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European Neurology 2011Midbrain atrophy is a well-known feature of progressive supranuclear palsy (PSP). Some clinical features of vascular parkinsonism (VP) such as pseudobulbar phenomena,...
BACKGROUND
Midbrain atrophy is a well-known feature of progressive supranuclear palsy (PSP). Some clinical features of vascular parkinsonism (VP) such as pseudobulbar phenomena, lower body predominance and early postural instability suggest that the brainstem could be associated with VP. The aim of this study was to determine whether midbrain atrophy was present in patients with VP.
METHODS
We measured the midbrain (Amd) and pons area (Apn) of 20 patients with VP, 15 patients with probable PSP and 30 patients with idiopathic Parkinson's disease (IPD). The Amd and Apn were measured on mid-sagittal T(1)-weighted MRI scans using a computerized image analysis system.
RESULTS
For the Amd, the patients with VP (99.86 mm(2)) and PSP (87.30 mm(2)) had significantly smaller areas than the patients with IPD (130.52 mm(2)). For the Apn, there was a significant difference only between the VP (407.23 mm(2)) and the IPD (445.05 mm(2)) patients. The Amd/Apn ratios of the patients with VP (0.245) and PSP (0.208) were significantly smaller than in the patients with IPD (0.292).
CONCLUSIONS
Our study shows that brainstem atrophy often occurs in patients with VP and the midbrain is more vulnerable than the pons to atrophic changes.
Topics: Aged; Analysis of Variance; Atrophy; Female; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Mesencephalon; Middle Aged; Parkinsonian Disorders; Pons; Retrospective Studies; Supranuclear Palsy, Progressive
PubMed: 21508644
DOI: 10.1159/000326907 -
Case Reports in Medicine 2013Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a...
Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome.
PubMed: 23476665
DOI: 10.1155/2013/513572 -
European Journal of Case Reports in... 2021Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles.
INTRODUCTION
Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles.
MATERIALS AND METHODS
A 62-year-old man was admitted to the emergency department after 9 hours of acute dysarthria and dysphagia. MRI showed restricted diffusion in the right operculum on diffusion-weighted imaging (DWI). No thrombolytic therapy was given. The patient had a history of mechanical aortic valve replacement under anticoagulation with a vitamin K antagonist. Work-up demonstrated suboptimal levels of INR. Due to severe dysphagia during hospitalization, a percutaneous endoscopic gastrostomy (PEG) was performed.
RESULTS
The patient was discharged 5 days later, with a modified Rankin scale (mRs) score of 3, and secondary stroke prevention. He had achieved an excellent functional outcome (mRs 1) at 6-month follow-up.
CONCLUSION
Our patient had a satisfactory recovery due to prompt diagnosis, secondary stroke prevention, and compliance with treatment.
LEARNING POINTS
In the presence of acute dysarthria and dysphagia, Foix-Chavany-Marie syndrome (FCMS) should be considered.FCMS may occur in the presence of unilateral opercular stroke.Swallowing and speech therapy play an essential role in rehabilitation after the acute setting.
PubMed: 33987126
DOI: 10.12890/2021_002462 -
BMJ Case Reports Oct 2021We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which...
We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which deteriorated rapidly to anarthria and aphonia within a few days. MRI scan of the head showed acute infarct in right internal capsule and an old infarct in the left corona radiata while the rest of the investigations were normal. Based on these findings, diagnosis was thought to be subopercular syndrome. He recovered significantly in a few weeks' time.
Topics: Aged; Dysarthria; Humans; Internal Capsule; Magnetic Resonance Imaging; Male; Pseudobulbar Palsy; Syndrome
PubMed: 34711624
DOI: 10.1136/bcr-2021-245613 -
Developmental Medicine and Child... Oct 1997Foix-Chavany-Marie syndrome (FCMS) is characterized by facio-linguo-masticatory diplegia in the absence of limb weakness. The most common cause is a cortical lesion...
Foix-Chavany-Marie syndrome (FCMS) is characterized by facio-linguo-masticatory diplegia in the absence of limb weakness. The most common cause is a cortical lesion resulting from a stroke but a congenital form has been reported. We present the case of a 53-year-old man who was admitted to hospital with worsening dysphagia which was know to have been present together with anarthria and facial palsy, since birth. He demonstrated features of FCMS with pseudobulbar palsy and unaffected reflexes and automatic responses. Cranial CT and MRI scans showed bilateral opercular lesions of CSF intensity in continuity with the lateral ventricles. We conclude that this case of static FCMS for over 50 years may represent a 'pure' form of congenital FCMS with motor symptomatology and unaccompanied by mental retardation or epilepsy.
