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Motor neuron diseases or motor neurone diseases (MNDs) are a group of rare neurodegenerative disorders that selectively affect motor neurons, the cells which control voluntary muscles of the body. They include amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular atrophy (PMA), primary lateral sclerosis (PLS), spinal muscular atrophy (SMA) and monomelic amyotrophy (MMA), as well as some rarer variants resembling ALS.
Central pontine myelinolysis is a neurological condition involving severe damage to the myelin sheath of nerve cells in the pons. It is predominately iatrogenic (treatment-induced), and is characterized by acute paralysis, dysphagia, dysarthria, and other neurological symptoms.
The medulla oblongata or simply medulla is a long stem-like structure which makes up the lower part of the brainstem. It is anterior and partially inferior to the cerebellum. It is a cone-shaped neuronal mass responsible for autonomic (involuntary) functions, ranging from vomiting to sneezing. The medulla contains the cardiac, respiratory, vomiting and vasomotor centers, and therefore deals with the autonomic functions of breathing, heart rate and blood pressure as well as the sleep–wake cycle.
Leigh syndrome is an inherited neurometabolic disorder that affects the central nervous system. It is named after Archibald Denis Leigh, a British neuropsychiatrist who first described the condition in 1951. Normal levels of thiamine, thiamine monophosphate, and thiamine diphosphate are commonly found, but there is a reduced or absent level of thiamine triphosphate. This is thought to be caused by a blockage in the enzyme thiamine-diphosphate kinase, and therefore treatment in some patients would be to take thiamine triphosphate daily. While the majority of patients typically exhibit symptoms between the ages of 3 and 12 months, instances of adult onset have also been documented.
The pyramidal tracts include both the corticobulbar tract and the corticospinal tract. These are aggregations of efferent nerve fibers from the upper motor neurons that travel from the cerebral cortex and terminate either in the brainstem (corticobulbar) or spinal cord (corticospinal) and are involved in the control of motor functions of the body.
Athetosis is a symptom characterized by slow, involuntary, convoluted, writhing movements of the fingers, hands, toes, and feet and in some cases, arms, legs, neck and tongue. Movements typical of athetosis are sometimes called athetoid movements. Lesions to the brain are most often the direct cause of the symptoms, particularly to the corpus striatum. This symptom does not occur alone and is often accompanied by the symptoms of cerebral palsy, as it is often a result of this physical disability. Treatments for athetosis are not very effective, and in most cases are simply aimed at managing the uncontrollable movement, rather than the cause itself.
Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain. The condition leads to symptoms including loss of balance, slowing of movement, difficulty moving the eyes, and cognitive impairment. PSP may be mistaken for other types of neurodegeneration such as Parkinson's disease, frontotemporal dementia and Alzheimer's disease. The cause of the condition is uncertain, but involves the accumulation of tau protein within the brain. Medications such as levodopa and amantadine may be useful in some cases.
Pseudobulbar affect (PBA), or emotional incontinence, is a type of neurological disorder characterized by uncontrollable episodes of crying or laughing. PBA occurs secondary to a neurologic disorder or brain injury. Patients may find themselves crying uncontrollably at something that is only slightly sad, being unable to stop themselves for several minutes. Episodes may also be mood-incongruent: a patient may laugh uncontrollably when angry or frustrated, for example. Sometimes, the episodes may switch between emotional states, resulting in the patient crying uncontrollably before dissolving into fits of laughter.
Pallidotomy is a neurosurgical procedure. It is used to treat Parkinson's disease and some other conditions, often as an alternative to deep brain stimulation. It involves placing a tiny electrical probe in the globus pallidus, one of the basal ganglia of the brain, to damage it. Unilateral pallidotomy can cause side effects including problems with language learning, visuospatial constructional ability, and executive functions. Bilateral pallidotomy is not effective, with many severe side effects.
The facial motor nucleus is a collection of neurons in the brainstem that belong to the facial nerve. These lower motor neurons innervate the muscles of facial expression and the stapedius.
Fazio–Londe disease (FLD), also called progressive bulbar palsy of childhood, is a very rare inherited motor neuron disease of children and young adults and is characterized by progressive paralysis of muscles innervated by cranial nerves. FLD, along with Brown–Vialetto–Van Laere syndrome (BVVL), are the two forms of infantile progressive bulbar palsy, a type of progressive bulbar palsy in children.
Progressive bulbar palsy (PBP) is a medical condition. It belongs to a group of disorders known as motor neuron diseases. PBP is a disease that attacks the nerves supplying the bulbar muscles. These disorders are characterized by the degeneration of motor neurons in the cerebral cortex, spinal cord, brain stem, and pyramidal tracts. This specifically involves the glossopharyngeal nerve (IX), vagus nerve (X), and hypoglossal nerve (XII).
Foix–Chavany–Marie syndrome (FCMS), also known as bilateral opercular syndrome, is a neuropathological disorder characterized by paralysis of the facial, tongue, pharynx, and masticatory muscles of the mouth that aid in chewing. The disorder is primarily caused by thrombotic and embolic strokes, which cause a deficiency of oxygen in the brain. As a result, bilateral lesions may form in the junctions between the frontal lobe and temporal lobe, the parietal lobe and cortical lobe, or the subcortical region of the brain. FCMS may also arise from defects existing at birth that may be inherited or nonhereditary. Symptoms of FCMS can be present in a person of any age and it is diagnosed using automatic-voluntary dissociation assessment, psycholinguistic testing, neuropsychological testing, and brain scanning. Treatment for FCMS depends on the onset, as well as on the severity of symptoms, and it involves a multidisciplinary approach.
