scholarly journals Fibrous Dysplasia of the Skull Base Presenting Acutely in an Adult

2006 ◽  
Vol 33 (3) ◽  
pp. 325-327 ◽  
Author(s):  
F. Amoozegar ◽  
A. Guberman
Keyword(s):  
Skull Base ◽  
Fibrous Dysplasia ◽  
Previous History ◽  
Acute Onset ◽  
Low Grade ◽  
Third Nerve Palsy ◽  
History Of ◽  
Acute Headache ◽  
Eye Closure ◽  
Time Of Presentation

Fibrous dysplasia (FD) of the bone is an idiopathic disorder in which bone-forming mesenchyme develops abnormally. Here, we describe an unusual case in an adult, with recurrent acute headache and third nerve palsy due to FD of the skull base and low-grade fibrosarcoma.A previously healthy 32-year-old female office clerk, presented with an eight day history of acute-onset, severe, right occipital and retro-orbital continuous headache, associated with nausea and vomiting. She had required dimenhydrinate, acetaminophen with 15 mg codeine, and meperidine with only mild relief of the headache. She also described a 5-day history of right eye ptosis which had progressed to eye closure by the time of presentation. She had otherwise been well, with no systemic or constitutional symptoms and no previous history of headaches.

Paroxysmal Stabbing Headache in The Multiple Dermatomes of The Head and Neck: A Variant of Primary Stabbing Headache or Occipital Neuralgia?

Cephalalgia ◽  
2007 ◽  
Vol 27 (10) ◽  
pp. 1101-1108 ◽  
Author(s):  
JH Shin ◽  
HK Song ◽  
JH Lee ◽  
WK Kim ◽  
MK Chu
Keyword(s):  
Medical Center ◽  
Previous History ◽  
Headache Disorder ◽  
Acute Onset ◽  
Occipital Neuralgia ◽  
Cervical Nerve ◽  
Primary Stabbing Headache ◽  
History Of ◽  
Stabbing Headache

A paroxysmal stabbing or icepick-like headache in the multiple nerve dermatomes, especially involving both trigeminal and cervical nerves, has not been fully explained or classified by the International Classification of Headache Disorder, 2nd Edition (ICHD-II). Of patients with acute-onset paroxysmal stabbing headache who had visited the Hallym University Medical Center during the last four years, 28 subjects with a repeated stabbing headache involving multiple dermatomes at the initial presentation or during the course were prospectively enrolled. All patients were neurologically and otologically symptom free. A coincidental involvement of both trigeminal and cervical nerve dermatomes included seven cases. Six cases involved initially the trigeminal and then cervical nerve dermatomes. Five cases showed an involvement of the cervical and then trigeminal nerve dermatomes. The remaining patients involved multiple cervical nerve branches (the lesser occipital, greater occipital and greater auricular). Pain lasted very shortly and a previous history of headache with the same nature was reported in 13 cases. Preceding symptom of an infection and physical and/or mental stress were manifested in seven and six subjects, respectively. All patients showed a self-limited benign course and completely recovered within a few hours to 30 days. Interestingly, a seasonal gradient in occurrence of a stabbing headache was found in this study. A paroxysmal stabbing headache manifested on multiple dermatomes can be explained by the characteristics of pain referral, and may be considered to be a variant of primary stabbing headache or occipital neuralgia.

scholarly journals Histomolecular characterization of intracranial meningiomas developed in patients exposed to high-dose cyproterone acetate: an antiandrogen treatment

2019 ◽  
Vol 1 (1) ◽  
Author(s):  
Sylvain Portet ◽  
Rania Naoufal ◽  
Gaëlle Tachon ◽  
Adrien Simonneau ◽  
Anaïs Chalant ◽  
...  
Keyword(s):  
Skull Base ◽  
Cyproterone Acetate ◽  
Comparative Genomic ◽  
High Dose ◽  
Low Grade ◽  
Pharmacological Interaction ◽  
Pik3ca Mutations ◽  
History Of ◽  
Underlying Mechanisms ◽  
Copy Number Changes

