scholarly journals Dejérine-Roussy syndrome associated with unilateral thalamic glioma

2021 ◽  
Author(s):  
Pedro Felipe Camelo Correa Alves Ferreira e Silva ◽  
Gustavo Ferreira Martins ◽  
Eduardo Augusto Guedes de Souza ◽  
Renato Miguel Rezende ◽  
Karine Cin Assenço ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Fatal Outcome ◽  
Cranial Nerves ◽  
Low Grade ◽  
Paretic Side ◽  
Unusual Condition ◽  
Thalamic Glioma ◽  
The Right ◽  
Ventral Posterolateral Nucleus ◽  
Anatomical Pathology

Context: Déjérine-Roussy Syndrome is a rare entity that occurs after an ischemia located in the ventral posterolateral nucleus, and it is characterized by hemiplegia, superficial hemianesthesia, mild hemiataxia and astereognosis, pain on the paretic side and choreoathetosis movements. This unusual condition can be caused by haemorrhage or neoplasm. Thalamic tumors make up less than 5% of all intracranial tumors. The rare clinical presentation of a thalamic tumor is a diagnostic and therapeutic challenge for neurology and neurosurgery practice and generally requires treatment without biopsy. Case report: A 54-years-old man presented complaining of burning and tingling paraesthesias, decreased sensitivity in left dimidium, associated with decreased visual acuity in the left eye. Physical examination showed complete left hemiparesis provided grade 4-, normoreflexia with athetoid movements of the left arm and hand, painful, thermal hemihipoesthesia and epicritic touch, allodyne in the left hemibody, pressure sensitivity present globally, visual campimetry by confrontation with heteronymous hemianopia without changes in the cranial nerves. Magnetic Resonance Imaging of the Skull Base showed an oval mass, with hyposignal in T1 and hypersignal in T2 and FLAIR, with peripheral contrast uptake in the thalamus and nuclei from the right base. The patient showed good clinical-surgical evolution after surgery with Glasgow Outcome Scale 4 and modified Rankin scale 2. Anatomical Pathology confirmed low-grade glioma. Conclusions: Early diagnosis and immediate therapy can delay a fatal outcome or decrease treatment-related morbidity.

Cystic hypersecretory ductal carcinoma (CHDC): a rare distinctive variant of ductal carcinoma

2021 ◽  
Vol 14 (7) ◽  
pp. e235488
Author(s):  
Ashley DiPasquale ◽  
Lashan Peiris ◽  
Svetlana Silverman
Keyword(s):  
Clinical Presentation ◽  
Ductal Carcinoma ◽  
Cystic Mass ◽  
Low Grade ◽  
Rare Form ◽  
Breast Malignancy ◽  
The Right

Cystic hypersecretory ductal carcinoma (CHDC) is a rare distinctive variant of ductal carcinoma that behaves in a low-grade fashion. This rare form of breast malignancy has only been reported a handful of times in the surgical literature. This article outlines the clinical presentation, workup and management of a 43-year-old woman who presented with a bilobed cystic mass of the right breast diagnosed as CHDC.

Isolated Congenital Absence of Cranial Nerves: Report of Two Cases

2018 ◽  
Vol 49 (06) ◽  
pp. 405-407
Author(s):  
Vivek Agarwal ◽  
Sumeet Dhawan ◽  
Naveen Sankhyan ◽  
Sameer Vyas
Keyword(s):  
High Resolution ◽  
Mr Imaging ◽  
Cranial Nerve ◽  
Clinical Presentation ◽  
Cranial Nerves ◽  
Rare Condition ◽  
Congenital Absence ◽  
The Third ◽  
Seventh Cranial Nerve ◽  
The Right

