-
Radiology Case Reports Jul 2024Intracranial epidermoid cysts are benign, slow-growing congenital tumors of ectodermal origin. They are rare embryonal benign cystic masses with an incidence rate of...
Intracranial epidermoid cysts are benign, slow-growing congenital tumors of ectodermal origin. They are rare embryonal benign cystic masses with an incidence rate of approximately 0.04%-0.6% of intracranial tumors. Computed tomography (CT) and magnetic resonance imaging (MRI) are fundamental diagnostic tools providing valuable information for surgical management. We reported a 59-year-old male patient with right limb weakness twelve hours prior to admission, slurred speech, and paresis of the facial nerve. Based on history taking, physical examination, and radiology examinations, we concluded a diagnosis of non-communicated hydrocephalus due to a right cerebellar intra-axial tumor with a suspicion of low-grade glioma (Pylocitic Astrocytoma). CT and MRI in intracranial epidermoid cysts are fundamental diagnostic tools for diagnosing and obtaining helpful information for surgical planning. Intracranial epidermoid cysts appear as lobulated lesions filling and expanding CSF spaces and exerting a gradual mass effect, insinuating between structures and encasing adjacent nerves and vessels. In this case, we noted a hypodense lesion with irregular calcifications and well-defined on the right cerebellar region measuring 6.15 × 5.47 × 5.7 cm, surrounded by a hypodense image suggesting an intra-axial mass suspected of low-grade glioma with a differential diagnosis of brain abscess. The hypointense lesion on the T1WI sequence found in the MRI examination, with no significant contrast enhancement and restricted diffusion area on DWI, was one of the notable features described in the epidermoid cyst. Intracranial epidermoid cyst rarely occurs in the intracranial, resulting in many symptoms in this case, which should be diagnosed and treated promptly. Imaging aids in proper diagnosis and provides more valuable information for further treatment.
PubMed: 38680739
DOI: 10.1016/j.radcr.2024.03.061 -
Cancers Apr 2024To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients.
OBJECTIVE
To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients.
STUDY DESIGN
Retrospective study.
METHOD
The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated.
RESULT
Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor ( < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold ( < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%.
CONCLUSIONS
Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature.
PubMed: 38672548
DOI: 10.3390/cancers16081466 -
Children (Basel, Switzerland) Mar 2024This paper reviews the current literature about epidemiology, etiologies, diagnosis, and management of pediatric bilateral vocal fold paralysis (PBVFP). (Review)
Review
OBJECTIVE
This paper reviews the current literature about epidemiology, etiologies, diagnosis, and management of pediatric bilateral vocal fold paralysis (PBVFP).
METHODS
According to PRISMA statements, a narrative review of the current literature was conducted through the PubMed, Scopus, and Cochrane Library databases about the epidemiology, etiologies, diagnosis, and management of PBVFP.
RESULTS
PBVCP is the second most common congenital laryngeal anomaly in the pediatric population, accounting for 10% to 20% of pediatric laryngeal conditions. PBVCP is related to idiopathic (42.2%), congenital (19.7%), and neurological (16.9%) conditions. A tracheotomy is required in 60% of cases regarding stridor and dyspnea, which are the most prevalent symptoms. The diagnosis is based on the etiological features, clinical presentation, laryngoscopic findings, and objective examinations. Laryngeal electromyography may be used to support the diagnosis in difficult cases, but its reliability depends on the practitioner's experience. The primary differential diagnosis is posterior glottis stenosis, which needs to be excluded regarding therapeutic and management differences with PBVCP. Transient surgical procedures consist of tracheotomy or laterofixation of the vocal fold. Current permanent procedures include uni- or bilateral partial arytenoidectomy, posterior transverse cordotomy, cricoid splits, and laryngeal selective reinnervation. There is no evidence of the superiority of some procedures over others.
CONCLUSIONS
PBVCP is the second most common laryngeal disorder in the pediatric population. Diagnosis is based on etiological and clinical findings and may require the use of laryngeal electromyography. Therapeutic management may involve several transient or permanent surgical procedures that are associated with overall subjective improvements in symptoms, laryngeal findings, and low complication rates.
PubMed: 38671615
DOI: 10.3390/children11040398 -
Cureus Mar 2024Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare neurological disorder characterized by recurring ipsilateral headache and paresis of one or more ocular...
Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare neurological disorder characterized by recurring ipsilateral headache and paresis of one or more ocular motor nerves. We report the case of a 56-year-old woman with systemic lupus erythematosus (SLE) and hypertension, who presented with severe recurring headaches, nausea, and vomiting. Initially misdiagnosed with cerebral venous sinus thrombosis, her symptoms persisted despite anticoagulant therapy. Further evaluation led to the diagnosis of RPON. Management included intravenous analgesia, hydration, and indomethacin for pain relief. Persistent headache episodes necessitated the introduction of lamotrigine, resulting in significant symptom improvement. However, discontinuation of lamotrigine led to a recurrence of symptoms, which resolved upon resuming the medication. This case contributes to the limited RPON literature, providing insights into its diagnosis and management, with the goal of enhancing awareness and improving patient care.