Abstract Background Meningiomas are the most common primary intracranial tumors in adults. The relationship between meningiomas and exogenous sex hormones such as cyproterone acetate (CPA) is well documented, yet the underlying mechanisms remain unknown. Defining the histomolecular status of meningiomas developed on CPA would help us to better understand the oncogenesis of these tumors. Methods We identified 30 patients operated for a meningioma after long-term high-dose CPA therapy and with a history of CPA discontinuation before establishing the indication for surgical intervention. We used array-comparative genomic hybridization (to characterize copy number changes in those 30 meningiomas and subsequently performed next-generation sequencing with the National Institute of Cancer (INCa) solid tumor panel, which is a targeted panel of clinically actionable genes. We also examined grade, type, and clinical features. Results We identified AKT1 mutations or PIK3CA mutations in 33.3% of CPA meningiomas. AKT1 and PIK3CA mutations were mutually exclusive. Enrichment in oncogenic PIK3CA mutations in the CPA cohort was detected. CPA meningiomas showed chromosomal stability and were located mainly in the skull base. Ninety percent of CPA meningiomas were low-grade meningiomas and 63.4% were meningotheliomas. Half of our CPA cohort had microcystic components. Conclusion Our study shows that low-grade meningothelial meningiomas of the skull base are predominant in CPA meningiomas. We identified PIK3CA/AKT1 pathway as a hypothetical actor in onco-pharmacological interaction between meningiomas and CPA. This signaling pathway could be an interesting target for precision medicine trials in meningioma patients who have been subjected to CPA. Our results could invite the scientific community to review the current classification of meningiomas and to evolve toward more specific histomolecular classification.

Pre-operative assessment of patients undergoing endoscopic, transnasal, transsphenoidal pituitary surgery

2007 ◽  
Vol 122 (6) ◽  
pp. 644-646 ◽  
Author(s):  
D Lubbe ◽  
P Semple
Keyword(s):  
Skull Base ◽  
Skull Base Surgery ◽  
Previous History ◽  
Pituitary Surgery ◽  
Anterior Skull Base ◽  
Nasal Discharge ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Pituitary Macroadenoma ◽  
Main Complaint ◽  
History Of

AbstractObjective:To demonstrate the importance of pre-operative ear, nose and throat assessment in patients undergoing endoscopic, transsphenoidal surgery for pituitary tumours.Case reports:Literature pertaining to the pre-operative otorhinolaryngological assessment and management of patients undergoing endoscopic anterior skull base surgery is sparse. We describe two cases from our series of 59 patients undergoing endoscopic pituitary surgery. The first case involved a young male patient with a large pituitary macroadenoma. His main complaint was visual impairment. He had no previous history of sinonasal pathology and did not complain of any nasal symptoms during the pre-operative neurosurgical assessment. At the time of surgery, a purulent nasal discharge was seen emanating from both middle meati. Surgery was abandoned due to the risk of post-operative meningitis, and postponed until the patient's chronic rhinosinusitis was optimally managed. The second patient was a 47-year-old woman with a large pituitary macroadenoma, who presented to the neurosurgical department with a main complaint of diplopia. She too gave no history of previous nasal problems, and she underwent uneventful surgery using the endoscopic, transnasal approach. Two weeks after surgery, she presented to the emergency unit with severe epistaxis. A previous diagnosis of hereditary haemorrhagic telangiectasia was discovered, and further surgical and medical intervention was required before the epistaxis was finally controlled.Conclusions:Pre-operative otorhinolaryngological assessment is essential prior to endoscopic pituitary or anterior skull base surgery. A thorough otorhinolaryngological history will determine whether any co-morbid diseases exist which could affect the surgical field. Nasal anatomy can be assessed via nasal endoscopy and sinusitis excluded. Computed tomography imaging is a valuable aid to decisions regarding additional procedures needed to optimise access to the pituitary fossa.

