scholarly journals A Case Report of Lipoid Proteinosis with Brain and Laryngeal Presentation

2020 ◽  
Vol 17 (3) ◽  
Author(s):  
Mohammad Ali Kazemi ◽  
Zahra Ahmadian Mazhin ◽  
Hashem Sharifian ◽  
Samira Hemmati ◽  
Behnaz Moradi
Keyword(s):  
Case Report ◽  
Periodic Acid ◽  
Clinical Manifestations ◽  
Genetic Mutation ◽  
Imaging Findings ◽  
True Vocal Cord ◽  
Periodic Acid Schiff ◽  
Lipoid Proteinosis ◽  
Voice Changes ◽  
The Right

: Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis. Genetic mutation leads to deposition of abnormal amounts of hyaline like material in the skin and viscera, which is the cause of clinical manifestations. It mostly involves the skin, intracranium, and the larynx. In this case report, we present a case with a long history of hoarseness. Imaging findings include bilateral true vocal cord (TVC) mucosal irregularity with hyperdense depositions, bilateral medial temporal amygdala parallel bean shape calcification (pathognomonic sign), and bilateral striatal (caudate and putamen) hypoattenuation. The patient also had multiple warty papules on the hands. Biopsy of the right TVC showed submucosal deposition of periodic acid-Schiff (PAS)-positive amorphous hyaline material and confirmed the diagnosis of lipoid proteinosis. Typical imaging findings especially in the brain could be very helpful in interpretation of laryngeal imaging findings in cases of lipoid proteinosis who manifest with long term voice changes and hoarseness.

Dematiaceous fungal invasion of a bandage contact lens

2021 ◽  
Vol 14 (2) ◽  
pp. e240029
Author(s):  
Anirban Dutta ◽  
Sujata Das ◽  
Himanshu Sekhara Behera ◽  
Ruchi Mittal
Keyword(s):  
Contact Lens ◽  
Periodic Acid ◽  
Significant Risk ◽  
Dematiaceous Fungi ◽  
Periodic Acid Schiff ◽  
Bandage Contact Lens ◽  
History Of ◽  
Eye Examination ◽  
The Right

A 61-year-old man presented with a 1-month history of reduced vision, redness and pain in the right eye. Examination revealed a bandage contact lens (BCL) in situ with diffuse, pigmented deposits. On removal, the underlying cornea was found to be clear. He had been prescribed the BCL 6 months ago following a deep-seated corneal foreign body removal and was unable to follow-up subsequently.The BCL was sent for microbiological and histopathological evaluation. The culture revealed growth of Cladosporium spp, a dematiaceous fungi. Periodic acid–Schiff staining revealed infiltration of pigmented fungal filaments into the substance of the BCL.While contact lens deposits are a frequent finding, fungal deposits are seldom noted. Irregular follow-up and improper lens maintenance are significant risk factors for the same. Early identification and subsequent removal of the lens is vital to prevent infection of the underlying ocular structures.

Segmental Neurofibromatosis Follows Blaschko's Lines or Dermatomes Depending on the Cell Line Affected: Case Report and Literature Review

2004 ◽  
Vol 8 (5) ◽  
pp. 353-356
Author(s):  
Fara P. Redlick ◽  
James C. Shaw
Keyword(s):  
Schwann Cells ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Cell Types ◽  
Genetic Mutation ◽  
The Body ◽  
Chromosome 17 ◽  
Medline Search ◽  
Characteristic Features ◽  
The Right

Background: Segmental neurofibromatosis type 1 (NF-1) has the characteristic features of generalized NF-1 but is isolated to a particular segment of the body. Segmental NF-1 results from a postzygotic mutation during embryogenesis in the NF-1 gene on chromosome 17. The embryologic timing of the mutation and cell types affected predict the clinical phenotype. Objective: We present a case of a 52-year-old woman with segmental neurofibromas isolated to the right cheek and neck. We review the recent literature on the genetic and cellular differences between the various clinical manifestations of segmental NF-1. Methods: A MEDLINE search for cases of segmental neurofibromatosis was conducted. Results: In patients with segmental NF-1 presenting as neurofibromas-only, the distribution follows a neural distribution in dermatomes because the genetic mutation appears to be limited to Schwann cells. In patients with pigmentary changes only, the NF-1 mutation has been shown to occur in fibroblasts and the distribution tends to follow the lines of Blaschko. Conclusion: Our patient's neurofibromas were secondary to a postzygotic mutation in the NF-1 gene of neural crest–derived cells. This mutation most likely occurred later in embryogenesis in cells that had already differentiated to Schwann cells and were committed to the dermatomal distribution of the right neck and cheek region (C2).

