Pseudobulbar palsy

From Wikipedia, the free encyclopedia - View original article

Pseudobulbar palsy
Classification and external resources
ICD-9335.23
DiseasesDB10826
MeSHD020828
 
Jump to: navigation, search
Pseudobulbar palsy
Classification and external resources
ICD-9335.23
DiseasesDB10826
MeSHD020828

Pseudobulbar palsy results from an upper motor neuron lesion to the corticobulbar pathways in the pyramidal tract. Patients have difficulty chewing, swallowing and demonstrate slurred speech (often initial presentation). Individuals with pseudobulbar palsy also demonstrate inappropriate emotional outbursts.

Pseudobulbar Palsy is a neurological condition caused by various brain disorders that results in the inability to control facial movements such as chewing and speaking. It is a result of bilateral degeneration of the corticobulbar pathways. It is an upper neuron condition, which the conditions occur usually due to the damage in the Corticobulbar Tract (Upper Motor Neuron tract to cranial nerve motor nuclei).

Since this condition is caused by bilateral impairment in function of the brain stem upper motor nuclei, weakness or paralysis of muscles supplied by the lower cranial nerves such as glossopharyngeal, vagus, accessory and hypoglossal occurs.[1] Patients would be involved with problems with muscle for mastication and facial expression. Also, they would demonstrate increase reflexes and spasticity of muscle such as increase in GAG reflex and jaw jerk reflex, spasticity in tongue and speech muscles.[2]

Contents

Causes

Pseudobulbar Palsy is caused by brain diseases that affect the motor fibers traveling from the cerebral cortex to the lower Brain Stem, which could be cause by conditions such as demyelination and bilateral corticobulbar lesions. Mechanisms that can explain Pseudobulbar Palsy are disinhibition of the motor neurons controlling laughter and crying and that a reciprocal pathway exists between the cerebellum and the brainstem that adjusts laughter and crying responses, making them appropriate to context.[3] The pseudobulbar crying could also be induced by stimulation in the region of the subthalamic nucleus of the brain.[4]

Symptoms

These include:

Signs

These include:

Bulbar palsy is a similar disorder but is caused by lower motor neuron lesions

Symptoms and Conditions by specific cases

There are general symptoms, signs, and conditions that occur in every case of Pseudolbular Palsy such as Dysarthria and limitation of tongue movement and drooling. However, there are different types of Pseudobulbar Palsy conditions that occur with different brain disorders. The followings are a few brain diseases that were researched and were described to cause Pseudobulbar Palsy symptoms and conditions.

Creutzfeldt–Jakob disease[7]

This was a case study of a women patient diagnosed with Creutzfeldt-Jakob disease. From MRI, the patients showed moderate diffuse spongiosis, as well as gliosis and neuronal loss of the cerebral cortex, basal ganglia, and thalamus.

Symptoms and conditions:

Bilateral Perisylvian Ulegyria[8]

Ulegyria is considered to be an important perinatal or postnatal structural abnormality, which can explain the etiological heterogeneity for Pseudobulbar Palsy, which results from bilateral perisylvian lesions. Histological criteria to diagnose ulegyria include groups of atrophic gyri, loss of the cortical tissue in the depth of the sulci and destruction and gliosis of subcortical white matter.

In this experimental study, 7 patients with the disorder of Bilateral Perisylvian Ulegyria were examined and divided into 3 levels of Pseudobulbar Palsy of mild, moderate, and severe. They were divided depending on the degree of dysarthria and restriction in the tongue movement. Surgical resection was performed in each patient and the samples were clinically examined. As a result, this study showed that ulegyria could be an important pathologic abnormality that explains Pseudobulbar Palsy. The treatment in this case was suggested to be surgical resection in certain selected cases.

Symptoms and conditions

Congenital Bilateral Perisylvian Syndrome (case study of infant)[9]

These patients were diagnosed with brain image by MRI. Their dystocic birth delivery had caused perinatal asphyxia. Proposed treatment for this brain disorder of phenobarbital, carbamazepine, lamotrigine, and gabapentin were found to be noneffective. However, Monotherapy with phenytoin (PHT), then topiramate (TPM) was found to be effective which cause the patients to gain control of seizure. Symptoms and conditions

Congenital Bilateral Perisylvian Polymicrogyria[10]

These patients were also diagnosed with brain image by MRI. Malformation of cortical development resulting in an irregular cortex with multiple small, partly fused gyri, divided by shallow sulci. Structural abnormality was found in these patients such as microcephaly. In addition, cerebral cortex was abnormally thickened and infolded on the borders and in the depth of the sylvian fissures and sylvian fissures were more vertically oriented and extend more posteriorly up to the parietal lobes. Treatment conducted for this brain disorder was Vigabatrine (160 mg/kg/d in 2 gifts), which was a treatment for seizures.

