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Surgical Neurology Oct 1997Papillary tumors of the temporal bone are very rare but aggressive neoplasms. In the past, a middle-ear origin was presumed. Only recently convincing evidence exists... (Review)
Review
BACKGROUND
Papillary tumors of the temporal bone are very rare but aggressive neoplasms. In the past, a middle-ear origin was presumed. Only recently convincing evidence exists that these tumors in fact arise from the endolymphatic sac.
METHODS
We present a case of an endolymphatic sac tumor (ELST) with detailed clinical, imaging, operative, and pathologic data. The literature on this rare tumor type is reviewed.
RESULTS
This 63-year-old woman had a progressive mass lesion in the temporal bone for a period of more than 35 years, resulting in unilateral fifth to eleventh cranial nerve palsy, progressive ataxia, and a pyramidal and pseudobulbar syndrome. Computerized tomography (CT) and magnetic resonance imaging (MRI) showed a tumor invading the pars squamosa and petrosa of the temporal bone, and extending into the middle and posterior fossa. Angiography demonstrated a hypervascular tumor mass. The patient underwent surgery, with nonradical removal of a tumor. Histologic examination demonstrated a papillary ELST. A search through the literature revealed 36 patients with ELST, based on convincing anatomic and histologic considerations.
CONCLUSIONS
It is important to make a distinction between ELST and the more benign middle-ear adenomas, since this leads to a different treatment and prognosis. ELST frequently invades the surrounding structures and extends intracranially. The treatment of choice is a radical resection, although complete resection is impossible in most of the cases. The value of adjunctive radiation therapy remains controversial.
Topics: Adenoma; Carotid Artery, Internal; Cerebral Angiography; Craniopharyngioma; Ear Neoplasms; Endolymphatic Sac; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Middle Aged; Neoplasm Invasiveness; Skull Neoplasms; Temporal Bone; Tomography, X-Ray Computed
PubMed: 9315135
DOI: 10.1016/s0090-3019(96)00553-8 -
Journal of Neurosciences in Rural... Jan 2014Neurocysticercosis (NCC) is the most common helminthic infestation of the central nervous system (CNS) and a leading cause of acquired epilepsy worldwide. The common...
Neurocysticercosis (NCC) is the most common helminthic infestation of the central nervous system (CNS) and a leading cause of acquired epilepsy worldwide. The common manifestations of NCC are seizures and headache. The NCC as a cause of pseudobulbar palsy is very unusual and not reported yet in the literature. A pseudobulbar palsy can occur in any disorder that causes bilateral corticobulbar disease. The common etiologies of pseudobulbar palsy are vascular, demyelinative, or motor neuron disease. We report a 38-year-old female patient who presented with partial seizures and pseudobulbar palsy. The MRI brain showed multiple small cysts with scolex in both the cerebral hemispheres and a giant intraparenchymal cyst. Our patient responded well to standard treatment of neurocysticercosis and antiepileptics.
PubMed: 24741260
DOI: 10.4103/0976-3147.127883 -
The Tohoku Journal of Experimental... Mar 2023Pseudobulbar palsy and bulbar palsy cause dysphagia in patients with amyotrophic lateral sclerosis (ALS). Dysphagia in patients with ALS not only increases the risk of...
Pseudobulbar palsy and bulbar palsy cause dysphagia in patients with amyotrophic lateral sclerosis (ALS). Dysphagia in patients with ALS not only increases the risk of aspiration and pneumonia but also leads to malnutrition and weight loss, which are poor prognostic factors. Gastrostomy is the preferred route of feeding and nutritional support in patients with dysphagia. However, there are no established standards to determine the ideal timing of gastrostomy for patients with ALS. Therefore, we used the videofluoroscopic dysphagia scale (VDS), which objectively quantifies swallowing function, in videofluoroscopic swallowing study (VFSS) to investigate whether this scale at diagnosis can be a useful predictor for the timing of gastrostomy. We retrospectively evaluated 22 patients with ALS who were diagnosed at our hospital. We assessed the VDS scores in all patients within 3 months of diagnosis. A decline in the ALS functional rating scale revised (ALSFRS-R) scores was used as an indicator of disease progression. As a result, we found that the VDS score of the pharyngeal phase and the total VDS score were significantly correlated with the ΔALSFRS-R scores. These scores were also associated with the existing indicators for the timing of gastrostomy, i.e., decreased body weight and percent-predicted forced vital capacity. We demonstrated the noninferiority of the VDS scores relative to the existing indicators. In addition, the VDS score of the pharyngeal phase was significantly correlated with the time from diagnosis to gastrostomy. The VDS score could estimate the timing of gastrostomy in patients with ALS with dysphagia at diagnosis.
Topics: Humans; Deglutition Disorders; Amyotrophic Lateral Sclerosis; Gastrostomy; Retrospective Studies; Deglutition
PubMed: 36696983
DOI: 10.1620/tjem.2023.J005 -
Psychiatry (Edgmont (Pa. : Township)) May 2010This article concludes the series on cranial nerves, with review of the final four (IX-XII). To summarize briefly, the most important and common syndrome caused by a...
