Pseudobulbar syndrome

Pseudobulbar paralysis (supranuclear bulbar paralysis) is a syndrome characterized by paralysis of the muscles innervated by V, VII, IX, X, XII by the cranial nerves, as a result of bilateral damage to the cortical-nuclear pathways to the nuclei of these nerves. In this case, bulbar functions suffer, mainly articulation, phonation, swallowing and chewing (dysarthria, dysphonia, dysphagia). But unlike bulbar paralysis, muscle atrophies are not observed and reflexes of oral automatism take place: increased proboscis reflex; the Nazo-labial reflex of Astvatsaturov appears; the Oppenheim reflex (sucking movements in response to the dashed stimulation of the lips), distant-oral and some other similar reflexes, as well as pathological laughter and crying. The study of the pharyngeal reflex is less informative.

[1], [2],

Causes of pseudobulbar palsy

Neurologists will highlight the following main causes of pseudobulbar paralysis:

1. Vascular diseases affecting both hemispheres (lacunar state in hypertensive disease, atherosclerosis, vasculitis).
2. Perinatal pathology and birth trauma, including.
3. Congenital bilaterally occipital conduction syndrome.
4. Craniocerebral injury.
5. Episodic pseudobulbar palsy with epileptiform opercular syndrome in children.
6. Degenerative diseases with pyramidal and extrapyramidal systems: ALS, primary lateral sclerosis, family spastic paraplegia (rarely), OPCA, Pick's disease, Creutzfeldt-Jacob disease, progressive supranuclear palsy, Parkinson's disease, multiple systemic atrophy, other extrapyramidal diseases.
7. Demyelinating diseases.
8. Consequences of encephalitis or meningitis.
9. Multiple or diffuse (glioma) neoplasm.
10. Hypoxic (anoxic) encephalopathy ("lively brain disease").
11. Other reasons.

[3], [4], [5], [6], [7]

Vascular diseases

Vascular diseases affecting both hemispheres are the most common cause of pseudobulbar paralysis. Repeated ischemic disorders of the cerebral circulation, usually in people over 50 years old, with hypertension, atherosclerosis, vasculitis, systemic diseases, heart and blood diseases, multiple lacunar cerebral infarctions, etc., usually lead to a picture of pseudobulbar paralysis. The latter can sometimes develop and with a single stroke, apparently due to decompensation of latent vascular cerebral insufficiency in the other hemisphere. In vascular pseudobulbar paralysis, the latter can be accompanied by hemiparesis, tetraparesis, or bilateral pyramidal insufficiency without a paresis. Vascular disease of the brain, usually confirmed by the MRI picture, is detected.

Perinatal pathology and birth trauma

Due to perinatal hypoxia or asphyxia, as well as birth trauma, various forms of cerebral palsy (cerebral palsy) can develop with the development of spastic-paretic (diplegic, hemiplegic, tetraplegic), dyskinetic (mainly dystonic), atactic and mixed syndromes, including with a picture of pseudobulbar paralysis. In addition to periventricular leukomalacia, these children often have a unilateral hemorrhagic infarction. More than half of these children show symptoms of mental retardation; about one-third develop epileptic seizures. In anamnesis, there are usually indications of perinatal pathology, delay of psychomotor development, and in the neurological status, residual symptoms of perinatal encephalopathy are revealed.

The differential diagnosis of infantile cerebral palsy includes some degenerative and hereditary metabolic disorders (glutaric acididia of type I, arginase deficiency, dopa-respondive dystonia, hyperexpension (with rigidity), Lesha-Nihana disease), and progressive hydrocephalus, subdural hematoma. MRI detects certain impairments in the brain in almost 93% of patients with cerebral palsy.

Congenital bilateral water supply syndrome

This defect occurs in pediatric neurological practice. He leads (as well as congenital bilateral hip- pocampal sclerosis) to a pronounced impairment of speech development, which sometimes even imitates children's autism and the picture of pseudobulbar paralysis (mainly with speech disorders and dysphagia). The lag in mental development and epileptic seizures are observed in about 85% of cases. MRI detects the malformation of the near-silvic gyri.

[8], [9], [10], [11], [12], [13]

Severe craniocerebral injury (CCI)

Severe craniocerebral trauma in adults and children often leads to different variants of the pyramidal syndrome (spastic mono-, hemi, tri- and tetraparesis or plegia) and pseudobulbar disorders with gross speech and swallowing disorders. The connection with a trauma in the anamnesis does not leave an occasion for diagnostic doubts.

Epilepsy

Describes episodic pseudobulbar paralysis in epileptiform opercular syndrome in children (paroxysmal oral apraxia, dysarthria and salivation) observed in the slow phase of night sleep. The diagnosis is confirmed by epileptic discharges in the EEG during a night attack.