AbstractIsolated cranial nerve absence is a rare condition that can be diagnosed using high-resolution cranial nerve magnetic resonance (MR) imaging. Thorough clinical examination with proper knowledge of the course of cranial nerves may help diagnose this rare condition. We describe two cases, one each of, isolated congenital absence of the third and seventh cranial nerve with their clinical presentation. High-resolution T2-weighted MR imaging was done in both patients which revealed absence of cisternal segment of the right-sided third nerve and cisternal with canalicular segment of the right-sided facial nerve.

scholarly journals A Case of Vanishing Metastatic Mass: Right Atrial Mass in the Setting of Primary Epithelioid Hemangioendothelioma of the Spine

2018 ◽  
Vol 11 (2) ◽  
pp. 534-540 ◽  
Author(s):  
Eshan Patel ◽  
P. Kancharla ◽  
B.K. Surapaneni ◽  
K. Hennrick ◽  
M. Goldfinger ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Epithelioid Hemangioendothelioma ◽  
The Body ◽  
Right Atrial ◽  
Low Grade ◽  
Vascular Neoplasm ◽  
Right Atrial Mass ◽  
Progression Of Disease ◽  
The Right ◽  
Age And Sex

Epithelioid hemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that is characterized as intermediate between benign hemangioma and high-grade angiosarcoma affecting 1 in 1,000,000 people worldwide. It has been described throughout the body with lung, liver, skin, and bone being the most frequent sites. Primary EHE of the spine has been reported in 56 cases so far with no correlation of age and sex. Our case highlights a rare clinical presentation, etiopathogenesis, diagnosis, and treatment of EHE of the spine with metastasis to the right atrium. This is the first documented case of EHE of the spine with metastatic spread to the heart treated with bevacizumab leading to resolution of the heart metastatic mass. Further studies are warranted to develop a treatment formula for this rare tumor, to consider combination chemotherapy and new adjuvant targeted immunotherapies to prevent progression of disease.

A Method for Cranial Nerve XI Silencing During Surgery of the Foramen Magnum Region: Technical Case Report

2018 ◽  
Vol 16 (4) ◽  
pp. E130-E133
Author(s):  
Derek G Southwell ◽  
Jonathan D Breshears ◽  
William R Lyon ◽  
Michael W McDermott
Keyword(s):  
Skull Base ◽  
Cranial Nerve ◽  
Clinical Presentation ◽  
Obstructive Hydrocephalus ◽  
Cranial Nerves ◽  
Foramen Magnum ◽  
Cerebellar Tonsil ◽  
Suboccipital Craniotomy ◽  
Far Lateral ◽  
The Right

Abstract BACKGROUND AND IMPORTANCE Skull base surgery involves the microdissection and intraoperative monitoring of cranial nerves, including cranial nerve XI (CN XI). Manipulation of CN XI can evoke brisk trapezius contraction, which in turn may disturb the surgical procedure and risk patient safety. Here we describe a method for temporarily silencing CN XI via direct intraoperative application of 1% lidocaine. CLINICAL PRESENTATION A 41-yr-old woman presented with symptoms of elevated intracranial pressure and obstructive hydrocephalus secondary to a hemangioblastoma of the right cerebellar tonsil. A far-lateral suboccipital craniotomy was performed for resection of the lesion. During the initial stages of microdissection, vigorous trapezius contraction compromised the course of the operation. Following exposure of the cranial and cervical portions of CN XI, lidocaine was applied to the course of the exposed nerve. Within 3 min, trapezius electromyography demonstrated neuromuscular silencing, and further manipulation of CN XI did not cause shoulder movements. Approximately 30 min after lidocaine application, trapezius contractions returned, and lidocaine was again applied to re-silence CN XI. Gross total resection of the hemangioblastoma was performed during periods of CN XI inactivation, when trapezius contractions were absent. CONCLUSION Direct application of lidocaine to CN XI temporarily silenced neuromuscular activity and prevented unwanted trapezius contraction during skull base microsurgery. This method improved operative safety and efficiency by significantly reducing patient movement due to the unavoidable manipulation of CN XI.