scholarly journals Bilateral temporal bone fractures: a case report

2017 ◽  
Vol 4 (1) ◽  
pp. 271
Author(s):  
Kiran Natarajan ◽  
Koka Madhav ◽  
A. V. Saraswathi ◽  
Mohan Kameswaran
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Facial Paralysis ◽  
Bone Fractures ◽  
Clinical Manifestations ◽  
Csf Leak ◽  
The Right ◽  
Temporal Bones

<p>Bilateral temporal bone fractures are rare; accounting for 9% to 20% of cases of temporal bone fractures. Clinical manifestations include hearing loss, facial paralysis, CSF otorhinorrhea and dizziness. This is a case report of a patient who presented with bilateral temporal bone fractures. This is a report of a 23-yr-old male who sustained bilateral temporal bone fractures and presented 18 days later with complaints of watery discharge from left ear and nose, bilateral profound hearing loss and facial weakness on the right side. Pure tone audiometry revealed bilateral profound sensori-neural hearing loss. CT temporal bones &amp; MRI scans of brain were done to assess the extent of injuries. The patient underwent left CSF otorrhea repair, as the CSF leak was active and not responding to conservative management. One week later, the patient underwent right facial nerve decompression. The patient could not afford a cochlear implant (CI) in the right ear at the same sitting, however, implantation was advised as soon as possible because of the risk of cochlear ossification. The transcochlear approach was used to seal the CSF leak from the oval and round windows on the left side. The facial nerve was decompressed on the right side. The House-Brackmann grade improved from Grade V to grade III at last follow-up. Patients with bilateral temporal bone fractures require prompt assessment and management to decrease the risk of complications such as meningitis, permanent facial paralysis or hearing loss. </p>

scholarly journals Multinodular and Vacuolating Neuronal Tumor

2018 ◽  
Vol 6 (9) ◽  
pp. 1697-1698 ◽  
Author(s):  
Charmaine Zahra ◽  
Reuben Grech
Keyword(s):  
Case Report ◽  
Brain Imaging ◽  
Temporal Lobe ◽  
Benign Lesion ◽  
Occipital Lobe ◽  
Imaging Findings ◽  
Cns Tumours ◽  
The Right ◽  
Rare Diagnosis ◽  
Neuronal Tumor

BACKGROUND: Multinodular and Vacuolating Neuronal Tumor (MVNT) of the cerebrum is a benign lesion described recently in the WHO CNS tumours in 2016. Although this tumour is uncommon, clinicians should be acquainted with the possible presentation and imaging findings. CASE REPORT: We present a case of a young gentleman whose only symptom was absence seizures. Brain imaging showed lesions, compatible with this rare diagnosis. CONCLUSION: Our description of imaging findings on MRI highlights the characteristic cystic appearances of note in the right occipital lobe, in contrast to the temporal lobe as the predominant location found in previous cases.

scholarly journals Benign monomelic amyotrophy with proximal upper limb involvement: case report

2007 ◽  
Vol 65 (2b) ◽  
pp. 524-527 ◽  
Author(s):  
Marco Antonio Orsini Neves ◽  
Marcos R.G. de Freitas ◽  
Mariana Pimentel de Mello ◽  
Carlos Henrique Dumard ◽  
Gabriel R. de Freitas ◽  
...  
Keyword(s):  
Case Report ◽  
Upper Limb ◽  
Lower Limb ◽  
Clinical Manifestations ◽  
Rare Condition ◽  
Insidious Onset ◽  
Rare Location ◽  
Muscle Groups ◽  
Clinical Stabilization ◽  
The Right

Monomelic amyotrophy (MA) is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Usually sporadic, it usually has an insidious onset with a mean evolution of 2 to 4 years following first clinical manifestations, which is, in turned, followed by stabilization. We report a case of 20-years-old man who presented slowly progressive amyotrophy associated with proximal paresis of the right upper limb, which was followed by clinical stabilization 4 years later. Eletroneuromyography revealed denervation along with myofasciculations in various muscle groups of the right upper limb. We call atention to this rare location of MA, as well as describe some theories concerning its pathophysiology .

scholarly journals Non-Mucinous Lepidic Predominant Adenocarcinoma Presenting with Extensive Aerogenous Spread