scholarly journals Low-grade Glial Tumor with Features of Astroblastoma in a Dog

2005 ◽  
Vol 42 (3) ◽  
pp. 366-369 ◽  
Author(s):  
J. R. Cowart ◽  
F. Y. Schulman ◽  
H. Mena
Keyword(s):  
Blood Vessels ◽  
Acute Onset ◽  
Low Grade ◽  
Glial Tumor ◽  
Cellular Processes ◽  
Limb Ataxia ◽  
History Of ◽  
Eosinophilic Inclusions ◽  
Mitotic Figures ◽  
Cytoplasmic Processes

A 12-year-old, neutered, male Belgian Malinois/Great Dane cross dog presented with a 5-month history of weakness and lack of endurance followed by acute onset of rear limb ataxia. At autopsy, a 9 x 16 mm, multilobular, firm, white to tan, expansile mass was found in the cerebellum. Mild dilatation of the lateral ventricles was also noted. Histologically, there was a well-demarcated glial neoplasm composed of medium-sized astrocytic elements that had homogeneous cytoplasm, sometimes with globular eosinophilic inclusions, irregular peripherally located nuclei with a single nucleolus, and short cytoplasmic processes. Prominent peri-vascular pseudorosettes with cellular processes in contact with blood vessels were present. Some blood vessels exhibited hyalinized walls. Mitotic figures were not observed. Immunohistochemically, neoplastic cells expressed glial fibrillary acidic protein and vimentin. These features are consistent with an astroblastoma. This is the first clinicopathologic correlation and detailed description of a low-grade glial tumor with features of astroblastoma in a dog.

scholarly journals Hashimoto’s Encephalopathy Presenting with Acute Behavioral Disturbances: A Case Report

2020 ◽  
Vol 4 (4) ◽  
pp. 309
Author(s):  
Rozi NR ◽  
RM Yousuf ◽  
Kok HT ◽  
Mohd Unit H ◽  
Ibrahim KA ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Previous History ◽  
Acute Onset ◽  
Neurological Deficits ◽  
Cns Infection ◽  
Behavioural Disturbance ◽  
Behavioral Disturbances ◽  
Hashimoto’S Encephalopathy ◽  
Intravenous Acyclovir ◽  
History Of

Hashimoto’s encephalopathy (HE) is a rare and poorly understood neuropsychiatric illness of presumed autoimmune origin, with elevated titres of anti-thyroid antibodies. Its clinical presentation is highly variable that mimic a variety of neurologic and/or psychiatric disorders. Clinical presentation often suggests an infectious etiology which often leads to a mistaken diagnosis. We present the case of 35 year-old female who presented with acute onset behavioural disturbance of one day duration. On examination she was unkempt, emotionally labile, appeared withdrawn and unable to respond to questions. She had no focal neurological deficits. CNS infection was suspected and lumbar puncture was suggested, which the family members refused.She was empirically treated with intravenous acyclovir and ceftriaxone.Metabolic disorder, infectious and toxic issues were ruled out through laboratory testing.In view of her previous history of hyperthyroidism, suspicion of Hashimoto encephalopathy arose.The diagnosis was supported by the elevated level of anti-thyroglobulin (TG) antibody.We report this case to increase its awareness as it is one of the few treatable and easily reversible causes of acute encephalopathy. It should be considered in the differential diagnoses in any patient who presents with acute behavioural disturbance and has concurrent thyroid disorder.International Journal of Human and Health Sciences Vol. 04 No. 04 October’20 Page : 309-312

scholarly journals Neurosarcoidosis presenting with a partial Claude syndrome

2019 ◽  
Vol 12 (11) ◽  
pp. e232317
Author(s):  
Hao Meng Yip ◽  
Kirtana Vallabhaneni ◽  
David Williams
Keyword(s):  
Previous History ◽  
Cranial Neuropathy ◽  
Resonance Imaging ◽  
Facial Weakness ◽  
Cutaneous Sarcoidosis ◽  
Third Nerve Palsy ◽  
Common Causes ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Csf Examination