Difficulty in Diagnosing Secondary Peripheral Chondrosarcoma: Radiology versus Anatomical Pathology

2016 ◽  
Vol 1 (4) ◽  
Author(s):  
Undang Ruhimat ◽  
Arie Hendarin
Keyword(s):  
Ultrasound Guidance ◽  
Classical Problem ◽  
Low Grade ◽  
Age Group ◽  
Multiple Osteochondromas ◽  
Younger Age ◽  
Secondary Chondrosarcoma ◽  
Primary Type ◽  
The Right ◽  
Anatomical Pathology

Chondrosarcoma is a malignant tumor derived from bone cartilage. This tumor can be either primary or secondary. Secondary chondrosarcoma has some differences compared to the primary type i.e. the incidence is rarer, the younger age group, and many are low grade. The authors report a case of a 26-year-old man came to Hasan Sadikin Hospital with a chief complaint of a lump in the right thigh with mild pain since 5 months before consulation that is increasingly enlarging. After undergoing physical and radiological examinations, the patient was diagnosed with suspect chondrosarcoma in right thigh. Histopathologic examinations were performed 2 times, one of which used ultrasound guidance, giving a diagnosis of chondroma in right thigh. However the lesion was finally treated as chondrosarcoma. We suspected that the patient was more likely to suffer from secondary peripheral chondrosarcoma originating from malignant transformation of multiple osteochondromas. As widely known, it is almost impossible to differentiate between the low grade chondrosarcoma and chondroma with full certainty and this poses a classical problem in diagnostic medicine. Case described here was an example of the difficulty in distinguishing between these two diseases. Key words: secondary chondrosarcoma, peripheral chondrosarcoma, chondrosarcoma arising  in osteochondroma

scholarly journals P040 An unusual case of giant cell arteritis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Jessica Ellis ◽  
Keziah Austin ◽  
Sarah Emerson
Keyword(s):  
Inflammatory Markers ◽  
Illicit Drugs ◽  
Unusual Case ◽  
Temporal Arteritis ◽  
White Cell Count ◽  
Healthcare Providers ◽  
Temporal Artery ◽  
Low Grade ◽  
Temporal Artery Biopsy ◽  
The Right

Abstract Background/Aims  A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods  The case is discussed below. Results  Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion  GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure  J. Ellis: None. K. Austin: None. S. Emerson: None.

scholarly journals Stereotactic Body Radiotherapy of a Solitary Metachronous Sphenoid Metastasis from Renal Cell Cancer: A Case Report

2021 ◽  
pp. 269-273
Author(s):  
Charles Marchand Crety ◽  
Estelle Vigneau ◽  
Camille Invernizzi
Keyword(s):  
Renal Cell Cancer ◽  
Renal Cell ◽  
Stereotactic Body Radiation Therapy ◽  
Clinical Presentation ◽  
Histopathological Examination ◽  
Cell Cancer ◽  
Papillary Renal Cell Carcinoma ◽  
Complete Response ◽  
Resonance Imaging ◽  
The Right

Nasosinus metastases from kidney cancer are an unusual clinical presentation although some cases are reported in the literature. Among these cases, sphenoidal metastases are even rarer. Here we report a case of lone sphenoid metastasis in patients with papillary renal cell cancer. Eight months after radical nephrectomy, the patient presented with progressively worsening diplopia. Magnetic resonance imaging showed a mass in the right sphenoid sinus. Histopathological examination of the biopsy sample confirmed diagnosis of sinonasal metastasis from papillary renal cell carcinoma. The patient was declined for surgical management and received stereotactic body radiation therapy. Reassessment MRI at 4 months showed a complete response of the treated sphenoid lesion.

scholarly journals Gasperini Syndrome: A Case Report and Systematic Review and Proposing a New Definition