Topics: Brain Diseases; Carcinoma, Squamous Cell; Cerebral Ventricles; Deglutition Disorders; Esophageal Neoplasms; Facial Paralysis; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neurologic Examination; Syndrome; Tomography, X-Ray Computed
PubMed: 9352733
DOI: 10.1111/j.1469-8749.1997.tb07366.x -
Journal of Neurology, Neurosurgery, and... May 1997To examine the relation between cognitive dysfunction and pseudobulbar features in patients with amyotrophic lateral sclerosis (ALS). (Comparative Study)
Comparative Study
OBJECTIVES
To examine the relation between cognitive dysfunction and pseudobulbar features in patients with amyotrophic lateral sclerosis (ALS).
METHODS
The performance of two patient groups, ALS with pseudobulbar palsy (n = 24) and ALS without pseudobulbar palsy (n = 28), was compared with 28 healthy age matched controls on an extensive neuropsychological battery. Tests used were the national adult reading test, short form of the WAIS-R, recognition memory test, Kendrick object learning test, paired associate learning, Wisconsin card sorting test, verbal fluency, Stroop and negative priming tests, a random movement joystick test, and a computerised Tower of Hanoi test.
RESULTS
Tests of executive function showed a pronounced deficit on written verbal fluency in both ALS groups in comparison to controls, which tended to be more prominent in patients with ALS with pseudobulbar palsy. The random movement joystick test (a non-verbal test of intrinsic movement generation) showed an impairment in the generation of random sequences in patients with pseudobulbar palsy only. The computerised Tower of Hanoi showed a subtle planning impairment (shorter planning times) in all the patients with ALS compared with controls on trials requiring more complex solutions. In addition the pseudobulbar patients displayed shorter planning times on complex trials, and tended to solve these trials less accurately. There was also evidence of a deficit for all patients with ALS in comparison with controls on total errors and number of categories achieved on the Wisconsin card sorting test and a strong tendency towards an impairment on a task of selective attention and cognitive inhibition (negative priming). A word recognition memory deficit was showed across both ALS groups.
CONCLUSIONS
This study elicited cognitive deficits (involving predominantly executive processes, with some evidence of memory impairment) in patients with ALS and further strengthened the link between ALS and frontal lobe dysfunction, this being more prominent in patients with pseudobulbar palsy. However, cognitive impairments suggestive of extramotor cortical involvement were not exclusive to this subgroup.
Topics: Adult; Amyotrophic Lateral Sclerosis; Cognition Disorders; Female; Frontal Lobe; Humans; Male; Middle Aged; Neuropsychological Tests; Paralysis; Retrospective Studies
PubMed: 9153602
DOI: 10.1136/jnnp.62.5.464 -
Annals of Neurology Sep 2015Duplication of the LMNB1 gene encoding lamin B1 causes adult-onset autosomal-dominant leukodystrophy (ADLD) starting with autonomic symptoms, which are followed by...
OBJECTIVE
Duplication of the LMNB1 gene encoding lamin B1 causes adult-onset autosomal-dominant leukodystrophy (ADLD) starting with autonomic symptoms, which are followed by pyramidal signs and ataxia. Magnetic resonance imaging (MRI) of the brain reveals characteristic findings. This is the first longitudinal study on this disease. Our objective is to describe the natural clinical and radiological course of LMNB1-related ADLD.
METHODS
Twenty-three subjects in two families with LMNB1 duplications were studied over two decades with clinical assessment and MRI of the brain and spinal cord. They were 29 to 70 years old at their first MRI. Repeated MRIs were performed in 14 subjects over a time period of up to 17 years.
RESULTS
Pathological MRI findings were found in the brain and spinal cord in all examinations (i.e., even preceding clinical symptoms). MRI changes and clinical symptoms progressed in a definite order. Autonomic dysfunction appeared in the fifth to sixth decade, preceding or together with gait and coordination difficulties. Motor signs developed ascending from spastic paraplegia to tetraplegia and pseudobulbar palsy in the seventh decade. There were clinical, radiological, and neurophysiological signs of myelopathy. Survival lasted more than two decades after clinical onset.
INTERPRETATION
LMNB1-related ADLD is a slowly progressive neurological disease. MRI abnormalities of the brain and spinal cord can precede clinical symptoms by more than a decade and are extensive in all symptomatic patients. Spinal cord involvement is a likely contributing factor to early autonomic symptoms and spastic paraplegia.
Topics: Adult; Aged; Brain; Female; Humans; Lamin Type B; Longitudinal Studies; Male; Middle Aged; Pelizaeus-Merzbacher Disease; Radiography; Spinal Cord; Survival Rate
PubMed: 26053668
DOI: 10.1002/ana.24452 -
Frontiers in Neurology 2019To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was...
