Infiltrative mass of the skull base and nasopharynx: A diagnostic conundrum

We present a case of idiopathic hypertrophic pachymeningitis (IHP) forming a central skull base mass to illustrate the process required when one investigates such skull base lesions. This is the first description of mass forming or tumefactive IHP extending into the nasopharynx. A 32-year old woman presented with frontal headaches and nasal discharge. She then deteriorated and was admitted with worsening headaches, serosanguinous nasal discharge and bilateral ophthalmoplegia. Multimodality imaging confirmed a destructive central skull base soft tissue mass involving the posterior clivus, floor of sphenoid sinus, nasopharynx and extending into both cavernous sinuses. Unfortunately, the patient continued to deteriorate despite treatment with broad-spectrum antibiotics. Cerebrospinal fluid, blood tests and transnasal biopsies for histology and microbiology did not reveal a diagnosis. Futher neuro-imaging revealed extension of the mass. Early corticosteroid treatment demonstrated radical improvement although an initial reducing regime resulted in significant rebound deterioration. She was stable on discharge with slowly reducing low dose oral prednisolone and azathioprine. We discuss the complexity of this case paying special attention to the process followed in order to arrive at a diagnosis of idiopathic hypertrophic pachymeningitis based on both the clinical progression and the detailed analysis of serial skull base imaging. Knowledge of the potential underlying aetiologies, c...
Source: Annals of Medicine and Surgery - Category: Journals (General) Source Type: research

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Rationale: Cosmetic hyaluronic acid injections for facial soft tissue augmentation are gaining popularity because of their convenience and favorable outcomes. Several associated complications have been described; however, ophthalmic artery occlusion (OAO) combined with superior sagittal sinus thrombosis (SSST) has been rarely reported. Patient concerns: A 21-year-old woman presented with sudden loss of vision and severe pain in the left eye, right upper limb weakness, and headache immediately after hyaluronic acid injection on the left side of her forehead. Diagnosis: Clinical manifestations and multimodal imaging,...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy was recently reported as a spectrum of autoimmune inflammatory central nervous system disorders characterized by the detection of cerebrospinal fluid GFAP immunoglobulin  G. The most common phenotypes of GFAP astrocytopathy are meningoencephalitis with or without myelitis. GFAP astrocytopathy is usually corticosteroid responsive in the acute stage. AbstractAutoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP ‐A) was recently reported as a spectrum of autoimmune inflammatory central nervous system disorders characterized by the detecti...
Source: Clinical and Experimental Neuroimmunology - Category: Neurology Authors: Tags: Review Article Source Type: research
Publication date: Available online 8 August 2019Source: Autoimmunity ReviewsAuthor(s): Alberto Romano, Donato Rigante, Clelia CipollaAbstractThe contribution of autoimmune phenomena to dysfunction of hypophysis or hypothalamus is far to be unraveled and also the specific pathways of hypophysitis are poorly understood until now, mostly for the pediatric population. Primary hypophysitis is rare in children and often regarded as an autoimmune disorder, following the evidence of lymphoplasmacytic infiltration in the pituitary gland, detection of anti-pituitary antibodies (APA) and anti-hypotalamus antibodies (AHA) by indirect ...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
CONCLUSIONS: Neuroimaging is very important in the diagnosis of NBD. We suggest that treatment with immunosuppressants and steroid treatment is essential to decrease the adverse events of corticosteroids in the pediatric population and decrease relapses. Further multicenter studies with prospective follow-up may guide us in better management of these patients. PMID: 31376249 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
ConclusionsThis case provides further evidence for the occurrence of NMOSD with AQP4-IgG-seropositive overlapping anti-NMDAR encephalitis in a Chinese patient. The mechanisms underlying the occurrence of double positive antibodies remains elusive. When NMOSD patients show unusual symptoms (abnormal behavior, prominent psychiatric manifestations, cognitive dysfunction, autonomic dysfunction), or atypical supratentorial lesions, the coexistence of anti-NMDAR encephalitis should be considered.
Source: Multiple Sclerosis and Related Disorders - Category: Neurology Source Type: research
ConclusionThe possibility ofA.  cantonensis infection should be considered in the effective use of albendazole or mebendazole as a treatment. Combining clinical history with laboratory examination is helpful in diagnosingA.  cantonensis infection. A final definite diagnosis can be confirmed by detecting larvae in the CSF. The administration of corticosteroids during pathogen therapy can substantially reduce the therapeutic response.
Source: Brain and Behavior - Category: Neurology Authors: Tags: ORIGINAL RESEARCH Source Type: research
We describe herein a patient with metastatic melanoma who developed epididymo‐orchitis followed by encephalitis while receiving pembrolizumab. The patient developed testicular pain and fever after his third dose of pembrolizumab; ultrasound evaluation demonstrated bilateral epididymo‐orchitis. He then developed headaches, fever, and altered mental status over the next week and was admitted to the hospital. Lumbar puncture revealed inflammatory changes consistent with meningoencephalitis; he did not improve with broad‐spectrum antibiotics, and an extensive workup for infectious etiologies, including cerebrospinal flui...
Source: The Oncologist - Category: Cancer & Oncology Authors: Tags: Melanoma and Cutaneous Malignancies Immune ‐ Related Adverse Events Source Type: research
AbstractPurpose of ReviewChronic headache is a significant worldwide problem despite advances in treatment options. Chronic headaches can have significant a detrimental impact on the activities of daily living.Recent FindingsPatients who do not obtain relief from chronic head and neck pain from conservative treatments are commonly being managed with interventional treatments. These interventional treatment options include botulinum toxin A, injections, local occipital nerve anesthetic and corticosteroid infiltration, occipital nerve subcutaneous stimulation and occipital nerve pulsed radiofrequency (PRF), sphenopalatine ga...
Source: Current Pain and Headache Reports - Category: Neurology Source Type: research
Germinomas are highly immunogenic tumors eliciting a strong peri-tumoral immune response that can spillover into the surrounding healthy tissues. This phenomenon can also occur in intracranial germinomas, manifesting as secondary hypophysitis. Herein, we report a case of 12-year-old-girl presenting with polyuria and polydispsia. She had central diabetes insipidus (CDI) and panhypopituitarism. Imaging revealed a sellar-suprasellar mass with infundibular stalk thickening. Transphenoidal biopsy revealed epitheloid granulomas with immunostaining negative for germinomatous cells. Other causes of hypophysitis were ruled out. Acc...
Source: Neuroendocrinology - Category: Endocrinology Source Type: research
ConclusionWe highlighted how pituitary apoplexy may mimic the clinical findings of an infectious meningoencephalitis, learning points on how clinical assessment can aid earlier diagnosis and the importance of considering this differential diagnosis, particularly with the associated morbidity and mortality.
Source: Interdisciplinary Neurosurgery - Category: Neurosurgery Source Type: research
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