Venous infarction secondary to septic cavernous sinus thrombosis

A 65-year-old woman with poorly controlled diabetes presented bilateral miosis, bilateral abducens nerve palsy, and left hemiparesis. On MRI, cavernous sinus thrombosis, subdural empyema and hemorrhagic infarction in the frontotemporal lobe were detected. Cerebral angiogram revealed filling defect in the cavernous sinus with venous congestion but no involvement of internal carotid artery. Postmortem examination demonstrated hemorrhagic infarction in the right frontotemporal lobe as well as hemorrhagic necrosis of the pituitary gland. It should be noted that venous congestion due to cavernous sinus thrombosis may cause these complications.     

Multiple myeloma presenting as cavernous sinus syndrome

We report on a 70-year-old male who developed cavernous sinus syndrome as the initial symptom of multiple myeloma. He was admitted with diplopia and ptosis in October 2004. The diagnosis of multiple myeloma and cavernous sinus syndrome due to a gross mass at the sinus base were made. Cerebral computed tomography revealed that the lesion occupied the sphenoid sinus and involved the oculomoter nerve. He underwent local irradiation of the mass followed by systemic chemotherapy. The symptoms caused by the mass disappeared after the treatment. Clinicians need to be aware of the rare manifestation of multiple myeloma.     

Cavernous sinus apoplexy presenting isolated sixth cranial nerve palsy: case report

The clinical and radiological findings of a patient affected by apoplexy of an adenoma located within cavernous sinus and presenting isolated Cranial Nerve (CN) VI palsy are reported. The differential diagnosis with other pathologies is debated, and pathogenesis of isolated abducens nerve palsy is investigated. The pertinent literature of this uncommon case of apoplexy is reviewed. A 59-year-old female presented sudden appearance of CN VI palsy associated to violent headache. Endocrinological assessment did not show pituitary insufficiency; MRI depicted an adenoma located within the left CS. She underwent an endoscopic endonasal procedure with extensive opening of the cavernous sinus, occupied by an ischemic adenoma. Afterward, neurological symptoms promptly resolved and the patient was discharged after 5?days. At 3?months follow-up MRI radical tumor removal was assessed, and the patient resulted neurologically intact. The review of the pertinent literature shows that apoplexy of pituitary tumor exclusively located within the Cavernous Sinus is a very uncommon event. The distinctive clinical presentation is represented by the sudden abducens nerve palsy and the absence of pituitary insufficiency and it could be explained by the vulnerability of the nerve along its course within the cavernous sinus, and by the sparing of the sellar content by the adenoma.     

Cavernous sinus apoplexy presenting isolated sixth cranial nerve palsy: case report

The clinical and radiological findings of a patient affected by apoplexy of an adenoma located within cavernous sinus and presenting isolated Cranial Nerve (CN) VI palsy are reported. The differential diagnosis with other pathologies is debated, and pathogenesis of isolated abducens nerve palsy is investigated. The pertinent literature of this uncommon case of apoplexy is reviewed. A 59-year-old female presented sudden appearance of CN VI palsy associated to violent headache. Endocrinological assessment did not show pituitary insufficiency; MRI depicted an adenoma located within the left CS. She underwent an endoscopic endonasal procedure with extensive opening of the cavernous sinus, occupied by an ischemic adenoma. Afterward, neurological symptoms promptly resolved and the patient was discharged after 5 days. At 3 months follow-up MRI radical tumor removal was assessed, and the patient resulted neurologically intact. The review of the pertinent literature shows that apoplexy of pituitary tumor exclusively located within the Cavernous Sinus is a very uncommon event. The distinctive clinical presentation is represented by the sudden abducens nerve palsy and the absence of pituitary insufficiency and it could be explained by the vulnerability of the nerve along its course within the cavernous sinus, and by the sparing of the sellar content by the adenoma.

     

Idiopathic giant cell granulomatous hypophysitis with hypopituitarism, right abducens nerve paresis and masked diabetes insipidus

A 38-year-old man presented with headache, fever, and double vision associated with right abducens nerve paresis. He had neither nuchal rigidity nor visual field defect. Laboratory data revealed elevated erythrocyte sedimentation rate (ESR), eosinophilia, and lymphocytic pleocytosis in the cerebrospinal fluid (CSF). Provocation tests of pituitary hormones showed partial hypopituitarism. Magnetic resonance imaging (MRI) revealed swelling of the hypophysis and a mass lesion expanding into the right cavernous sinus. The supplement dose of dexamethasone for hypothalamic hypocortisolism manifested diabetes insipidus. Biopsy, carried out through the transsphenoidal approach, revealed giant cell granuloma. Systemic granulomatous diseases were ruled out, and the lesion was considered to be idiopathic giant cell granulomatous hypophysitis. Right abducens nerve paresis, diabetes insipidus and dysfunction of the anterior lobe were amended by the treatment with prednisolone for 4 months, and findings of the pituitary gland and stalk were normalized. The present case shows that glucocorticoid has an effect on amendment of idiopathic giant cell granulomatous hypophysitis.     