To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was performed on 11 patients diagnosed with bilateral cerebral peduncular infarction in the Affiliated Hospital of Xuzhou Medical University from December 2014 to December 2018. Their clinical and imaging features were analyzed and summarized in combination with the relevant national and international literature. Among all the patients, there were eight cases with a history of hypertension, four cases with a history of diabetes mellitus, and four cases with a history of smoking. Conscious disturbance was observed in nine cases, quadriplegia in seven cases, pseudobulbar paralysis in three cases, and ataxia in one case. Brain magnetic resonance (MR) scans of bilateral cerebral peduncles showed patchy abnormal shadows with a hypointense signal on T1-weighted imaging (T1WI) and apparent diffusion coefficient (ADC) and hyperintense signal on T2-weighted imaging (T2WI), fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI). Computed tomography angiography (CTA) scans of head and neck showed severe stenosis or occlusion of vertebral artery, basilar artery, or posterior cerebral artery. All the patients received standardized treatment for cerebral infarction. Six patients died while five were left disabled. Bilateral cerebral peduncle infarction may be related to cerebral perfusion insufficiency caused by the stenosis or occlusion of vertebrobasilar artery and its branches. The main clinical manifestations are locked-in syndrome and persistent vegetative state. The specific imaging feature of "Mickey Mouse ear"-like infarction is associated with a poor prognosis.
PubMed: 31708855
DOI: 10.3389/fneur.2019.01107 -
Neural Regeneration Research Feb 2016Tongguan Liqiao acupuncture therapy has been shown to effectively treat dysphagia after stroke-based pseudobulbar paralysis. We presumed that this therapy would be...
Tongguan Liqiao acupuncture therapy has been shown to effectively treat dysphagia after stroke-based pseudobulbar paralysis. We presumed that this therapy would be effective for dysphagia after bulbar paralysis in patients with brainstem infarction. Sixty-four patients with dysphagia following brainstem infarction were recruited and divided into a medulla oblongata infarction group (n = 22), a midbrain and pons infarction group (n = 16), and a multiple cerebral infarction group (n = 26) according to their magnetic resonance imaging results. All patients received Tongguan Liqiao acupuncture for 28 days. The main acupoints were Neiguan (PC6), Renzhong (DU26), Sanyinjiao (SP6), Fengchi (GB20), Wangu (GB12), and Yifeng (SJ17). Furthermore, the posterior pharyngeal wall was pricked. Before and after treatment, patient swallowing functions were evaluated with the Kubota Water Test, Fujishima Ichiro Rating Scale, and the Standard Swallowing Assessment. The Barthel Index was also used to evaluate their quality of life. Results showed that after 28 days of treatment, scores on the Kubota Water Test and Standard Swallowing Assessment had decreased, but scores on the Fujishima Ichiro Rating Scale and Barthel Index had increased in each group. The total efficacy rate was 92.2% after treatment, and was most obvious in patients with medulla oblongata infarction (95.9%). These findings suggest that Tongguan Liqiao acupuncture therapy can repair the connection of upper motor neurons to the medulla oblongata motor nucleus, promote the recovery of brainstem infarction, and improve patient's swallowing ability and quality of life.
PubMed: 27073382
DOI: 10.4103/1673-5374.177737 -
European Journal of Neurology May 2016Familial Creutzfeldt-Jakob disease (fCJD) in Jews of Libyan ancestry is caused by an E200K mutation in the PRNP gene. The typical presenting symptoms include cognitive...
BACKGROUND AND PROPOSE
Familial Creutzfeldt-Jakob disease (fCJD) in Jews of Libyan ancestry is caused by an E200K mutation in the PRNP gene. The typical presenting symptoms include cognitive decline, behavioral changes and gait disturbances; however, some patients may have an unusual presentation such as a stroke-like presentation, alien hand syndrome or visual disturbances. The aim of this paper is to describe uncommon presentations in our series of consecutive patients with E200K fCJD.
METHODS
The study group included consecutive fCJD patients followed up as part of a longitudinal prospective study ongoing since 2003 or hospitalized since 2005. The clinical diagnosis of probable CJD was based on accepted diagnostic criteria and supported by typical magnetic resonance imaging, electroencephalographic findings, elevated cerebrospinal fluid tau protein levels and by genetic testing for the E200K mutation. Disease symptoms and signs were retrieved from the medical files.
RESULTS
The study population included 77 patients (42 men) with a mean age of disease onset of 60.6 ± 7.2 years. The most prevalent presenting symptoms were cognitive decline followed by gait impairment and behavioral changes. However, six patients had an unusual presentation including auditory agnosia, monoparesis, stroke-like presentation, facial nerve palsy, pseudobulbar syndrome and alien hand syndrome.
CONCLUSIONS
Our case series illustrates the wide phenotypic variability of the clinical presentation of patients with fCJD and widens the clinical spectrum of the disease. A high level of clinical suspicion may prove useful in obtaining early diagnosis and therefore avoiding costly and inefficient diagnostic and therapeutic strategies.
Topics: Aged; Animals; Cognition Disorders; Creutzfeldt-Jakob Syndrome; Female; Humans; Jews; Magnetic Resonance Imaging; Male; Middle Aged; Movement Disorders; Mutation; Prion Proteins; Prospective Studies; Symptom Assessment
PubMed: 26806765
DOI: 10.1111/ene.12955