Rare Tumors ◽  
2016 ◽  
Vol 8 (4) ◽  
pp. 169-172 ◽  
Author(s):  
Yusuke Takanashi ◽  
Shogo Tajima ◽  
Masaru Tsukui ◽  
Kazuya Shinmura ◽  
Takamitsu Hayakawa ◽  
...  
Keyword(s):  
Cancer Progression ◽  
Histopathological Examination ◽  
Periodic Acid ◽  
Lower Lobe ◽  
Poor Response ◽  
Clara Cells ◽  
Invasive Adenocarcinoma ◽  
Rare Presentation ◽  
Periodic Acid Schiff ◽  
The Right

An extremely rare case of non-mucinous lepidic-predominant invasive adenocarcinoma (LPA) showing extensive aerogenous spread with a pneumonic presentation is reported. A 73-year-old woman was referred to our hospital because of an infiltrative shadow on chest xray. Chest computed tomography revealed extensive ground glass opacities in the right lower lobe, which was accompanied by infiltrative shadow with a pneumonic presentation. Invasive mucinous adenocarcinoma was presumed, and a partial resection of the right lower lobe was done. Histopathological examination revealed lepidic growth-predominant invasive adenocarcinoma with Clara type tumor cells, and there were innumerable aerogenous metastases also consisting of Clara cells. Because Alcian Blue and periodic acid-Schiff staining disclosed no mucus, the tumor was diagnosed as a non-mucinous LPA. The patient showed a poor response to 5 courses of pemetrexed, and she died one year after the diagnosis due to cancer progression. Nonmucinous LPA showed a rare presentation characterized by extensive aerogenous spread followed by a poor prognosis.

scholarly journals Pathological Findings in the Pituitary Glands of Dogs and Cats

2018 ◽  
Vol 55 (6) ◽  
pp. 880-888 ◽  
Author(s):  
Laura Polledo ◽  
Guy C. M. Grinwis ◽  
Peter Graham ◽  
Mark Dunning ◽  
Kerstin Baiker
Keyword(s):  
Adrenocorticotropic Hormone ◽  
Periodic Acid ◽  
Clinical Manifestations ◽  
Histopathological Diagnosis ◽  
Periodic Acid Schiff ◽  
Melanocyte Stimulating Hormone ◽  
Nodular Hyperplasia ◽  
Cystic Changes ◽  
Pituitary Glands ◽  
Hematoxylin And Eosin

With the exception of classic functional adenomas in dogs and horses, pituitary lesions are infrequently described in the veterinary literature. Approximately 10% of pituitary glands from asymptomatic humans contain abnormalities, but the equivalent proportion in small animals is unknown. Pituitary glands from 136 dogs and 65 cats collected during routine necropsies were examined to determine the prevalence of pituitary lesions and their histopathological diagnosis. Lesions were characterized in sections stained with hematoxylin and eosin, periodic acid-Schiff (PAS), Gordon and Sweet’s and reticulin stains, and immunohistochemistry for adrenocorticotropic hormone (ACTH), growth hormone, melanocyte stimulating hormone–α, and prolactin. Pituitary abnormalities were identified in 36 of 136 (26.4%) dogs and 10 of 65 (15.3%) cats. Cystic changes were the most common lesion, occurring in 18 (13.2%) dogs and 8 (12.3%) cats. Pituitary neoplasia was detected in 14.1% (12/85) of middle-aged and old dogs; 1 (1.5%) cat had pituitary nodular hyperplasia. PAS and reticulin stains helped differentiate ACTH-immunoreactive adenomas from hyperplastic nodules: adenomas contained PAS-positive intracytoplasmic granules and loss of the normal reticulin network. One dog had a pituitary carcinoma with infiltration into the thalamus. Other pituitary abnormalities included secondary metastases (2 dogs) and hypophysitis (4 dogs, 1 cat). In most cases, the lesion appeared to be subclinical and could be considered incidental, whereas clinical manifestations were apparent in only 4 dogs (2.9%) and none of the cats with pituitary lesions. Pituitary abnormalities are common in dogs and cats, and their clinical relevance requires further investigation.

scholarly journals Rhinosinusitis in an Australian mare caused by Flavodon flavus, a recently recognized invasive fungal pathogen of the horse

2019 ◽  
Vol 32 (1) ◽  
pp. 162-165
Author(s):  
Willy W. Suen ◽  
Steven Zedler ◽  
Rochelle Price ◽  
Tina Maguire ◽  
Catriona Halliday ◽  
...  
Keyword(s):  
Fungal Pathogen ◽  
Periodic Acid ◽  
The United States ◽  
Nasal Passage ◽  
Periodic Acid Schiff ◽  
Ct Analysis ◽  
Bone Lysis ◽  
Methenamine Silver ◽  
The Right ◽  
Suppurative Inflammation