Hepatocellular carcinoma metastasizing to the skull base involving multiple cranial nerves

INTRODUCTION Hepatocellular carcinoma (HCC), the most common primary tumor of the liver, is estimated to cause more than a quarter of a million deaths throughout the world each year. Metastasis is one of the most significant factors affecting prognosis. I…     

A case of non-small-cell lung cancer with intramedullary spinal cord metastasis diagnosed pre-mortem]

The patient was a 54-year-old man who in May 1999 received a diagnosis of squamous cell carcinoma, T4 N2 M1, stage IV. Systemic chemotherapy and stereotactic radiosurgery were performed only to result in further progression of the disease. In August 1999, he experienced gait disturbance due to lumbar pain. Rehabilitation improved the gait disturbance and he was discharged. In October, since the pain reappeared and there was numbness in the right leg, he was readmitted. Brain MRI revealed multiple brain metastasis and whole brain irradiation was performed. But his symptoms deteriorated, and palsy of the right leg ensued. Later, bladder dysfunction also developed. Since spinal cord MRI revealed intramedullary metastasis at Th 12 and L1 levels, we performed radiotherapy for the lumbar medullary lesion, together with systemic chemotherapy. After chemoradiotherapy the tumor size decreased and the pain improved. Cases of lung cancer with intramedullary metastasis are rare, especially those diagnosed before death.     

Hepatocellular carcinoma metastasis to the brain mimicking primary pituitary tumor around the sella turcica

We report on two cases of hepatocellular carcinoma (HCC) with metastasis to the cavernous sinus and sphenoid sinus. Both cases presented with diplopia and retro-orbital headache and both underwent surgery for a primary pituitary gland tumor. After surgery, both cases were diagnosed with metastases from HCC. Case 1 was a 67-year-old male with a history of HCC who was referred to our hospital for pituitary tumor surgery. The tumor appeared to be in the sella turcica and to invade the sphenoid sinus and right cavernous sinus. Transnasal transsphenoidal surgery (TSS) was performed. The tumor was postoperatively diagnosed by histology to be a metastatic pituitary tumor from HCC. Radiotherapy was administered to the metastatic site. Case 2 was a 58-year-old male with a history of TSS for a pituitary tumor 16 years previously. He was referred to our hospital for TSS for a recurrent pituitary adenoma. TSS was performed twice in 3 months. During a preoperative general examination, HCC and chronic hepatitis B were revealed. TSS was performed initially, followed by arterial infusion chemotherapy. After TSS, the pituitary tumor was diagnosed by histology to be a metastasis from HCC. As with Case 1, radiotherapy was administered to the metastasis. Most tumors in the sella turcica are pituitary adenomas, although some cases of metastatic pituitary tumors and skull base metastases have been reported. Distant metastases generally have a poor prognosis; however, surgery to the metastatic site can effectively control symptoms caused by the metastatic tumor.     

Rathke’s cleft cyst presenting as bilateral abducens nerve palsy

We present a patient with a Rathke’s cleft cyst who presented with rapidly progressive bilateral 6th nerve palsy. A 20-year-old woman with a history of cleft palate, hypertension, and hydronephrotic kidneys presented with a one month history of headache, associated with dizziness and diplopia on horizontal gaze. Examination was significant for profound bilateral 6th nerve palsies. Magnetic resonance imaging showed a hypodense mass that filled the sella and compressed the right cavernous sinus without contacting the optic chiasm. Pituitary function was normal. An endoscopic, transnasal transsphenoidal resection of the lesion was performed; microscopic examination revealed a Rathke’s cleft cyst. Surgical excision resulted in near complete resolution of the bilateral 6th nerve palsy. Rathke’s cleft cysts are an unusual cause of bilateral sixth nerve palsy and represent a potential cause of cranial neuropathy.     

