramidal and extrapyramidal syndromes. These leading clinical syndromes may be accompanied by other manifestations (dementia, ataxia, apraxia, and others), but often the indicated combination of syndromes constitutes the main clinical core of the disease.

Main reasons:

1. Atrophic processes in the brain
2. Progressive supranuclear palsy
3. Corticobasal degeneration
4. Multiple system atrophy (strionigral degeneration, Shy-Drager syndrome, OPCA)
5. Traumatic brain injury
6. Parkinsonism - ALS - Dementia Complex
7. Creutzfeldt-Jakob disease
8. Vascular parkinsonism
9. Binswanger's disease
10. Dopa-responsive dystonia (Segawa disease)
11. Encephalitis (including HIV infection)
12. Storage diseases
13. Metabolic encephalopathies
14. Huntington's chorea
15. Volumetric formations in the brain

Atrophic processes in the brain

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

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

The paper describes patients with progressive visual impairment (lower visual field defect reflecting cortical dysfunction above the fissura calcarina) in Alzheimer's disease, which until now was attributed to manifestations of different diseases. The literature contains references to cases in which the histopathological picture in the brain overlapped corticobasal degeneration, Pick's disease, and Alzheimer's disease. It also contains descriptions of Alzheimer's disease with progressive spasticity (primary lateral sclerosis) or cases of primary lateral sclerosis with frontal neuropsychological impairment. Separate descriptions are devoted to observations in which the histopathological picture included manifestations of corticobasal degeneration and Pick's disease; Pick's disease and diffuse Lewy body disease; Alzheimer's disease, parkinsonism, and ALS; corticobasal degeneration, progressive supranuclear palsy, and multiple system atrophy; Parkinson's disease and motor neuron disease.

All of these cases show asymmetrical relatively focal cortical atrophy on MRI (in addition to ventricular dilation in some cases).

Further studies, including molecular genetic ones, will shed light on the nature of these rare atrophic-degenerative mixed syndromes.

Progressive supranuclear palsy

The disease usually begins in the 5th-6th decade of life, has a progressive course and is difficult to treat. Clinical manifestations are characterized mainly by a triad of syndromes:

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

The most characteristic signs are gaze disturbances when looking down, postural instability with dysbasia and unexplained falls, "straight" posture (rigid neck in the extension position), moderate hypokinesia. Progressive supranuclear palsy only resembles parkinsonism due to the presence of hypokinesia, postural instability and dysarthria; it is hardly possible to speak of true parkinsonism in this disease. In some cases, mild pyramidal signs are possible. Diagnostic criteria and exclusion criteria for progressive supranuclear palsy have been developed.

Corticobasal degeneration

This is a sporadic, slowly progressive degenerative disease of adulthood, manifested by an asymmetric akinetic-rigid syndrome, accompanied by other ("parkinsonism plus") involuntary movements (myoclonus, dystonia, tremor) and lateralized cortical dysfunction (apraxia of the limb, alien hand syndrome, sensory disturbances in the form of astereognosis, disorders of the discriminative sense, sense of localization). Cognitive defect develops at later stages of the disease. If the dominant hemisphere is affected, aphasia may appear, which is usually not very severe. Pyramidal signs are also common, but are usually moderately expressed, manifesting only by hyperreflexia of varying degrees of severity.

Characteristic features include progressive slowness of movements, mask-like face, muscle rigidity, flexor posture, dysbasia (postural disorders and falls), awkwardness in one of the limbs (most often in the hand), and cortical myoclonus. Frontal signs in the form of a grasping reflex and paratonia are possible. Macroscopically, corticobasal degeneration reveals asymmetric atrophy in the frontal and parietal cortex, especially in the area surrounding the Rolandic and Sylvian fissures. Depigmentation of the substantia nigra is no less characteristic. In advanced stages of the disease, asymmetric frontoparietal atrophy is confirmed by CT or MRI data. In the initial stages, the disease can easily be confused with manifestations of Parkinson's disease. For correct diagnosis, great importance is attached to the detection of asymmetric apraxic disorders. Let us recall that another name for this disease is progressive apraxic rigidity. Dopa-containing drugs are usually not effective.

