Tension pneumocephalus: a case report and literature review

Following a bifrontal craniotomy with frontal sinus involvement, a 64-year-old woman developed cerebrospinal fluid (CSF) rhinorrhoea. At a second operation the skull base dural defect was repaired from within the anterior cranial fossa. Ten days later, the patient developed signs and symptoms of raised intracranial pressure and mass effect. Following radiological investigation, the diagnosis of tension pneumocephalus was made and confirmed at the time of decompression. Within 24 h air had reaccumulated under pressure and concurrently an ongoing CSF leak, which until then had been subclinical, became apparent. Definitive repair of the fistula was performed via a frontal sinus approach and the patient made a total recovery. The relationship between CSF leak and delayed onset tension pneumocephalus is discussed, along with other significant issues regarding the diagnosis and management of this uncommon but dangerous condition.     

Tension pneumocephalus after skull base surgery. A case report and review of literature

Pneumocephalus describes the presence of air within the cranial cavity and is often self-limiting. Tension pneumocephalus is a neurosurgical emergency manifested by headaches, seizures, reduced consciousness and even death resulting from raised intracranial pressure. Differentiating both entities clinically is often challenging but crucial. We present a case involving a sixty-year-old male who was transferred to our unit after he collapsed while undergoing rehabilitation. The patient had undergone a combined bifrontal craniotomy and transnasal endoscopic resection of recurrent sinonasal adenocarcinoma with anterior skull base involvement eight days prior. Imaging demonstrated the classic Mt. Fuji sign and a diagnosis of tension pneumocephalus was formed. The patient proceeded for definitive management which included a multi-layered repair of the anterior skull base. The three mechanisms that propose the development of tension pneumocephalus include the ball-valve mechanism, the inverted soda-bottle effect and rarely, infection from gas forming organisms. A review of current literature on PubMed/MEDLINE revealed tension pneumocephalus after skull base surgery to be a rare entity with only eleven cases reported. Most patients achieved complete recovery of symptoms post-treatment. Clinicians should recognise tension pneumocephalus as a potential complication after skull base surgery. Accurate diagnosis requires appreciation of imaging features and a high index of suspicion. Prompt management is imperative to prevent possible devastating outcomes.     

Sneddon's syndrome. Case report and literature review

Diagnostic and therapeutic problems of Sneddon's syndrome are reviewed on the basis of the observation of a pregnant 36-year-old female. She had had Hodgkin's disease stage I, curatively treated when she was 23 years old. She developed cerebral ischemic events, initially ascribed to isolated cerebral angiitis, associated with progressive dermatological lesions (generalised livedo racemosa). A temporal artery biopsy did not reveal giant cell angiitis, while the cutaneous arterioles in a biopsy showed marked intimal proliferation without inflammatory cell infiltration. The literature on Sneddon's syndrome is reviewed.     

Hashimoto's encephalopathy. Case report and literature review

We present a 43-year-old man with recurrent episodes of Hashimoto's encephalopathy who was diagnosed with autoimmune thyroiditis in childhood. Encephalopathy started with subacute dementia followed by extrapyramidal and psychiatric symptoms of insidious onset. He had also status epilepticus which occurred within the first year of the disease. The patient was in euthyreosis, but increased levels of antithyroid antibodies were found. MRI of the brain was normal. Electroencephalography was initially normal and later showed diffuse slowing with generalized theta/delta activity. The cerebrospinal fluid examination revealed a high level of protein which decreased when remission of the disease was achieved. After other etiology was excluded Hashimoto's encephalopathy was diagnosed. Almost complete clinical recovery after steroid administration was observed. Attempts of prednisone withdrawal led to recurrence of neurological and psychiatric symptoms. The diagnosis of Hashimoto's encephalopathy should be considered in each case with subacute encephalopathy associated with high levels of antithyroid antibodies (despite normal thyroid function) and in the absence of other brain diseases.     

Central neurofibromatosis. Case report and review of the literature

An unusual case of central neurofibromatosis associated with a unilateral acoustic neuroma, multiple intracranial meningiomas, spinal neurofibroma and visceral involvement is presented. The progressive clinical course of this syndrome is described and discussed with a review of the relevant literature.     

Hoigne syndrome. Case report and current literature review

Hoigne's syndrome is currently considered a pseudoanaphylactic or pseudoallergic reaction following intramuscular and aqueous procaine penicillin administration. This disorder is characterized predominantly by neuropsychiatric alterations including severe psychomotor agitation with confusion, sensations of disintegration, depersonalization, and derealization, perceived changes of body shape, visual and auditory hallucinations, panic-like anxiety including fear of death as well as alterations of consciousness and seizures. Beside the "classic" immediate manifestation of Hoigne's syndrome, subacute forms as well as reactions of the so-called latent type are also known. Including a typical case report, we present a review of the currently available literature concerning clinical picture, hypotheses on origin, and possible therapy regimens of this underdiagnosed complication of antibiotic penicillin treatment.     