Case of Heerfordt's syndrome with prolonged peripheral nerve involvement]

A 28-year-old man complaining of myiodesopsia was given a diagnosis of uveitis. Subsequently he complained facial nerve palsy and enlargement of parotid gland. Heerfordt's syndrome was diagnosed based on the results of several examinations. Facial nerve palsy, enlargement of the parotid gland and uveitis were improved by systemic corticosteroid therapy. At present he is receiving systemic corticosteroid therapy, but numbness in the mouth, thought to be the involvement of the trigeminal nerve, remains. Systemic corticosteroid therapy is usually effective for most cases with Heerford's syndrome. On the other hand, there are some cases with the prolonged peripheral nerve involvement despite systemic corticosteroid therapy, as seen in this case. If peripheral nerve involvement is prolonged, it is necessary to consider small-fibre neuropathy as one possible cause.     

Primary central nervous system lymphoma mimicking pituitary apoplexy: case report

Lymphoma involving the pituitary gland is very rare and usually results from metastatic spread of systemic lymphoma. We present a case of primary central nervous system (CNS) large B cell lymphoma that manifested as pituitary apoplexy. A 45-year-old woman presented with headache, and then rapidly developed a third nerve palsy and bitemporal hemianopsia. Imaging suggested a pituitary macroadenoma, with spontaneous necrosis, extending into the suprasellar region, compressing the optic chiasm and invading the right cavernous sinus. The patient underwent transsphenoidal resection which revealed a vascular, firm tumor. An aggressive decompression of the optic chiasm was performed with complete resolution of both visual fields and third nerve palsy. Final pathology showed B cell lymphoma. Systemic work-up including bone marrow aspiration and CSF studies showed no other foci of lymphoma, and the patient was HIV-negative. Chemotherapy with methotrexate, vincristine, procarbazine, and dexamethasone was administered for primary CNS lymphoma. This is an uncommon diagnosis of which the clinician should be aware in order to tailor surgical intervention and provide early institution of proper therapy.     

多发性脑神经损害68例临床病因分析

目的提高对多发性脑神经损害的临床特点和病因的认识,以达到早诊断,早治疗,提高诊断率。方法从主要症状、辅助检查等方面对我院多发性脑神经损害68例患者的临床资料进行回顾性分析。结果病因依次是:鼻咽癌脑神经侵害19例,糖尿病周围神经病12例,多发性脑神经炎10例,颅内动脉瘤6例,海绵窦动静脉瘘4例,海绵窦血栓形成、脑干脑炎各3例,结核性脑膜炎、痛性眼肌麻痹(Tolosa-hunt综合征)、脑干梗死、格林-巴利综合征各2例,多发性硬化、新型隐球菌性脑膜炎、脑干肿瘤各1例。结论主要病因:鼻咽癌脑神经侵害、糖尿病周围神经病、多发性脑神经炎、颅内动脉瘤。     

Peptide receptor radionuclide therapy in a patient with disabling non-functioning pituitary adenoma

Non-functioning pituitary adenoma (NFPA) with higher proliferation index (WHO II) are often a therapeutical challenge. Low somatostatin receptor expression in these tumors usually prevents a treatment with somatostatin analogs. In 1996, a 55-year-old patient was referred due to right-sided headache. A pituitary macroadenoma with infiltration into the right cavernous sinus was diagnosed. There was no visual field deficit and the clinical and biochemical work up was consistent with a NFPA. The patient underwent transsphenoidal surgery. Residual adenoma remained in the right cavernous sinus. Histologically, a null-cell adenoma with a high proliferation index was documented (MIB-1: 11.6 %, WHO II). Somatostatin receptor autoradiography was performed in the surgical specimen showing a homogenous expression of sst₂ receptors. Radiosurgery was completed with stable disease for 8 years. In 2004, the patient was diagnosed with an incomplete palsy of the right oculomotorius nerve and a significant increase in the volume of the adenoma in the right cavernous sinus. After a positive Octreoscan^® the patient consented to an experimental therapy approach using Lutetium DOTATOC (3 × 200 mCi). The palsy of the oculomotorius nerve improved and remained stable until today (March 2013), the follow-up MRI scans demonstrated stable disease. This is the first case of a patient with a NFPA (WHO II) in whom PRRT successfully improved the local complications of the tumor for more than 8 years after ineffective surgery and gamma knife therapy. The determination of sst₂ in vitro using autoradiography and in vivo by Octreoscan was instrumental to administer this therapy in a challenging situation.     

Intracavernous carotid aneurysm--an unusual cause of isolated abducens nerve palsy

We discuss the case of a 59-year-old woman who presented to the emergency department with complaints of diplopia for five days. Physical examination disclosed an isolated complete right abducens nerve palsy. Angiography revealed a giant aneurysm from the postcavernous portion of the right internal carotid artery. This case illustrates an unusual etiology of a unilateral abducens nerve palsy.     