We describe herein the clinical, endoscopic, computed tomography (CT), pathologic, and microbiologic features of an infection caused by an under-recognized fungal pathogen, Flavodon flavus, in a 25-y-old Australian Quarter Horse. The horse had a unilateral obstructive nasal mass, resulting in stertor and dyspnea. On endoscopy, the mass was tan, multinodular, and completely obstructed the nasal passage. CT analysis revealed a large, soft tissue–attenuating and partially mineralized mass in the right nasal passage and dorsal-conchofrontal sinus, expanding into adjacent paranasal sinuses with associated bone lysis and rhinosinusitis. Histopathology of the mass on 2 occasions revealed suppurative inflammation initially, and pyogranulomatous inflammation subsequently. The inflammatory reaction surrounded numerous spherical fungal structures (~60–80 µm diameter) that stained positively on periodic acid–Schiff and Grocott methenamine silver stains. PCR for the fungal internal transcribed spacer 1 and 2 regions followed by Sanger sequencing on a cultured isolate identified the agent as F. flavus, which has only been reported previously as pathogenic in one horse in the United States, to our knowledge. Previous reports described this fungus as a nonpathogenic, environmental commensal fungus associated with insects and plants.

scholarly journals Subclinical onychomycosis in patients with type II diabetes

2015 ◽  
Vol 7 (3) ◽  
Author(s):  
Amira Elbendary ◽  
Amira El Tawdy ◽  
Naglaa Zaki ◽  
Mostafa Alfishawy ◽  
Amr Rateb
Keyword(s):  
Early Detection ◽  
Type Ii Diabetes ◽  
Periodic Acid ◽  
Clinical Manifestations ◽  
Diabetic Patients ◽  
Type Ii ◽  
Periodic Acid Schiff ◽  
Cross Sectional ◽  
Uncontrolled Diabetes ◽  
Fungal Organisms

Fungal organisms could be present in the nail without any clinical manifestations. As onychomycosis in diabetics has more serious complications, early detection of such infection could be helpful to prevent them. We aim in this study to assess the possibility of detecting subclinical onychomycosis in type II diabetic patients and addressing possible associated neuropathy. A cross sectional, observational study included patients with type II diabetes with normal big toe nail. All were subjected to nail clipping of the big toe nail, followed by staining with Hematoxylin and Eosin and Periodic-Acid-Schiff (PAS) stains and examined microscopically. A total of 106 patients were included, fungal infection was identified in eight specimens, all were uncontrolled diabetes, and six had neuropathy. Using the nail clipping and microscopic examination with PAS stain to detect such subclinical infection could be an applicable screening test for diabetic patients, for early detection and management of onychomycosis.

The unusual case of neck lymphadenopathy - oculoglandular form of tularemia

2018 ◽  
Vol 7 (2) ◽  
pp. 1-5
Author(s):  
Katarzyna Baranowska-Kempisty ◽  
Jolanta Kluz-Zawadzka ◽  
Sylwia Warzybok-Bajda ◽  
Krzysztof Szuber
Keyword(s):  
Adverse Effect ◽  
Case Report ◽  
Francisella Tularensis ◽  
Unusual Case ◽  
Drug Research ◽  
Delayed Diagnosis ◽  
Clinical Manifestations ◽  
Elisa Test ◽  
Gram Negative ◽  
The Right

Tularemia is anthropozoonosis caused by Bacteria Francisella tularensis a gram negative, non-motile aerobic bacillus.[5][8] The bacteria is transmitted mostly by rabbits, hares, rodents and arthropods. The pathogen penetrates into an organism via damaged skin, conjunctiva or mucosa- either through inhalation or ingestion. The clinical manifestations depend on the route of acquisition. Six forms of the disease can be distinguished: ulceroglandular or glandular, oculoglandular, oropharyngeal, respiratory, typhoidal, and intestinal. [11] We present a case report of the rarest oculoglandular form of tularemia. The patient was admitted to the hospital with right pre-auricular swelling, right sided neck lymphadenopathy, conjunctivitis and a nodule of the right eyelid. After excluding more common differential diagnoses, an ELISA test was performed and Francisella tularensis antibodies were identified. Patient administered proper antibiotic therapy. Tularemia is a rarely occurring disease in Poland and is nearly never taken into consideration by otolaryngologists when diagnosing patients with neck lymphadenopathy. In clinical practice, otorhinolaryngologists should always consider this infectious zoonosis, especially the oculoglandular and oropharyngeal forms, as delayed diagnosis and treatment may causes serious health consequences in patients. In the future new drug research should be conducted because of the adverse effect of widely used medications specially for children and pregnant woman.

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