Neurosarcoidosis when encountered by neurologists most commonly presents as cranial neuropathy, peripheral mononeuropathy,polyneuropathy, myopathy, meningitis or myelopathy. There are limited reports in the current literature on the cases of neurosarcoidosis patients presenting with ischaemic stroke. We discuss a 52-year-old patient with a known previous history of cutaneous sarcoidosis presenting with an acute third nerve palsy, facial weakness and ataxia. His magnetic resonance imaging (MRI) brain demonstrated focal signal changes in the midbrain consistent with an acute ischaemic event in the region of his third nucleus, suggesting a partial Claude syndrome presentation. Cerebrospinal fluid (CSF) examination demonstrated an elevated angiotensin-converting enzyme (ACE) level. We discuss the difficulties associated with confirming a diagnosis for his presentation and consider distinctions in stroke in neurosarcoid and its management in comparison to more common causes.

A Contemporary Clinicopathologic Analysis of Primary Urothelial Carcinoma of the Urethra Without Concurrent Renal Pelvic, Ureteral, or Bladder Carcinoma

2021 ◽  
pp. 106689692110324
Author(s):  
Fengming Chen ◽  
Shreyas Joshi ◽  
Bradley C. Carthon ◽  
Adeboye O. Osunkoya
Keyword(s):  
Urothelial Carcinoma ◽  
Renal Pelvis ◽  
Previous History ◽  
Low Grade ◽  
High Grade ◽  
Divergent Differentiation ◽  
History Of ◽  
Variant Histology

Primary urothelial carcinoma (UCa) of the urethra is relatively uncommon, and the underlying pathogenesis has not been well characterized, especially in the absence of concurrent UCa at other sites. A search for cases of primary UCa of the urethra was conducted. Patients with concurrent UCa of the renal pelvis, ureter, or bladder at the time of diagnosis of the primary tumor were excluded. Clinicopathologic and follow-up data were obtained. A total of 35 cases from 30 patients (27 male and 3 female) were included in the study. The mean patient age at the initial diagnosis was 71 years (range: 41-90 years). Cases were composed of high-grade UCa (26 of 35 = 74%), low-grade UCa (4 of 35 = 11%), and UCa in situ (5 of 35 = 14%). Invasion was present in 14 of 26 (54%) cases of high-grade UCa. Interestingly, 23 of 30 (77%) patients had a previous history of UCa including 7 (30%) cases with divergent differentiation or variant histology. Follow-up data were available in 23 patients with a mean duration of 26.7 months (range: 0.6-87 months). Eleven patients (31%) died of metastatic UCa. This is one of the largest studies to date of primary UCa of the urethra without concurrent UCa of the renal pelvis, ureter, or bladder. Previous history of UCa of the bladder, especially with divergent differentiation or variant histology is conceivably a key risk factor for developing subsequent primary UCa of the urethra. These findings are important for the development of surveillance protocols and therapeutic strategies.

SPORADIC CREUTZFELDT-JAKOB DISEASE PRESENTING WITH A ‘JERKY ALIEN LIMB’

2015 ◽  
Vol 86 (11) ◽  
pp. e4.186-e4
Author(s):  
Maruthi Ravi Vinjam ◽  
Steven Butterworth ◽  
Richard Davey
Keyword(s):  
Cognitive Function ◽  
Acute Onset ◽  
Motor Tasks ◽  
Initial Examination ◽  
Alien Limb ◽  
Simple Motor ◽  
History Of ◽  
Jakob Disease ◽  
Time Of Presentation ◽  
Sporadic Cjd