Extradural meningeal cyst. Case report and review of the literature

Extradural meningeal cysts are rare spinal lesions. The clinical presentation is most often characterized by a slowly progressive spastic paraparesis, frequently associated with pain. Adequate drainage of the cyst with obliteration of the neck of the cyst or complete removal of the cyst can bring about a permanent resolution of the symptoms. The use of somatosensory cortical evoked responses also has an important role in the surgical management of this problem.     

Intraventricular tension pneumocephalus after transsphenoidal surgery: a case report and literature review

Tension pneumocephalus is a rare complication of transsphenoidal approaches. The case of a 37 year old woman with a transsphenoidal resection of a pituitary adenoma who presented self-limited rhinoliquorrhea postoperatively is reported. Three days later the patient developed progressive decreased consciousness, amnesia and headache, showing an intraventricular tension pneumocephalus on CT scan. Urgent treatment with bilateral external ventricular drainage and anterior nasal tamponade was performed with good clinical outcome. Later transsphenoidal sealing of the dural defect was achieved without recurrence. Tension pneumocephalus following transsphenoidal surgery usually occurs after the presentation of a cerebrospinal fluid leak due to an incomplete sealing of the sphenoid sinus. The postoperative insertion of a lumbar drainage seems to be a predisposing condition for this complication. The combined approach of tension pneumocephalus with external ventricular drainage and repair of the sphenoid sinus offers optimal results solving the acute neurological deterioration and avoiding recurrence.     

Cryptococcal meningoencephalitis. Case report and review of Italian literature

A case of cryptococcal meningoencephalitis, as presented by a hypertensive hydrocephalus, is described. To our knowledge, this is the 24th case described in Italy since 1953. The diagnosis was made with ventricular fluid examination: the patient was successfully treated with amphotericin B and 5-fluorocytosine, thus avoiding the risks of surgical treatment of hydrocephalus. Early diagnosis and proper therapy are necessary in order to decrease the high lethality of cryptococcosis.     

Ectopic pituitary adenoma. Case report and review of literature

Ectopic pituitary adenomas are very rare neoplasms. They are usually located in the sphenoid sinus or nasopharynx. Ectopic tumors derive from a residual pituitary fragment in the craniopharyngeal duct. They are similar in morphology, immunohistochemistry and hormonal activity to the typical pituitary adenoma. One third ectopic pituitary tumors reported in the literature are endocrine inactive, while the remaining ones indicate a hormonal activity and usually secrete ACTH, causing the Cushing disease. About 50% of pituitary adenomas are located at the skull base and present as aggressive neoplasms. The computed axial tomography (CAT) and magnetic resonance imaging (MRI) are very useful in revealing the presence of these tumors. Preoperative diagnosis of ectopic pituitary adenomas located at the skull base is difficult. Diagnostic procedures usually suggest another type of neoplasm, e.g. chordoma, nasopharyngeal carcinoma, or a metastatic tumor. Pathological examination including immunohistochemical studies of neuroendocrine markers is important in establishing the diagnosis. A unique case of ectopic, nonfunctioning pituitary adenoma involving the sphenoid sinus and nasopharynx and causing palsy of cranial nerves is presented. MRI examination revealed the presence of a large invasive tumor. The neoplasm was partially resected by transnasal approach. The diagnosis was confirmed by a pathological examination including immunohistochemical smears.     

Intramedullary spinal cysticercosis. Case report and review of literature

Intramedullary spinal cysticercosis is extremely uncommon; only 29 cases have been reported previously. A case of solitary intramedullary spinal cysticercosis is described. Factors accounting for the rarity of intramedullary cysticerci are reviewed and the probability of reaching a preoperative diagnosis is discussed. This is the fifth such case reported from India.     

Third ventricle glioblastoma. Case report and review of literature

A glioblastoma presenting as a solitary third ventricle mass is exceptional. CASE DESCRIPTION: We report the case of a 29-year-old woman who lost consciousness, was taken to hospital and referred a previous history of depression and diabetes insipidus. Magnetic resonance imaging study revealed a heterogeneous anterior third ventricle mass with ring enhancement after contrast administration. It was approached and subtotally resected by a transcortical-transventricular route and histological diagnosis proved it to be a glioblastoma. There are only two other similar well-described cases and another nine have been previously reported in surgical series of high grade gliomas and glioblastomas. The possible origin of this lesion is discussed.     