Bulbar palsy refers to a range of different signs and symptoms linked to impairment of function of the glossopharyngeal nerve, the vagus nerve, the accessory nerve, and the hypoglossal nerve. It is caused by a lower motor neuron lesion in the medulla oblongata, or from lesions to these nerves outside the brainstem, and also botulism. This may be caused by any of a number of genetic, vascular, degenerative, inflammatory, and other underlying conditions. It can be differentiated from pseudobulbar palsy. When there is airway obstruction, intubation is used.
Conjugate gaze palsies are neurological disorders affecting the ability to move both eyes in the same direction. These palsies can affect gaze in a horizontal, upward, or downward direction. These entities overlap with ophthalmoparesis and ophthalmoplegia.
Cranial nerve disease is an impaired functioning of one of the twelve cranial nerves. Although it could theoretically be considered a mononeuropathy, it is not considered as such under MeSH.
Brown-Vialetto-Van-Laere syndrome (BVVL), sometimes known as Brown's syndrome, is a rare degenerative disorder often initially characterized by progressive sensorineural deafness.
Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) or Lou Gehrig's disease, is a rare and terminal neurodegenerative disease that results in the progressive loss of motor neurons that control voluntary muscles. ALS is the most common form of the motor neuron diseases. Early symptoms of ALS include stiff muscles, muscle twitches, gradual increasing weakness, and muscle wasting. Limb-onset ALS begins with weakness in the arms or legs, while bulbar-onset ALS begins with difficulty in speaking or swallowing. Around half of people with ALS develop at least mild difficulties with thinking and behavior, and about 15% develop frontotemporal dementia. Motor neuron loss continues until the abilities to eat, speak, move, or, lastly, breathe are lost.
Tumefactive multiple sclerosis is a condition in which the central nervous system of a person has multiple demyelinating lesions with atypical characteristics for those of standard multiple sclerosis (MS). It is called tumefactive as the lesions are "tumor-like" and they mimic tumors clinically, radiologically and sometimes pathologically.
Basal ganglia disease is a group of physical problems that occur when the group of nuclei in the brain known as the basal ganglia fail to properly suppress unwanted movements or to properly prime upper motor neuron circuits to initiate motor function. Research indicates that increased output of the basal ganglia inhibits thalamocortical projection neurons. Proper activation or deactivation of these neurons is an integral component for proper movement. If something causes too much basal ganglia output, then the ventral anterior (VA) and ventral lateral (VL) thalamocortical projection neurons become too inhibited, and one cannot initiate voluntary movement. These disorders are known as hypokinetic disorders. However, a disorder leading to abnormally low output of the basal ganglia leads to reduced inhibition, and thus excitation, of the thalamocortical projection neurons which synapse onto the cortex. This situation leads to an inability to suppress unwanted movements. These disorders are known as hyperkinetic disorders.
References
- ↑ Tidy C (21 October 2021). Knott L (ed.). "Bulbar and Pseudobulbar Palsy. What is Bulbar Palsy?". Patient. Retrieved 2016-03-26.
- ↑ McCormick WE, Lee JH (May 2002). "Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases". Skull Base. 12 (2): 67–71. doi:10.1055/s-2002-31568-1. PMC 1656925 . PMID 17167648.
- ↑ Saleem, Fatima; Munakomi, Sunil (2024). "Pseudobulbar Palsy". StatPearls. StatPearls Publishing. Retrieved 20 February 2024.
- ↑ Bourgouin PM, Chalk C, Richardson J, Duang H, Vezina JL (August 1995). "Subcortical white matter lesions in osmotic demyelination syndrome". AJNR. American Journal of Neuroradiology. 16 (7): 1495–1497. PMC 8338057 . PMID 7484639.]
- 1 2 Graham KC, Spiegel DR (2008). "Pseudobulbar palsy and affect in a case of progressive multifocal leukoencephalopathy". The Journal of Neuropsychiatry and Clinical Neurosciences. 20 (1): 110–111. doi:10.1176/jnp.2008.20.1.110. PMID 18305298.
- ↑ Okun MS, Raju DV, Walter BL, Juncos JL, DeLong MR, Heilman K, et al. (June 2004). "Pseudobulbar crying induced by stimulation in the region of the subthalamic nucleus". Journal of Neurology, Neurosurgery, and Psychiatry. 75 (6): 921–923. doi:10.1136/jnnp.2003.016485. PMC 1739063 . PMID 15146017.
- ↑ Asfora WT, DeSalles AA, Abe M, Kjellberg RN (April 1989). "Is the syndrome of pathological laughing and crying a manifestation of pseudobulbar palsy?". Journal of Neurology, Neurosurgery, and Psychiatry. 52 (4): 523–525. doi:10.1136/jnnp.52.4.523. PMC 1032309 . PMID 2738597.