PubMed: 38665741
DOI: 10.7759/cureus.56924 -
Journal of Patient-reported Outcomes Apr 2024Chronic obstructive pulmonary disease (COPD) and muscle weakness can cause impaired physical function, significantly impacting patients' health-related quality of life...
Qualitative interviews of patients with COPD and muscle weakness enrolled in a clinical trial evaluating a new anabolic treatment: patient perspectives of disease experience, trial participation and outcome assessments.
BACKGROUND
Chronic obstructive pulmonary disease (COPD) and muscle weakness can cause impaired physical function, significantly impacting patients' health-related quality of life (HRQoL). Loss of muscle strength is usually assessed through clinical and performance outcome (PerfO) assessments, which consists of tasks performed in a standardized manner, providing evidence of a patient's functional ability. However, evidence documenting the patient experience of COPD and muscle weakness is limited.
METHODS
This two-stage qualitative study used semi-structured interviews in patients aged 45-80 years with COPD (post-bronchodilator forced expiratory volume in 1s [FEV]/forced vital capacity ratio < 0.70, and FEV% predicted of 30-80%) and muscle weakness. In Stage 1, 30-minute concept elicitation interviews were conducted with participants recruited across three US sites to explore impacts on physical functioning and activities of daily living. In Stage 2, interviews were performed with participants exiting a Phase IIa trial investigating the efficacy of a selective androgen receptor modulator (GSK2881078) on leg strength, whereby PerfOs were used to evaluate strength and physical functioning endpoints. These participants completed either 60-minute in-depth (n = 32) or 15-minute confirmatory (n = 35) interviews exploring trial experience, completion of outcome measures, disease experience and treatment satisfaction.
RESULTS
In Stage 1 (n = 20), most participants described their muscles as weak (83.3%). Difficulties with walking (100%) and lifting heavy objects (90%) were reported. In Stage 2, 60-minute interviews, all participants (n = 32) reported a positive trial experience. Most participants reported that the home exercise program was easy to fit into daily life (77.8%), the PROactive daily diary was easy to complete (100%) and wearable sensors were easy to use (65.6%). However, technical issues were reported (71%), and few participants (19.4%) found physical assessments easy to complete. Improvements in muscle strength and functional limitations were reported by most participants. The shorter 15-minute confirmatory interviews (n = 35) supported the in-depth interview results.
CONCLUSION
The qualitative interviews generated in-depth evidence of key concepts relevant to patients with COPD and muscle weakness and support the assessments of patient strength and physical function as outcome measures in this population in future studies.
TRIAL NUMBER
GSK Stage 1: 206869; Stage 2: 200182, NCT03359473; Registered December 2, 2017, https://clinicaltrials.gov/ct2/show/NCT03359473 .
Topics: Humans; Activities of Daily Living; Muscle Weakness; Outcome Assessment, Health Care; Paresis; Pulmonary Disease, Chronic Obstructive; Quality of Life
PubMed: 38641716
DOI: 10.1186/s41687-024-00712-0 -
BMC Neurology Apr 2024Although contrast extravasation on follow-up head computed tomography (CT) is frequently visualized after endovascular treatment, this phenomenon is rare after...
BACKGROUND
Although contrast extravasation on follow-up head computed tomography (CT) is frequently visualized after endovascular treatment, this phenomenon is rare after intravenous thrombolytic treatment in patients with acute ischemic stroke (AIS). Here, we report a case of contrast extravasation mimicking intracerebral hemorrhage (ICH) with intraventricular extension after intravenous thrombolytic treatment and computed tomography angiography (CTA).
CASE PRESENTATION
A 52-year-old man presented with right-sided hemiparesis and hypoesthesia. Initial non-contrast head CT was negative for intracranial hemorrhage and acute ischemic changes. He received intravenous treatment with tenecteplase 3.8 h after the onset of stroke. CTA of the head and neck was performed at 4.3 h after stroke onset. It showed no stenosis or occlusion of the carotid and major intracranial arteries. At about 1.5 h after CTA, the right-sided hemiparesis deteriorated, accompanied by drowsiness, aphasia, and urinary incontinence. Immediate head CT showed hyperdense lesions with mild space-occupying effect in the left basal ganglia and both lateral ventricles. The hyperdense lesions were reduced in size on follow-up CT after 5 h. Two days later, CT showed that the hyperdense lesions in the lateral ventricles almost completely disappeared and only a small amount remained in the infarcted area.
CONCLUSIONS
Contrast extravasation into the brain tissue and lateral ventricles, mimicking ICH with intraventricular extension, could occur after intravenous thrombolytic treatment and CTA in a patient with AIS, which might lead to misdiagnosis and wrong treatment of the patient. The rapid resolution of intracranial hyperdense lesions is key to differentiate contrast extravasation from ICH on serial non-enhanced CT.