This article concludes the series on cranial nerves, with review of the final four (IX-XII). To summarize briefly, the most important and common syndrome caused by a disorder of the glossopharyngeal nerve (craniel nerve IX) is glossopharyngeal neuralgia. Also, swallowing function occasionally is compromised in a rare but disabling form of tardive dyskinesia called tardive dystonia, because the upper motor portion of the glossopharyngel nerve projects to the basal ganglia and can be affected by lesions in the basal ganglia. Vagus nerve funtion (craniel nerve X) can be compromised in schizophrenia, bulimia, obesity, and major depression. A cervical lesion to the nerve roots of the spinal accessory nerve (craniel nerve XI) can cause a cervical dystonia, which sometimes is misdiagnosed as a dyskinesia related to neuroleptic use. Finally, unilateral hypoglossal (craniel nerve XII) nerve palsy is one of the most common mononeuropathies caused by brain metastases. Supranuclear lesions of cranial nerve XII are involved in pseudobulbar palsy and ALS, and lower motor neuron lesions of cranial nerve XII can also be present in bulbar palsy and in ALS patients who also have lower motor neuron involvement. This article reviews these and other syndromes related to cranial nerves IX through XII that might be seen by psychiatry.
PubMed: 20532157
DOI: No ID Found -
Case Reports in Critical Care 2024Traumatic brain injury (TBI) can have profound acute and chronic effects, leading to permanent disabilities and diminished quality of life. Pseudobulbar palsy and its...
Traumatic brain injury (TBI) can have profound acute and chronic effects, leading to permanent disabilities and diminished quality of life. Pseudobulbar palsy and its infrequent subtype, Foix-Chavany-Marie Syndrome (FCMS), represent rare complications of TBI, manifesting as deficits in craniofacial motor function and automatic-voluntary dissociation. We present a case of a 58-year-old male who developed FCMS following severe TBI from a cycling accident. Initial imaging revealed extensive brain injury with subsequent development of FCMS characterised by bilateral cranial nerve dysfunction, notably facio-pharyngo-glosso-masticatory diplegia with preserved automatic motor function. This case contributes to the limited literature on traumatic FCMS, highlighting its distinct clinical features and potential for favourable outcomes compared to nontraumatic cases. Early recognition and comprehensive management, including supportive therapy and addressing underlying conditions, are paramount for optimising patient outcomes.
PubMed: 38766550
DOI: 10.1155/2024/6652867 -
Emotional Incontinence: A Case Report of Pseudobulbar Affect in the Setting of Alcohol Use Disorder.Cureus May 2023Pseudobulbar affect (PBA) manifests as a disconnect between emotional feelings and emotional expressions. The impact of pseudobulbar affect on social, occupational, and...
Pseudobulbar affect (PBA) manifests as a disconnect between emotional feelings and emotional expressions. The impact of pseudobulbar affect on social, occupational, and interpersonal functioning is substantial. It results in poor quality of social interactions and poor overall quality of life. Instances of pseudobulbar affect with no underlying neuropsychiatric disorders are rarely reported in the literature. Although alcohol use has been associated with traumatic brain injuries (TBI), alcohol as a direct cause of pseudobulbar palsy has rarely been reported. Our case presents a unique situation with no known underlying primary neurologic disorder but evidence from clinical history, physical examination, and laboratory tests indicative of severe alcohol use disorder. This case represents the rare instances where the disease etiology is unusual and reminds the health care provider to consider the role of alcohol in the pathophysiology of pseudobulbar affect. More research is needed to understand the role of alcohol in the etiology of pseudobulbar affect in the absence of any known underlying neuropsychiatric disorder.
PubMed: 37313061
DOI: 10.7759/cureus.38976 -
BMC Neurology Mar 2019We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite...
BACKGROUND
We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP).
METHODS
Pyramidal motor system pathologies were analyzed in 18 cases with neuropathologically confirmed PSP. Based on a retrospective clinical analysis, cases were subtyped according to Movement Disorder Society criteria for clinical diagnosis of PSP as probable, possible or suggestive of PSP with Richardson's syndrome (n = 10), PSP with predominant corticobasal syndrome (n = 3), PSP with predominant parkinsonism (n = 3), PSP with predominant speech/language disorder (n = 1), and PSP with progressive gait freezing (n = 1). Clinical manifestations of motor neuron involvement (pseudobulbar or bulbar signs and spasticity) were retrospectively assessed semiquantitatively. Neuropathologically, hyperphosphorylated tau-related pyramidal motor system neuronal, neuritic, and glial pathology using anti-tau AT8 clone immunohistochemistry, was also evaluated.
RESULTS
Clinical manifestations of pyramidal motor system involvement were found in patients with different PSP subtypes. A statistically significant higher load of tau pathology was found in the pyramidal system in PSP-Richardson's syndrome compared to other PSP subtypes (p = 0.016); however, there was no significant correlation between pyramidal system tau pathology and related motor clinical symptoms.