Acute confusional migraine. Case report and review of literature

The history of a girl is described who experienced two separate episodes of acute confusion with agitation. The first occurred at the age of four after a mild head injury, the second recently at the age of thirteen after prolonged physical effort. EEG's performed during both episodes showed marked diffuse slowing. Both clinical picture and EEG normalized rapidly. Clinical features and prompt recovery made the diagnosis 'acute confusional migraine' most likely. The relevant literature and importance of recognizing the syndrome are discussed     

Dural metastases mimicking meningioma. Case report and review of the literature.

The typical appearance of meningioma on CT and MRI is well known. Particularly in the elderly, the imaging appearance is sometimes considered diagnostic of these benign tumours without histopathological confirmation. However, other more aggressive neoplasms can present with a classical CT and MRI appearance of meningioma, indicating the need for histopathological confirmation wherever possible. We report a case of dural metastases which, on both pre-operative CT and MRI and at surgery, had the typical appearance of a falcine meningioma. Histopathology and immunohistochemistry revealed adenocarcinoma of renal cell origin, and the renal primary was identified on subsequent abdominal investigation. The literature regarding dural metastases is reviewed. To our knowledge, this is the first reported case of a renal carcinoma metastasizing directly to the dura. Although rare, dural metastases can mimic meningioma, and this needs to be considered if conservative therapy or radiosurgery are to be offered to a patient with radiological diagnosis of meningioma.     

Intra-spinal clear cell meningioma. Case report and literature review

Clear cell meningioma is a rare variety of meningiomas, occurring frequently at the cerebellopontine angle and spinal canal. A case of a female patient 28 years of age with a complete cauda equina syndrome was described. Magnetic resonance imaging of lumbo-sacral spine revealed a mass lesion occupying the spinal canal from L3 to S1. Subtotal resection was performed and pathological pathological examination revealed a clear cell meningioma. Radiotherapy was indicated, however, it was delayed because we discovered that the patient was pregnant. The recurrence was clinically evident at seven months and a new surgical resection previous complementary radiotherapy was necessary. A systematic review of literature was performed, exposing the behavior of clear cell meningioma in the spinal canal.     

Giant cerebral cavernoma. Case report with literature review

Giant cerebral cavernoma is a rare malformation classified as a brain occult vascular lesion. We report a case of initially misdiagnosed giant cavernous angioma revealed by a spontaneous intracerebral hemorrhage. A 40-year-old woman was admitted with right hemiplegia and altered consciousness occurring 3 days prior to presentation. A non-contrast CT scan showed a left parietal gross hematoma, and she was immediately taken to the operating room for emergency surgery. However, the procedure was interrupted by massive bleeding, and therefore a vascular lesion was suspected. Magnetic resonance images suggested a giant cavernous malformation. Afterward, she underwent total removal of this lesion. The postoperative period was uneventful, and histopathological examination confirmed the diagnosis of cavernous angioma. Thus, the differential diagnosis of gross spontaneous intracerebral hematoma should include giant cavernous angioma.     

Primary intradural extramedullary hydatidosis. Case report and review of the literature

The authors report a case of cauda equina compression by intradural hydatid cyst. An 18-year-old man presented with paraparesis and sphincter dysfunction. MRI showed an intradural cystic lesion extending from L1 to L2 with low signal intensity on T1 and high signal intensity on T2. The cyst was removed after laminectomy and opening of the dural sac. Histological and parasitic examinations confirmed a diagnosis of hydatid cyst. The patient improved progressively after surgery. The similar 22 cases of intradural extramedullary hydatid disease reported in the literature were reviewed. All spinal areas were involved, with a predilection for the thoracic region. Neurological complications were usual with rapid spinal cord compression in this rare form of hydatid disease. The treatment was by surgery with a favourable outcome compared to the classic hydatid cyst of the spine.     

Lipoastrocytoma: Case report and review of the literature

Lipoastrocytoma is an extremely rare tumor, with only six cases described. We report the case of an astrocytoma involving the upper part of the cerebellar‐pontine angle and the right portion of the clivus starting from the brainstem with a diffuse lipomatous component in a 39 year‐old man. The patient was admitted with headache of 1 year's duration and diplopia over the previous 3 months. MRI revealed a ponto‐cerebellar lesion that showed irregular enhancement after contrast administration. Subtotal excision of the tumor was accomplished. Adjuvant chemotherapy and radiation therapy were not administered. Histologically the tumor showed the classical histology of low‐grade astrocytoma and a portion of the lesion was composed of lipid‐laden cells. Immunohistochemistry for glial fibrillary acid and S‐100 proteins clearly demonstrated the glial nature of these cells. Ki‐67/Mib‐1 labeling index was low (2%). The patient remains in good neurological conditions after 10 months. Our case has a benign postoperative behavior, also after subtotal excision, with restrictions due to the short follow‐up. It is important to record each new case of this rare tumor to produce a better characterization of this lesion.