Topics: Male; Humans; Middle Aged; Ischemic Stroke; Cerebral Hemorrhage; Fibrinolytic Agents; Stroke; Extravasation of Diagnostic and Therapeutic Materials; Paresis
PubMed: 38641592
DOI: 10.1186/s12883-024-03618-y -
Cureus Mar 2024Myasthenia gravis (MG) is an autoimmune disorder characterized by fluctuating weakness and fatigue in ocular, bulbar, limb, or respiratory muscles. Initially, more than...
Myasthenia gravis (MG) is an autoimmune disorder characterized by fluctuating weakness and fatigue in ocular, bulbar, limb, or respiratory muscles. Initially, more than half of MG patients experience isolated ocular symptoms, such as ptosis, diplopia, or muscle paresis. This case report presents a unique occurrence of MG in a four-year-old female, showcasing a two-year history of sudden onset, persistent yet fluctuating unilateral ptosis accompanied by exo-deviation and adduction deficit in the right eye. No diplopia or systemic features were observed. Positive findings in tests, including the ice pack test, Cogan twitch sign, fatiguability, and neostigmine test, indicated ocular myasthenia. Electromyography revealed a decremental response, while anti-acetylcholine antibodies showed borderline results. Computed tomography of the brain ruled out central causes, and routine laboratory testing yielded normal results. Treatment with pyridostigmine and corticosteroids led to significant improvement in symptoms. This case emphasizes the diverse presentation of MG in ophthalmology, with ocular signs serving as indicators in approximately half of the cases. Early diagnosis and prompt treatment are crucial for enhancing long-term prognosis. Emergency physicians should consider MG as a potential cause for unilateral ocular symptoms after excluding central causes. Accurate diagnosis and comprehensive management of MG are complex yet essential for ensuring optimal patient health.
PubMed: 38633942
DOI: 10.7759/cureus.56337 -
JCEM Case Reports Apr 2024Glucocorticoid resistance syndrome is a rare disorder with no genetically proven cases reported from India; in addition, there are no descriptions available regarding...
Glucocorticoid resistance syndrome is a rare disorder with no genetically proven cases reported from India; in addition, there are no descriptions available regarding its management during pregnancy. A 27-year-old woman, hypertensive since the age of 17 years, presented with hypokalemic paresis. She reported regular menses and acne. On investigation, she had elevated serum cortisol that remained unsuppressed after a low-dose dexamethasone suppression test. Genetic analysis revealed a novel, homozygous missense variant in exon 5 of the gene confirming glucocorticoid resistance syndrome. She was managed with oral dexamethasone followed by tapering of antihypertensive drugs. A year later, she conceived with assisted reproductive techniques when dexamethasone was replaced with prednisolone, necessitating the reintroduction of antihypertensive drugs to maintain normotension and potassium supplements to manage hypokalemia. She presented with acute abdomen at 36 weeks of gestation; evaluation revealed right adrenal hemorrhage, which was managed conservatively. Postpartum, the right adrenal lesion reduced in size and an underlying right adrenal myelolipoma was unveiled.
PubMed: 38633359
DOI: 10.1210/jcemcr/luae052 -
Surgical Neurology International 2024Primary extranodal marginal zone mucosa-associated lymphoid tissue-type B-cell lymphoma (EMZMBCL), which presents as a dural mass, is a rare intracranial tumor that...
BACKGROUND
Primary extranodal marginal zone mucosa-associated lymphoid tissue-type B-cell lymphoma (EMZMBCL), which presents as a dural mass, is a rare intracranial tumor that mimics a subdural hematoma or meningioma.
CASE DESCRIPTION
A 49-year-old woman presented to our hospital with transient right upper limb paresis, dysarthria for 10 min, and ongoing right upper-limb numbness. Computed tomography (CT) of the head revealed extra-axial lesions in the left frontal and parietal lobes. Based on the initial CT findings in the emergency room, an acute subdural hematoma was suspected. However, meningiomas and other intracranial tumors were also listed as differential diagnoses because there was no history of head trauma or coagulation abnormalities on blood examination, and further imaging studies were performed. Imaging findings suggested a subdural neoplastic lesion. A partial resection was performed for the lesion. Based on histopathological and immunohistochemical examinations, the patient was diagnosed with EMZMBCL. Whole-brain and intensity-modulated radiation therapies were administered as adjuvant therapies. The patient was discharged without neurological deficits.
CONCLUSION
EMZMBCL is a rare disease that should be considered in the differential diagnosis of subdural lesions, especially when there is no history of trauma or abnormalities in the coagulation system. The patient had a favorable outcome after selecting radiotherapy as the adjuvant therapy.
PubMed: 38628522
DOI: 10.25259/SNI_792_2023