WordNet

a condition marked by uncontrollable tremor
affect with palsy
become friends; act friendly towards (同)pal up, chum up
a solid bulblike enlargement of the stem of some orchids

PrepTutorEJDIC

麻痺(まひ),中風 / …‘を'麻痺させる(paralyze)
《話》『友達』,仲間,親友 / (…と)仲よくなる《[『up』]『with』+『名』》

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/08/15 18:47:44」(JST)

wiki en

Pseudobulbar palsy is a medical condition characterised by the inability to control facial movements (such as chewing and speaking) and caused by a variety of neurological disorders. Patients experience difficulty chewing and swallowing, have increased reflexes and spasticity in tongue and the bulbar region, and demonstrate slurred speech (which is often the initial presentation of the disorder), sometimes also demonstrating uncontrolled emotional outbursts.

The condition is usually caused by the damage (bilateral degeneration) to the neurons of the brain stem, specifically to the corticobulbar tract (upper motor neuron tract to cranial nerve motor nuclei).

Causes

Pseudobulbar palsy is the result of damage of motor fibers traveling from the cerebral cortex to the lower brain stem. This damage might arise in the course of a variety of neurological conditions that involve demyelination and bilateral corticobulbar lesions. Examples include:

Vascular causes: bilateral hemisphere infarction, CADASIL syndrome
Progressive supranuclear palsy
Amyotrophic lateral sclerosis
Parkinson's disease and related multiple system atrophy
Various motor neuron diseases, especially those involving demyelination
Multiple sclerosis and other inflammatory disorders
High brain stem tumors
Metabolic causes: osmotic demyelination syndrome^[1]
Neurological involvement in Behçet's disease
Brain trauma

Pathophysiology

The proposed mechanism of pseudobulbar palsy points to the disinhibition of the motor neurons controlling laughter and crying, proposing that a reciprocal pathway exists between the cerebellum and the brain stem that adjusts laughter and crying responses, making them appropriate to context.^[2] The pseudobulbar crying could also be induced by stimulation in the region of the subthalamic nucleus of the brain.^[3]

Signs and symptoms

Signs and symptoms of pseudobulbar palsy include:

Slow and indistinct speech
Dysphagia (difficulty in swallowing)
Small, stiff and spastic tongue
Brisk jaw jerk
Dysarthria
Labile affect^[4]
Gag reflex may be normal, exaggerated or absent
Examination may reveal upper motor neuron lesion of the limbs

Diagnosis

Diagnosis of pseudobulbar palsy is based on observation of the symptoms of the condition. Tests examining jaw jerk and gag reflex can also be performed. It has been suggested that the 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.^[5] To further confirm the condition, MRI can be performed to define the areas of brain abnormality.

Treatment

Since pseudobulbar palsy is a syndrome associated with other diseases, treating the underlying disease may eventually reduce 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. Nuedexta is an FDA approved medication for pseudobulbar affect. 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, and acts as a sigma ligand, binding to the sigma-1 receptors that mediate the emotional motor expression.^[2]

References

^ 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. ]
^ ^a ^b Graham, K., Spiegel, D. "Pseudobulbar Palsy and Affect in a Case of Progressive Multifocal Leukoencephalopathy" J Neuropsychiatry Clin Neurosci 20:1, Winter 2008
^ 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.
^ 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.
^ 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

248512525 at GPnotebook - "pseudobulbar palsy"
456458269 at GPnotebook - "comparison of bulbar and pseudobulbar palsy"
eNotes

UpToDate Contents

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1. 進行性核上性麻痺（PSP） progressive supranuclear palsy psp
2. 成人における詳細な神経学的検査 the detailed neurologic examination in adults
3. 筋萎縮性側索硬化症の症状に基づくマネージメント symptom based management of amyotrophic lateral sclerosis
4. ラクナ梗塞 lacunar infarcts
5. 成人における精神状態検査 the mental status examination in adults

English Journal

Frontotemporal lobar degeneration with writing disturbance mainly consisting of omission of kana letters.