Gasperini syndrome, a report of two cases

We report 2 cases of Gasperini syndrome and consider them with the 11 previously reported cases to describe the clinical characteristics of this rare syndrome: Core neurological signs are peripheral facial nerve palsy and abducens nerve palsy of the affected side: Among all cases, imaging demonstrated a small lesion in the mediolateral tegmental pons (10/13 cases of microinfarction; 2/13 cases of microbleeding). We found that the responsible artery in ischemic Gasperini syndrome is mainly the long circumferential branch of the anterior inferior cerebellar artery; Case 1 is the first case thought to be caused by infarction of the basilar artery's paramedian branch.     

A case of primary lung cancer with initial symptoms due to orbital metastases]

We encountered a case of lung cancer in which symptoms due to orbital metastasis were recognized. A 55-year-old man presented with a chief complaint of double vision. Orbital MR image demonstrated a right intraorbital mass with bone destruction, which resulted in oculomotor nerve palsy and optic nerve disturbance. Chest CT scan showed a 4 cm mass in the right S6, which was diagnosed on biopsy as a poorly differentiated adenocarcinoma. A whole-body scintigram revealed multiple bone metastases: the right orbital wall, the lower cervical spine, the left knee joint, and so on. Based on the clinical findings, we believed that the orbital tumor was a metastasis from the lung. Systemic chemotherapy and irradiation of the right orbital tumor and the left knee joint were performed. Though a favorable response was achieved in ocular movement, the patient died 3 months after initial treatment because of progression of the primary lesion. Including this case, seventeen reported cases in which lung cancer metastasized to the orbit in Japan were also reviewed.     

Tumors metastatic to the pituitary gland: case report and literature review

Tumors metastatic to the pituitary gland are an unusual complication of systemic cancer typically seen in elderly patients with diffuse malignant disease. Breast and lung are the commonest sites of the primary tumor, whereas diabetes insipidus is the most frequent symptom at presentation. Their rarity and usually indolent course, as well as the lack of specific clinical and radiological features, impede their differentiation from other more common sellar area lesions, particularly when history of malignancy is absent. Management of these patients may also be very difficult because the prognosis depends on the course of the primary neoplasm. A 68-yr-old man, with no history of malignancy, presented with recent onset of hypopituitarism, mild diabetes insipidus, headaches, left oculomotor nerve palsy, and progressive bilateral deterioration of visual acuity and visual fields. Magnetic resonance imaging revealed a large sellar mass compressing the optic chiasm and invading the left cavernous sinus, whereas a prolactin elevation at 438.6 ng/ml (19.73 nmol/liter) was noted. Decompression of the sellar region was attempted, and pathology disclosed a metastatic hepatocellular carcinoma. On postoperative investigation, primary liver tumor was identified and confirmed by biopsy. The patient improved transiently but died 3 months after diagnosis because of deterioration of the liver disease. The relevant literature is reviewed in light of this unusual case, illustrating the problems in the diagnosis and management of patients with metastasis to the pituitary.     

Case report: one-and-a-half-syndrome and tuberculosis of the pons in a patient with AIDS

A patient with advanced AIDS presented with right conjugate gaze palsy and impaired adduction on left gaze (the one-and-a-half syndrome). The responsible pontine lesion involved the ipsilateral abducens nucleus and the adjacent medial longitudinal fasiculus (MLF), as demonstrated by magnetic resonance imaging (MRI). Tuberculosis (TB) was the etiology of the brainstem lesion. The patient had complete recovery after anti-tuberculosis treatment. The nuclear location of the tuberculoma is unusual and the reversal of this neuro-ophthalmologic syndrome is noteworthy.     

Benign recurrent unilateral abducens nerve palsy

Abstract The recurrence of an isolated unilateral left abducens nerve palsy on five occasions over five years is described in an adult. No evidence of any systemic or intracranial disorder has been identified. The aetiology remains uncertain and the clinical course is benign. The diagnosis of ‘benign’ abducens nerve palsy is one of exclusion and is made retrospectively after an adequate period of serial evaluations.     

Abducens nerve palsy in a girl with incomplete Kawasaki disease

Kawasaki disease (KD) is a systemic vasculitis that can involve the nervous system, including the cranial nerves. Central nervous system findings, especially irritability, lethargy, and aseptic meningitis, occur in 1–30 % of KD patients (1). Cranial nerve palsies are seen rarely, and abducens nerve palsy has been reported in only three children. We describe a 2.5-year-old girl with incomplete KD who developed transient abducens nerve palsy after intravenous immunoglobulin (IVIG) treatment.