2020 ◽  
Vol 12 (3) ◽  
pp. 433-439
Author(s):  
Riwaj Bhagat ◽  
Siddharth Narayanan ◽  
Marwa Elnazeir ◽  
Thong Diep Pham ◽  
Robert Paul Friedland ◽  
...  
Keyword(s):  
Systematic Review ◽  
Brain Mri ◽  
Cranial Nerves ◽  
Clinical Manifestations ◽  
The Other ◽  
Neurological Deficits ◽  
Brainstem Stroke ◽  
Neurological Features ◽  
The Right ◽  
Core Features

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.

scholarly journals A Case of Atypical Bartonellosis in a 4-Year-Old Immunocompetent Child

2021 ◽  
Vol 9 (5) ◽  
pp. 950
Author(s):  
Chiara Sodini ◽  
Elena Mariotti Zani ◽  
Francesco Pecora ◽  
Cristiano Conte ◽  
Viviana Dora Patianna ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Bartonella Henselae ◽  
Delayed Diagnosis ◽  
Mitral Valve Regurgitation ◽  
Low Grade ◽  
Systemic Involvement ◽  
Mild Disease ◽  
Precise Diagnosis ◽  
Major Illness ◽  
The Right

In most cases, infection due to Bartonella henselae causes a mild disease presenting with a regional lymphadenopathy frequently associated with a low-grade fever, headache, poor appetite and exhaustion that spontaneously resolves itself in a few weeks. As the infection is generally transmitted by cats through scratching or biting, the disease is named cat scratch disease (CSD). However, in 5–20% of cases, mainly in immunocompromised patients, systemic involvement can occur and CSD may result in major illness. This report describes a case of systemic CSD diagnosed in an immunocompetent 4-year-old child that can be used as an example of the problems that pediatricians must solve to reach a diagnosis of atypical CSD. Despite the child’s lack of history suggesting any contact with cats and the absence of regional lymphadenopathy, the presence of a high fever, deterioration of their general condition, increased inflammatory biomarkers, hepatosplenic lesions (i.e., multiple abscesses), pericardial effusion with mild mitral valve regurgitation and a mild dilatation of the proximal and medial portion of the right coronary artery, seroconversion for B. henselae (IgG 1:256) supported the diagnosis of atypical CSD. Administration of oral azithromycin was initiated (10 mg/kg/die for 3 days) with a progressive normalization of clinical, laboratory and US hepatosplenic and cardiac findings. This case shows that the diagnosis of atypical CSD is challenging. The nonspecific, composite and variable clinical features of this disease require a careful evaluation in order to achieve a precise diagnosis and to avoid both a delayed diagnosis and therapy with a risk of negative evolution.

scholarly journals Provocation and prediction of visual peripersonal neglect-like symptoms in preoperative planning and during awake brain surgery

2021 ◽  
Author(s):  
Viktória Tamás ◽  
Gabriella Sebestyén ◽  
Szilvia Anett Nagy ◽  
Péter Zsolt Horváth ◽  
Ákos Mérei ◽  
...  
Keyword(s):  
Preoperative Planning ◽  
Right Hemisphere ◽  
Superior Temporal Gyrus ◽  
Low Grade ◽  
Navigated Transcranial Magnetic Stimulation ◽  
Resonance Imaging ◽  
Parietal Area ◽  
Routine Magnetic Resonance Imaging ◽  
Visuospatial Functions ◽  
The Right

AbstractNeglect is a severe neuropsychological/neurological deficit that usually develops due to lesions of the posterior inferior parietal area of the right hemisphere and is characterized by a lack of attention to the left side. Our case is a proven right-handed, 30-year-old female patient with a low-grade glioma, which was located in the temporo-opercular region and also in the superior temporal gyrus of the right hemisphere. Upon presurgical planning, the motor, language, and visuospatial functions were mapped. In order to achieve this, the protocol for routine magnetic resonance imaging and navigated transcranial magnetic stimulation has been expanded, accordingly.

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