scholarly journals IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Xiaohai Liu ◽  
Renzhi Wang ◽  
Mingchu Li ◽  
Ge Chen

IgG4-related inflammatory pseudotumors are very uncommon and are characterized histologically by the presence of inflammatory swellings with increasing IgG4-positive plasma cells and lymphocytes infiltrating the tissues. As reports of intracranial IgG4-related pseudotumors are very rare, we report a case of an IgG4-related inflammatory pseudotumor involving the clivus mimicking meningioma. A 46-year-old male presented with intermittent headache for 2 years and a sudden onset of dysphagia and dysphonia of 7 days’ duration along with lower limb weakness. Enhanced magnetic resonance imaging (MRI) of the skull base revealed an isointense signal on T1- and T2-weighted images from an enhanced mass located at the middle of the upper clivus region, for which a meningioma was highly suspected. Then, an endoscopic transsphenoidal approach was adopted and the lesion was partially resected, as the subdural extra-axial lesion was found to be very tough and firm, exhibiting fibrous scarring attaching to the brain stem and basal artery. After the surgery, brain stem and posterior cranial nerve decompression was achieved, and the patient’s symptoms, such as dysphagia, dysphonia and lower limb weakness, improved. Pathological findings showed many IgG4-positive plasma cells and lymphocytes surrounded by collagen-rich fibers. The patient was sent to the rheumatology department for further glucocorticoids after the diagnosis of an IgG4-related inflammatory pseudotumor was made. This case highlights the importance of considering IgG4-related inflammatory pseudotumors as a differential diagnosis in patients with lesions involving the clivus presenting with a sudden onset of symptoms of dysphagia and dysphonia along with lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumors are etiologically enigmatic and unpredictable, and total resection might not be warranted. Glucocorticoids are usually the first line of treatment after diagnosis.

2018 ◽  
Vol 89 (10) ◽  
pp. A2.2-A2
Author(s):  
Salini Sumangala ◽  
Ben Simpson ◽  
Jithin George

A 47 year old with subacute onset of walking difficulties, asymmetric lower limb and facial weakness and areflexia was admitted as a suspected case of Guillain-Barre syndrome. Clinical examination revealed left lower motor neuron facial weakness, right upper limb and lower limb weakness and a suspended area of pain and allodynia at T7. Structural imaging of the brain and spine was normal. CSF protein was elevated with pleocytosis. Nerve conduction studies was suggestive of proximal demyelinating polyradicular neuropathy. The clinical suspicion of neuroborreliosis was confirmed in CSF with Borrelia VlsE antigen positivity and serum Borrelia Burgdorferi IgG EIA positivity. The patient had no recollection of tick exposure but did recall a presumed horsefly bite on the forearm two weeks earlier of uncertain significance. This is a case of Bannwarth syndrome - meningoradiculoneuritis due to neuroborreliosis endemic in Northern Europe. Our patient was treated with Ceftriaxone with rapid improvement of symptoms. This case highlights the importance of careful history taking including ascertainment of travel to Borrelia endemic areas and recognition of this eminently treatable meningoradiculoneuropathy.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Albaraa T. Alfaraidi ◽  
Abdulaziz A. Alqarni ◽  
Mohammed T. Aqeel ◽  
Turki A. Albalawi ◽  
Ahmed S. Hejazi

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that causes systemic inflammation which can progress to multiorgan failure and death. Symptoms and signs commonly seen in HLH include high fever, hepatosplenomegaly, pancytopenia, and hypertriglyceridemia. This report describes the 8-month clinical course of a 17-year-old male with G6PD deficiency who presented with intermittent high fever of unknown origin for 8 months accompanied by pancytopenia and bilateral lower limb weakness. A pathogenic homozygous missense mutation (c.1081A > T p.(Arg361Trp)) in the PRF1 gene was detected by whole exome sequencing (WES). The brain and the whole spine MRI showed leptomeningeal enhancement at different levels involving both the brain and the spine. Therefore, a diagnosis of familial HLH type 2 with CNS involvement was confirmed. Accordingly, treatment with dexamethasone, cyclosporin, and etoposide in addition to intrathecal methotrexate and hydrocortisone was given. The patient showed a dramatic response with significant neurological improvement of the bilateral lower limb weakness. Genetic analysis has helped the patient’s family with appropriate genetic counselling. This case highlights the importance of immediate treatment with immunosuppressants and the high clinical suspicion of physicians regarding HLH in areas where consanguinity is common.


2019 ◽  
Vol 12 (12) ◽  
pp. e232967 ◽  
Author(s):  
Cathy Rowland ◽  
Daniel Kane ◽  
Maeve Eogan

A 34-year-old primiparous woman presented in spontaneous labour and had an unassisted vaginal birth of a 3.5 kg infant. Postnatally, the patient experienced lower limb weakness and was unable to mobilise unassisted. A diagnosis of postpartum femoral neuropathy was made. Full recovery of normal motor function was not achieved until 5 months postpartum. She returned in her next pregnancy, seeking advice on how to avoid this complication from reoccurring. It was decided that an elective caesarean section was an appropriate mode of delivery, which she underwent at 39 weeks without complication and without recurrence of the femoral neuropathy.


Author(s):  
Akbar Hojjati Najafabadi ◽  
Saeid Amini ◽  
Farzam Farahmand

Physical problems caused by fractures, aging, stroke, and accidents can reduce foot power; these, in the long term, can dwindle the muscles of the waist, thighs, and legs. These conditions provide the basis for the invalidism of the harmed people. In this study, a saddle-walker was designed and evaluated to help people suffering from spinal cord injury and patients with lower limb weakness. This S-AD works based on body weight support against the previously report designs. This saddle-walker consisted of a non-powered four-wheel walker helping to walk and a powered mechanism for the sit-to-stand (STS) transfer. A set of experiments were done on the STS in the use of the standard walker and the saddle-assistive device(S-AD). A comparison of the results showed that this device could reduce the vertical ground reaction force (GRF) of the legs up to 70%. Using this device could help a wide range of patients with lower limb weakness and SCI patients in changing from sitting to standing.


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