Symptoms and conditions

Capsular Infarction[11]

This brain disorder can be diagnosis by MRI brain imaging. Physiological changes in the brain included infarction at middle portion of right posterior limb of the internal capsule (PLIC) and left corona radiata Symptoms and conditions

Human African Trypanosomiasis[1]

Human African Trypanosomiasis is a sleeping sickness that is a parasitic tropical disease endemic to sub-Saharan Africa. This disease sometimes invade the central nervous system, and Pseudobulbar Palsy can be a consequence of this very rare medical problem. For people who have this sleeping sickness, their central nervous system could be damaged thus develop conditions of Pseudobublar Palsy. Due to the paralysis of the muscle innervated by the medulla, they may have following symptoms and conditions:

Symptoms and Conditions:

Herpes Simplex Virus(HSV) Encephalitis in AIDS[12]

HSV Encephalitis is a very common viral brain disorder of immunocompetent individuals. Favorable outcome for this disorder depends on early therapy. A patient of HIV positive was diagnosed with HSV Encephalitis. Through this brain disorder, he developed symptoms of Pseudobulbar Palsy.

Symptoms and Conditions:

Treatment: This patient was treated with 3 weeks of intravenous aciclovir therapy and 3 days of dexamethasone. The confusion subsided and the Pseudobulbar Palsy resolved completely. Thereafter, the patient recovered to normal speech, strong gag reflex, and resumed eating and drinking. Before this case study, resolving of Pseudobublar Palsy had never been reported before. Therefore intravenous aciclovir therapy and dexamethasone could be potential treatments.

Intrathecal methotrexate injections[13]

Intrathecal Methotrexate injections was used to treat a patient with acute lymphocytic leukaemia. However, methotrexate had been reported to induce neurotoxicity, and it could be acute or chronic. In this case, it was shown that methotrexate caused Pseudobublar Palsy in a dose dependent manner. This patient acquired signs of Pseudobulbar Palsy after his second weekly injection.

Symptoms and Conditions:

Diagnosis: Examination of cerebrospinal fluid revealed a mild lymphocytosis of 8 cells/cmm, which was most likely to be n acute inflammatory neuropathy.

Treatment: In this case, the patient was commenced on IVIG (intravenous immunoglobulin). 36 hours later, the patient returned normal from Pseudobublar Palsy conditions.

ALS with Pseudobulbar Palsy[14]

In this study, both patients who are diagnosed with only ALS(Amyotrophic lateral sclerosis) and patients with ALS with Pseudobulbar Palsy were experimented to their severity of cognitive impairment. The results showed that patients with Pseudobulbar palsy had more severe cognitive impairment. The tests included written verbal fluency test and random movement joystick tasks test. For written verbal fluency test, it is a test depending on intrinsic response initiation. Therefore the results showed that Pseudobulbar Palsy patients tended to require longer thinking times to generate words. As for random movement joystick tasks, this test has been associated with bilateral activation of the DLPFC (dorsolateral prefrontal cortex). Therefore, Pseudobublar Palsy patients demonstrated a shorter planning time, which the patients seemed to be more “impulsive”.

For these patients to require longer thinking times to generate words and shorter planning time, it was postulated that the damages in the brain should be in the left prefrontal cortex, which controls these physiological functions and seems to be causing these cognitive impairments.

Multifocal Leukoencephalopathy[3]

A patient with Multifocal Leukoencephalopthy was examined, and the patient demonstrated signs of Pseudobulbar palsy as follows:

Symptoms and Conditions:

Diagnosis: MRI showed subcortical white matter, brainstem, and cerebellum had abnormality. Most likely diagnosis was postviral related demyelination due progressive multifocal leukoencephalopathy.

Proposed mechanisms for cause of Psuedobublar Palsy from Multifocal Leukoencaphalopathy:

Pharmacologic interventions for pseudobulbar affect include the tricyclic antidepressants, serotonin reuptake inhibitors and a novel approach utilizing dextromethorphan and quinidine sulfate. Dextromethorphan, an N-methyl-D-aspartate receptor antagonist, inhibits glutamatergic transmission in the regions of the brainstem and cerebellum, which are hypothesized to be involved in pseudobulbar symptoms. Dextromethorphan acts as a sigma ligand, binding to the sigma- 1 receptors that mediate the emotional motor expression.

Diagnosis

Pseudobulbar Palsy is a syndrome caused by diseases that affect the motor fibers that travel from the cerebral cortex to the lower brain stem. Tests such as examining any increase in Jaw Jerk and Gag Reflex are ways to detect the occurrence of Pseudobulbar Palsy. Patients who have this condition also demonstrate tongue spasticity, absence of palatal movement, and inappropriate emotional outburst. Most of the time, majority of patients with pathological laughter and crying have pseudobulbar palsy due to bilateral corticobulbar lesions and often a bipyrimidal involvement of arms and legs.[15] To further confirm the condition, MRI can be performed to define the areas of brain abnormality.