A 64 yr old right-handed mechanic presented with four weeks history of clumsy right hand with ongoing problems at work. He described stiffness and problems with co-ordination. At the time of presentation he was noted to be walking with his right arm behind him and not being aware of this. His initial examination showed Mini Mental Score of 26/30, with ongoing involuntary movements of his right arm (Video).Video shows spontaneous elevation of right arm with occasional myoclonic jerks. Video also demonstrates patient's difficulty in following simple motor tasks and his comments that his arm “has a mind of its own”.His MRI head (Figure 1) showed typical cortical ribboning pattern described in sporadic CJD and CSF Protein for 14-3-3, s100b and RT-QUIC findings were consistent with the diagnosis of CJD.Over next 4 weeks his cognitive function rapidly deteriorated with progressive worsening of his myoclonus. He died 4 weeks after his hospital discharge.There are three broad varieties of alien limb phenomenon (ALP) types described in the literature, frontal, callosal and sensory. Jerky (myoclonic) ALP is well described in patients with CJD, so CJD should be in the differentials in any patient presenting with sub-acute onset of ALP.

Thrombosed arteriovenous malformations of the brain

1982 ◽  
Vol 57 (4) ◽  
pp. 520-526 ◽  
Author(s):  
Robert E. Wharen ◽  
Bernd W. Scheithauer ◽  
Edward R. Laws
Keyword(s):  
Arteriovenous Malformations ◽  
Previous History ◽  
Low Grade ◽  
Spontaneous Thrombosis ◽  
Radiographic Findings ◽  
Content Type ◽  
Seizure Disorders ◽  
History Of ◽  
Mass Lesions ◽  
The Brain

✓ Thrombosed arteriovenous malformations (AVM's) in patients with no previous history of hemorrhage are uncommon but benign lesions that present with clinical and radiographic findings which are often indistinguishable from those of other mass lesions, particularly low-grade gliomas. The authors report seven cases of thrombosed AVM's presenting as intractable seizure disorders in which the radiographic studies had suggested a low-grade glioma. All seven patients are now seizure-free 2 to 40 months postoperatively. The importance of surgical exploration in the management of such patients is emphasized. A review of 32 cases reported in the literature is presented. The reasons why angiography may fail to demonstrate an AVM, and the possible etiologies for the spontaneous thrombosis of an AVM are discussed.

scholarly journals MULTIPLE SYNCHRONOUS SMALL BOWEL ADENOCARCINOMAS IN A CASE OF BOWEL OBSTRUCTION DUE TO FAECALOMAS SECONDARY TO MULTIPLE STRICTURES - A DIAGNOSTIC SURPRISE

2021 ◽  
Vol 9 (11) ◽  
pp. 1194-1198
Author(s):  
Umesh O. Paprunia ◽  
◽  
Girija A. Patil ◽  
Bhushan H. Mahamulkar ◽  
◽  
...  
Keyword(s):  
Small Bowel ◽  
Elderly Patients ◽  
Bowel Obstruction ◽  
Psychiatric Patients ◽  
Previous History ◽  
Low Grade ◽  
Small Bowel Adenocarcinoma ◽  
History Of ◽  
Hirschsprungs Disease ◽  
Single Tumour

Small bowel adenocarcinoma(SBA)is a rare neoplasm presenting usually in elderly patients as a single tumour. Multiple synchronous SBA is unique and difficult to diagnose due to non-specific presentations.Faecalomas have been described in association with Hirschsprungs disease, psychiatric patients, Chagas disease, both inflammatory and neoplastic conditions, and in patients suffering with chronic constipation.We herein report a case of multiple faecalomas secondary to multiple small bowel strictures leading to small bowel obstruction in a patient with previous history of pulmonary tuberculosis.Thepatient was successfully treated surgically. The strictures on histopathology turned out to be low grade adenocarcinoma. Multiple synchronous SBA as well as multiple fecalomas are individually rare entities, and their combined occurrence is even rarer.

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