Multiple system atrophies

Clinically, all forms of multiple system atrophy (strionigral degeneration, Shy-Drager 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 in MSA and clinical manifestations, it can be presented either in the form of OPCA (cerebellar signs dominate), or in the form of strionigral degeneration (the picture of Parkinsonism prevails), or fit the description of the Shy-Drager variant (symptoms of PVN come first). In cases where clinical manifestations are less specific, it is legitimate to use "multisystem atrophy" as a diagnostic term. In general, among the main motor manifestations, parkinsonism comes first (about 90% of all cases), followed by cerebellar signs (about 55%) and pyramidal symptoms (about 50%-60%). Most patients exhibit some degree of PVN (74%). As a rule, patients do not respond to L-DOPA treatment (with a few exceptions).

Parkinsonism in MSA, unlike Parkinson's disease, is manifested by a symmetrical akinetic-rigid syndrome without tremor. Only as an exception can asymmetrical parkinsonism with resting tremor be observed, but cerebellar and pyramidal signs are not characteristic of Parkinson's disease. Cerebellar ataxia in the picture of MSA, as a rule, is manifested during walking (dysbasia) and speech. Both dysbasia and dysarthria in MSA are of a mixed nature, since they are caused mainly by extrapyramidal and cerebellar disorders. Muscle tone in the limbs is of a mixed pyramidal-extrapyramidal type, in which it is often difficult to isolate and measure elements of spasticity and rigidity.

In most cases, symptoms of PVN precede motor disorders and are manifested by orthostatic hypotension, anhidrosis, bladder disorders (so-called neurogenic bladder) and impotence. Other symptoms of PVN (fixed heart rate, pupillary disorders, sleep apnea, etc.) may also appear.

Traumatic brain injury

Traumatic brain injury, especially severe, can lead to any variants of pyramidal syndrome (mono-, hemi-, para-, tri- and tetraparesis) and their combination with various extrapyramidal disorders (usually in the form of akinetic-rigid syndrome, less often - other hyperkinetic syndromes).

Parkinsonism - amyotrophic lateral sclerosis - dementia complex

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 course (usually 1-2 years) with a fatal outcome. Creutzfeldt-Jakob disease is characterized, in addition to dementia, akinetic-rigid syndrome and other extrapyramidal disorders (myoclonus, dystonia, tremor), pyramidal, cerebellar and anterior corneal symptoms. Epileptic seizures develop in about one third of cases.

In the diagnosis of Creutzfeldt-Jakob disease, great importance is attached to the combination of such manifestations as subacute progressive dementia, myoclonus, typical periodic complexes on the EEG (triphasic 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), and normal cerebrospinal fluid composition.

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

Vascular parkinsonism

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

Unfortunately, it is not always possible to detect clinical signs of discirculatory encephalopathy in the neurological status of these patients in the form of microsymptoms or rather severe neurological syndromes indicating damage to one or more vascular basins of the brain. Only in typical cases is there pyramidal insufficiency, pseudobulbar syndrome of varying severity, various residual manifestations of cerebellar circulation disorders in the form of cerebellar signs, sensory disturbances, mnemonic and intellectual disorders. If such signs are detected, they may be accompanied by symptoms of vascular myelopathy, which indicates diffuse vascular insufficiency at both the cerebral and spinal levels. Signs of vascular damage to internal organs (heart, kidneys, eyes) and vessels of the extremities can often be detected.

It is extremely important to pay attention to the features of the neurological manifestations of Parkinsonism itself. In typical cases, the onset of vascular Parkinsonism is acute or subacute (but can also be chronic) with subsequent spontaneous improvement or stationary course. In diagnostically difficult cases, the course can be progressive, but even in such cases, as a rule, there is a stepwise progression, reflecting fluctuations and relative reversibility of symptoms.

Neurological manifestations of vascular parkinsonism are also peculiar. Let us recall that it is characterized by the absence of tremor and, as a rule, non-response to dopa-containing drugs, often predominantly affecting the lower extremities on both sides (the so-called "parkinsonism of the lower half of the body") with pronounced dysbasia. At the same time, vascular hemiparkinsonism has also been described (as a fairly rare syndrome).