[14], [15]

Degenerative diseases

Many degenerative diseases involving pyramidal and extrapyramidal systems may be accompanied by pseudobulbar syndrome. These diseases include amyotrophic lateral sclerosis, progressive supranuclear palsy (these forms are the most common cause of pseudobulbar syndrome) primary lateral sclerosis, family spastic paraplegia (rarely leads to severe pseudobulbar syndrome), Pick's disease, Creutzfeldt-Jakob disease, Parkinson's disease, secondary Parkinsonism, multiple systemic atrophy, less often - other extrapyramidal diseases.

[16], [17], [18], [19]

Demyelinating diseases

Demyelinating diseases often involve corticobulbar pathways on both sides, leading to pseudobulbar syndrome (multiple sclerosis, post-infection and post-vaccination encephalomyelitis, progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis, AIDS dementia complex, adrenoleukodystrophy).

In the same group ("Myelin Disease") can be attributed metabolic myelin (Pelicius-Merzbacher disease, Alexander's disease, metachromatic leukodystrophy, globoid leukodystrophy).

[20], [21], [22], [23], [24],

Consequences of encephalitis and meningitis

Encephalitis, meningitis and meningoencephalitis, along with other neurological syndromes, may include pseudobulbar syndrome in their manifestations. Symptoms of the main infectious brain lesion are always revealed.

[25], [26], [27], [28], [29], [30]

Multiple or diffuse glioma

Some variants of glioma of the brain stem manifest variable clinical symptoms depending on its location within the caudal, middle (variolium bridge) or oral sections of the brainstem. Most often this tumor begins in childhood (in 80% of cases up to 21 years) with symptoms of involvement of one or more cranial nerves (usually VI and VII on one side), progressive hemiparesis or paraparesis, ataxia. Sometimes the conductor symptoms precede the defeat of the cranial nerves. Headaches, vomiting, edema on the fundus are added. A pseudobulbar syndrome develops.

Differential diagnosis with pontine form of multiple sclerosis, vascular malformation (usually cavernous hemangioma) and stem encephalitis. In the differential diagnosis, an essential aid is provided by MRI. It is important to distinguish the focal and diffuse form of glioma (astrocytoma).

Hypoxic (anoxic) encephalopathy

Hypoxic encephalopathy with serious neurologic complications is typical for patients who survived resuscitation after asphyxia, clinical death, prolonged coma, etc. The consequences of severe hypoxia, in addition to a prolonged coma in the acute period, include several clinical options, including dementia with or without extrapyramidal syndromes, cerebellar ataxia, myoclonic syndromes, and Korsakov's amnestic syndrome. Delayed decicive encephalopathy with a poor outcome is considered separately .

Sometimes patients with hypoxic encephalopathy are found in whom persistent residual phenomena consist in the predominant hypokinesia of bulbar functions (hypokinetic dysarthria and dysphagia) against a background of minimal or completely regressing general hypokinesia and hypomia (this variant of pseudobulbar disorders is called "extrapyramidal pseudobulbar syndrome" or "pseudo pseudobulbar syndrome "). These patients have no violations in the extremities and trunk, but are disabled in connection with the above manifestations of a peculiar pseudobulbar syndrome.

Other causes of pseudobulbar syndrome

Sometimes pseudobulbar syndrome manifests itself as an integral part of more extensive neurological syndromes. For example, pseudobulbar syndrome in the picture of central bridge myelinolysis (malignant neoplasm, hepatic insufficiency, sepsis, alcoholism, chronic renal failure, lymphoma, cachexia, severe dehydration and electrolyte disorders, hemorrhagic pancreatitis, pellagra) and overlapping syndrome of "locked man" (occlusion main artery, craniocerebral trauma, viral encephalitis, postvaccinal encephalitis, swelling, hemorrhage, central myelinolysis of the bridge).

Central myelinolysis of the bridge is a rare and potentially lethal syndrome that is manifested by the rapid development of tetraplegia (against the background of somatic disease or Wernicke's encephalopathy) and pseudobulbar paralysis due to demyelination of the central sections of the bridge, which is visible on the MRI and in turn can lead to the syndrome of " person ". The "locked man" syndrome ("isolation" syndrome, de-efferentation syndrome) is a condition in which selective supranuclear motor de-afference leads to paralysis of all four limbs and caudal sections of craniocerebral innervation without disturbance of consciousness. The syndrome is manifested by tetraplegia, mutism (aphonia and anarthria of pseudobulbar origin) and inability to swallow with conserved consciousness; while the possibility of communication is limited only by vertical movements of the eyes and eyelids. CT or MRI reveals the destruction of the medioventral part of the variolium bridge.