Treatment

Currently there are not any definite and specific treatment for Pseudobulbar Palsy as it is an occurring condition or side effect from other brain disorders. Therefore, treating for that specific brain disease may eventually resolve the symptoms of Pseudobulbar Palsy. Possible pharmacological interventions for pseudobulbar affect include the tricyclic antidepressants, serotonin reuptake inhibitors, and a novel approach utilizing dextromethorphan and quinidine sulfate. Dextromethorphan, an N-methyl-D-aspartate receptor antagonist, inhibits glutamatergic transmission in the regions of the brainstem and cerebellum, which are hypothesized to be involved in pseudobulbar symptoms. Dextromethorphan acts as a sigma ligand, binding to the sigma-1 receptors that mediate the emotional motor expression.[3]

Prospective Research

Since Pseudobulbar Palsy is a condition caused by various forms of brain diseases instead of being a disease itself, currently it can only be researched through clinical trials and studies as the clinical and research foundations admit patients demonstrating Pseudobulbar Palsy related symptoms and conditions.

See also

References

  1. ^ a b Frost, L. “Dental management of the tropical disease human African trypanosomiasis: an unusual case of pseudobulbar palsy”. british dental journal. volume 210 No. 1 Jan 8 2011.
  2. ^ https://www.youtube.com/watch?v=Up-oOhRO1N0
  3. ^ a b c Graham, K., Spiegel, D. "Pseudobulbar Palsy and Affect in a Case of Progressive Multifocal Leukoencephalopathy" J Neuropsychiatry Clin Neurosci 20:1, Winter 2008
  4. ^ Okun, M., Raju, D., Walter, B., Juncos, J., DeLong, M., Heilman, K., McDonald, W., Vitek, J. "Pseudobulbar crying induced by stimulation in the region of the subthalamic nucleus". J Neurol Neurosurg Psychiatry 2004;75:921–923.
  5. ^ Bourgouin PM, Chalk C, Richardson J, Duang H, Vezina JL (Aug 1995). "Subcortical white matter lesions in osmotic demyelination syndrome". American Journal of Neuroradiology 16 (7): 1495–7. PMID 7484639.]
  6. ^ McCormick WE, Lee JH (May 2002). "Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases". Skull Base 12 (2): 067–072. doi:10.1055/s-2002-31568-1. PMC 1656925. PMID 17167648. //www.ncbi.nlm.nih.gov/pmc/articles/PMC1656925/.
  7. ^ H.-G. Frank a, H. Schnorf a, D. Genoud a, P. Pizzolato b, M. Glatzel c, T. Landis a. "Creutzfeldt-Jakob Disease Presenting as Isolated Dysarthria and Dysphagia due to Pseudobulbar Palsy". Eur Neurol 2000;44:126–127
  8. ^ Hyoung-Ihl Kim, Min-Cheol Lee, Ji-Shin Lee, Hyung-Seok Kim, Myeong-Kyu Kim, Young-Jong Woo, Jae-Hyoo Kim, Shin Jung, Andre Palmini and Seung U. Kim. "Bilateral perisylvian ulegyria: Clinicopathological study of patients presenting with pseudobulbar palsy and epilepsy". Neuropathology 2006; 26, 236–242
  9. ^ Lucia, M., Anna, P., Patrizia, V., Maura, B., Cosma, A., Tommaso, P. Congeital bilateral perisylvian syndrome with partial epilepsy. Case report with long-term follow-up. Brain & Development 27. 2005. 53-57.
  10. ^ Coene, A., Coster, R., Verhelst, H. Perisylvian polymicrogyria, infantile spasms and arthrogyrposis: The severe end of the spectrum of congenital bilateral perisylvian polymicrogyria. European Journal of Paediatric Neurology 14. 2010; 270-273.
  11. ^ Maeda, K., Idehara, R., Shiraishi T. Pure Pseudobulbar Palsy due to a Capsular Infarction. Internal Medicine 51: 993. 2012.
  12. ^ Kulasegaram, R., Kapur, N., Bingham, J., Rodgers, C. “Pseudobulbar palsy recovery following HSV encephalitis in AIDS”. International Journal of STD & AIDS 2000; 11: 201-202
  13. ^ Kinirons, P., Fortune, A., Enright, H., Murphy, R. “Acute pseudobulbar palsy due to methotrexate with rapid response to intravenous immunoglobulin”. J Neurol (2005) 252 : 1401–1403.
  14. ^ Abrahams, S., Goldstein, L., Al-Chalabi, A., Pickering, A., Morris, R., Passingham, R., Brooks, D., Leigh, P. “Relation between cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral sclerosis”. Journal of Neurology, Neurosurgery, and Psychiatry 1997; 62:464-472.
  15. ^ Asfora, W., Desalles, A., ABE, M., Kjellberg, R. "Is the syndrome of pathological laughing and crying a manifestation of pseudobulbar palsy?" Journal of Neurology, Neurosurgery, and Psychiatry 1989;52:523-525

External links