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

Binswanger's disease

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

Dopa-responsive dystonia

Dystonia sensitive to levodopa (Segawa disease) is an independent form of hereditary primary dystonia. It is characterized by onset in the first decade of life, the first symptoms in the legs, pronounced daily fluctuation of symptoms (another name for the disease: dystonia with pronounced daily fluctuations), the presence of parkinsonism symptoms and a dramatic effect of small doses of levodopa. Daily fluctuations in symptoms are sometimes so pronounced that the patient, moving normally in the morning, is no longer able to walk without assistance during the day. Sometimes severe dystonia simulates paraplegia. The gait looks spastic with bent knees, crossed, with dragging feet. Tendon reflexes are lively or high. Sometimes elements of parkinsonism are revealed: rigidity with the "cogwheel" phenomenon, hypomimia, hypokinesia, rarely - resting tremor. Such patients are often misdiagnosed with cerebral palsy or Strumpell's disease, or juvenile parkinsonism.

Encephalitis

Encephalitis (meningoencephalitis and encephalomyelitis) viral (including HIV infection) or post- and parainfectious can sometimes include in their motor manifestations various hyperkinetic and other extrapyramidal, as well as pyramidal syndromes. In typical cases, accompanying or preceding general infectious symptoms, changes in consciousness and other possible syndromes (cephalgic, epileptic, cerebrospinal fluid) are characteristic.

Storage diseases

Storage diseases (lipidoses, leukodystrophies, mucopolysaccharidoses) are hereditary, most 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. Changes in the eyes (retinitis) and internal organs are often observed.

Metabolic encephalopathies

Metabolic encephalopathies, which can develop with diseases of the liver, kidneys, pancreas, endocrine and other somatic diseases, are manifested by acute or slowly progressing general cerebral disorders (including impaired consciousness), asterixis, sometimes epileptic seizures, mental disorders (confusion, cognitive impairment), slowing of electrical activity on the EEG. In addition, metabolic tremor, myoclonus, tendon hyperreflexia, pathological foot signs are sometimes detected in the neurological status. Somatic examination of the patient and screening for metabolic disorders are of great importance in diagnostics.

Huntington's chorea

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

Volumetric formations in the brain

Space-occupying lesions in the brain most often begin to manifest as progressive pyramidal syndrome or cranial nerve damage against the background of increasing general cerebral disorders; however, a combined pyramidal-extrapyramidal syndrome may occur (for example, hemiparkinsonism syndrome with pyramidal signs). Parkinsonism symptoms often dominate the clinical picture. In fact, any hemiparkinsonism requires, at a certain stage of diagnostic procedures, the exclusion of a space-occupying process, especially when combined with other (pyramidal and/or general cerebral) manifestations. Diagnostics is most often ensured by the use of CT or MRI. Each patient with parkinsonism requires examination using neuroimaging methods.

Hypomimia can be observed:

• In Parkinsonism syndrome
• In case of depression (changes in mood, behavior, anamnesis data)
• As one of the manifestations of pseudobulbar paralysis in bilateral lesions of the corticobulbar tract
• As an isolated disorder of voluntary motor activity, with preservation of automatic motor activity of the mouth and swallowing muscles due to bilateral damage to the anterior part of the tegmentum, the brainstem, the so-called Fox-Cavany-Marie syndrome.

Combination of psychogenic and organic hyperkinesis (characteristic clinical features)

• Psychogenic dyskinesias usually complicate a previous organic disease that occurs with dyskinetic syndrome, that is, psychogenic movement disorders (PMD) in the form of psychogenic hyperkinesis are, as it were, “superimposed” on the already existing organic hyperkinesis.
• The new (psychogenic) type of dyskinesia is phenomenologically different from the original (organic) dyskinesia, but can also be similar (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 the patient's maladaptation: it causes more pronounced maladaptation than the accompanying organic hyperkinesia.
• Suggestion or placebo reduce or stop PDR, "layered" on organic dyskinesia. Motor patterns of PDR do not correspond to organic types of hyperkinesia.
• Presence of other MDD (multiple movement disorders). Multiple somatization and obvious mental disorders.