Shimizu T1, Mochizuki Y, Bandoh M, Taguchi T, Mishima K, Takeda K, Nojima K, Mizutani T, Matsubara S.Author information 1a Department of Neurology , Tokyo Metropolitan Neurological Hospital , Tokyo , Japan.AbstractA right-handed woman developed pseudobulbar palsy and a particular writing disturbance mainly composed of omission of kana letters (OKL) at the age of 79, followed by gradual progression of generalized motor disturbance and mutism. She died at the age of 88. Postmortem examination revealed frontotemporal lobar degeneration. The precentral cortex and premotor area were the most severely degenerated among the affected frontal, parietal, and temporal lobes. The omission of kana letters has been recently reported as a characteristic feature of writing disturbance in Japanese amyotrophic lateral sclerosis (ALS). Our case indicates that OKL is not specific to ALS, and that the prefrontal and precentral cortices, common lesions between our case and ALS, are responsible for OKL. This case also shows that OKL can be caused by a pathomechanism independent from other types of writing error. The neurolinguistic analysis of our case suggests the disturbance of the moraic frame of words in the transcription process of morae into kana letters or kana-letter cards.
Neurocase.Neurocase.2014 Jun;20(3):355-60. doi: 10.1080/13554794.2013.791860. Epub 2013 May 16.
A right-handed woman developed pseudobulbar palsy and a particular writing disturbance mainly composed of omission of kana letters (OKL) at the age of 79, followed by gradual progression of generalized motor disturbance and mutism. She died at the age of 88. Postmortem examination revealed frontotem
PMID 23679336

Epileptic Seizure as a Precipitating Factor of Vascular Progressive Supranuclear Palsy: A Case Report.

Lanza G1, Papotto M2, Pennisi G3, Bella R3, Ferri R4.Author information 1Department of Neurology I.C., Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina (EN), Italy. Electronic address: giuseppelanza2003@yahoo.it.2Department of Neurorehabilitation, Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina (EN), Italy.3Department of Neurosciences, University of Catania, Catania, Italy.4Department of Neurology I.C., Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina (EN), Italy.AbstractBACKGROUND: Vascular progressive supranuclear palsy (vPSP) is an uncommon akinetic-rigid syndrome characterized by asymmetric lower body involvement, predominant corticospinal and pseudobulbar signs, urinary incontinence, cognitive impairment, and increased frequency of stroke risk factors, together with neuroimaging evidence of vascular disease.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association.J Stroke Cerebrovasc Dis.2014 Mar 18. pii: S1052-3057(13)00560-0. doi: 10.1016/j.jstrokecerebrovasdis.2013.12.043. [Epub ahead of print]
BACKGROUND: Vascular progressive supranuclear palsy (vPSP) is an uncommon akinetic-rigid syndrome characterized by asymmetric lower body involvement, predominant corticospinal and pseudobulbar signs, urinary incontinence, cognitive impairment, and increased frequency of stroke risk factors, together
PMID 24656241

Japanese Journal

急性期脳梗塞による嚥下障害における改訂水飲みテストと1％とろみつき水飲みテストの併用法の有用性について

脳卒中 advpub(0), 2016
NAID 130005147209

The sensory nerves that innervate the area near the K-point

看護科学研究 13(2), 30-34, 2015-07
NAID 110009921787

発声困難な脳卒中後重症仮性球麻痺患者4 例の摂食・嚥下機能予後と開口障害について

脳卒中 advpub(0), 2015
NAID 130005122378

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★リンクテーブル★

リンク元	「仮性球麻痺」「pseudobulbar syndrome」
関連記事	「palsy」

「仮性球麻痺」

Pseudobulbar palsy
Classification and external resources
ICD-9-CM	335.23
DiseasesDB	10826
MeSH	D020828

　　[★]

英: pseudobulbar palsy
同: 偽性球麻痺
関: 球麻痺

[show details]

偽性球麻痺 : 98 件
仮性球麻痺 : 約 16,900 件

	球麻痺(bulbar palsy)	偽性球麻痺(pseudobulbar palsy)
障害部位	脳幹の運動性脳神経核以下の障害	両側の核上性の障害
障害部位	下位運動ニューロン障害	上位運動ニューロン障害
筋萎縮(舌)	あり	なし
舌の線維束性収縮	あり	なし
下顎反射	低下	亢進
咽頭反射	消失	亢進が多い
軟口蓋反射	消失はまれ	消失
代表的疾患	筋萎縮性側索硬化症(ALS)	両側の大脳・脳幹の血管疾患