CONCLUSIONS
Tau pathology in the spinal cord and pyramidal motor system structures is very common in progressive supranuclear palsy and may neuropathologically supplement the distinction between classic Richardson's syndrome from other progressive supranuclear palsy subtypes.
Topics: Aged; Aged, 80 and over; Cerebral Cortex; Female; Humans; Immunohistochemistry; Male; Middle Aged; Movement Disorders; Parkinsonian Disorders; Pyramidal Tracts; Retrospective Studies; Supranuclear Palsy, Progressive; tau Proteins
PubMed: 30894142
DOI: 10.1186/s12883-019-1270-1 -
BMC Neurology Apr 2010Alexander disease (ALX) is a rare neurological disorder characterized by white matter degeneration and cytoplasmic inclusions in astrocytes called Rosenthal fibers,... (Review)
Review
BACKGROUND
Alexander disease (ALX) is a rare neurological disorder characterized by white matter degeneration and cytoplasmic inclusions in astrocytes called Rosenthal fibers, labeled by antibodies against glial fibrillary acidic protein (GFAP). Three subtypes are distinguished according to age at onset: infantile (under age 2), juvenile (age 2 to 12) and adult (over age 12). Following the identification of heterozygous mutations in GFAP that cause this disease, cases of adult-onset ALX have been increasingly reported.
CASE PRESENTATION
We present a 60-year-old Japanese man with an unremarkable past and no family history of ALX. After head trauma in a traffic accident at the age of 46, his character changed, and dementia and dysarthria developed, but he remained independent. Spastic paresis and dysphagia were observed at age 57 and 59, respectively, and worsened progressively. Neurological examination at the age of 60 revealed dementia, pseudobulbar palsy, left-side predominant spastic tetraparesis, axial rigidity, bradykinesia and gaze-evoked nystagmus. Brain MRI showed tadpole-like atrophy of the brainstem, caused by marked atrophy of the medulla oblongata, cervical spinal cord and midbrain tegmentum, with an intact pontine base. Analysis of the GFAP gene revealed a heterozygous missense mutation, c.827G>T, p.R276L, which was already shown to be pathogenic in a case of pathologically proven hereditary adult-onset ALX.
CONCLUSION
The typical tadpole-like appearance of the brainstem is strongly suggestive of adult-onset ALX, and should lead to a genetic investigation of the GFAP gene. The unusual feature of this patient is the symmetrical involvement of the basal ganglia, which is rarely observed in the adult form of the disease. More patients must be examined to confirm, clinically and neuroradiologically, extrapyramidal involvement of the basal ganglia in adult-onset ALX.
Topics: Alexander Disease; Atrophy; Basal Ganglia; Brain Stem; Functional Laterality; Humans; Japan; Magnetic Resonance Imaging; Male; Middle Aged
PubMed: 20359319
DOI: 10.1186/1471-2377-10-21 -
Brain & NeuroRehabilitation Jul 2021Bilateral cerebral peduncular infarction (BCPI) is a very rare disorder among stroke patients. The main clinical manifestations in the previously reported BCPI case...
Bilateral cerebral peduncular infarction (BCPI) is a very rare disorder among stroke patients. The main clinical manifestations in the previously reported BCPI case reports was associated with locked-in syndrome or persistent vegetative state. Here, we present a 51-year-old woman who had pseudobulbar palsy and quadriplegia. Magnetic resonance imaging showed an acute infarction in the middle areas of the cerebral peduncle with a unique "Mickey Mouse ears" sign. Diffusion tensor imaging and tractography showed relatively preserved corticospinal tracts, but the corticobulbar tracts were not detected. Magnetic resonance angiography showed posterior cerebral artery and vertebrobasilar artery occlusion. Cerebral perfusion insufficiency due to stenosis or occlusion of the vertebrobasilar artery and its branches may lead to BCPI. The prognosis and clinical manifestations of BCPI are related to the extent of the infarction in the involved cerebral peduncle and whether other territories are involved. Isolated BCPI may present a severe pseudobulbar palsy with relatively preserved limb function depending on the involvement pattern.
PubMed: 36743434
DOI: 10.12786/bn.2021.14.e16 -
Behavioural Neurology 1992The clinical and neuroradiological findings of three patients with marked palilalia due to different neurological disorders are reported. These cases and others in the...
The clinical and neuroradiological findings of three patients with marked palilalia due to different neurological disorders are reported. These cases and others in the literature suggest the possibility of different variations. "Spasmodic, heterophonic palilalia" is typically observed in Parkinson's syndrome and pseudobulbar palsy: the content of palilalia is characteristically changed by interruption. "Atonic, homophonic, autoecholalic palilalia" is mainly seen in Pick's disease, and is not affected by external interruption.
PubMed: 24487810
DOI: 10.3233/BEN-1992-5407