A case of spontaneous intracranial hypotension in a 45-year-old male with headache, behavior changes and altered mental status.

Spontaneous intracranial hypotension is a rare disease that results from low cerebrospinal fluid (CSF) volume caused by leakage of CSF from the spine in the absence of lumbar puncture, spine surgery, or intervention. The most common presentation is the headache that is usually but not invariably orthostatic. The underlying pathology is a CSF leak resulting from dural weakness involving the nerve root sleeves, ventral dural tears associated with calcified disc herniations, or CSF venous fistula. In severe cases, neuropsychiatric symptoms and changes in mental status may develop. Some case reports also mention gait disturbances, slurred speech, and urinary incontinence. The constellation of neuropsychiatric symptoms similar to behavior variant frontotemporal dementia in the presence of "brain sag" on MRI is known as frontotemporal brain sagging syndrome, first described by Wicklund et al. (4). The disease presents a diagnostic challenge to the primary care physicians, who are the first to see these patients. Brain and spine imaging is key to diagnoses but requires a high index of suspicion, as very rarely are all classic findings of intracranial hypotension present in the same patient. Here we discuss a case of spontaneous intracranial hypotension in a 45-year-old male patient who presented with headache, drowsiness, incoherent speech, behavior symptoms, and altered mental status.

Pfeiffer syndrome in an adult with previous surgical correction: A case report of CT findings.

Pfeiffer syndrome, affecting roughly 1 in 100,000 individuals is characterized by acrocephalosyndactyly - the premature closure of skull sutures (craniosynostosis). These acrocephalosyndactyly syndromes which are often sporadic de novo but also autosomal dominant in inheritance can be characterized by the fact that they often involve FGFR and TWIST genes. In the presented case, a 27-year old male level three trauma admission displayed skull abnormalities on physical examination that history taking confirmed was the result of pediatric surgically corrected Pfeiffer syndrome. Noncontrast brain CT as part of his trauma work-up revealed characteristic Pfeiffer syndrome imaging pattern of midface hypoplasia, nonvisualization of coronal and sagittal sutures, and a degree of obstructive hydrocephalus. Pfeiffer syndrome requires extensive pediatric surgery often with poor adult follow up. The case presented provides good visualization of characteristic skull abnormalities in a surgically corrected adult. By virtue of imaging an adult, this provides readers with a unique look at the long-term viability and the body's resulting physiological adaptations of the extensive mandatory pediatric surgery these patients undergo.

The Missing Link: A Case of Absent Pituitary Infundibulum and Ectopic Neurohypophysis in a Pediatric Patient with Heterotaxy Syndrome.

We report a case of absent pituitary infundibulum and ectopic neurohypophysis in a 4-year-old patient presenting clinically with hypopituitarism as well as heterotaxy syndrome complicated by global developmental delay and growth retardation. The clinical and laboratory workup of our patient suggested underlying hypopituitarism related to either congenital or acquired pathology, necessitating MRI to distinguish between them. We explain the various structural causes of hypopituitarism and detail how to predict the MRI findings and treatment, based on a fundamental understanding of the anatomy and pathophysiology of the hypothalamic pituitary axis and distinguishing anterior versus posterior pituitary hormone derangements. We also discuss two important theories widely acknowledged in the literature to explain congenital hypopituitarism: 1. Head trauma typically during birth resulting in a stretch injury to the infundibulum. 2. Congenital fetal maldevelopment of midline structures.

The accuracy of [(18)F]fluorodeoxyglucose positron emission tomography as confirmed by biopsy in the diagnosis of spine metastases in a cancer population.

OBJECTIVE: To determine the accuracy of [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET) in the diagnosis of vertebral metastases in patients with cancer using needle-biopsy results and patient follow-up data. METHODS: A retrospective chart review of all patients who underwent a needle biopsy of a spinal lesion and underwent FDG-PET within 6 weeks of the biopsy was performed. Biopsy results and magnetic resonance imaging and computed tomographic appearance of the biopsied lesion, as well as long-term clinical follow-up data, were recorded for each patient. A total of 82 patients with solid tumors and hematological spine metastases were included in this study. RESULTS: The mean standardized uptake values of lesions with active cancer were 7.1 and 2.1 in benign lesions (P < 0.02). In patients with metastatic solid tumors, the mean standardized uptake value was 7.3. Stratification of solid tumor lesions according to whether they had a sclerotic appearance on computed tomographic scans showed that FDG-PET was a significantly better predictor of cancer status in lytic or mixed lesions. In patients with a history of solid tumors, there was 100% concordance between the FDG-PET and needle-biopsy diagnoses in nonsclerotic lesions, when the standardized uptake value cutoff of 2 was used. CONCLUSION: FDG-PET is an accurate screening test for vertebral metastases in cancer patients. It is especially accurate in patients with nonsclerotic vertebral lesions and a history of solid malignancy.

Percutaneous CT-guided biopsy of osseous lesion of the spine in patients with known or suspected malignancy.

BACKGROUND AND PURPOSE: CT-guided spinal biopsy (CTGSB) is considered a safe and accurate procedure. Our goal was to determine the accuracy of a CTGSB of osseous spinal lesions in patients with known or suspected underlying malignancy in reference to major variables such as the radiographic appearance of the biopsied lesion and its location within the spinal column. METHODS: We retrospectively reviewed results of 410 consecutive percutaneous CTGSB procedures of osseous spinal lesions. Biopsy was determined to be adequate if diagnostic tissue was obtained (n = 401) or unsatisfactory (n = 9) if only blood without cellular elements was present on final pathologic-cytologic examination. RESULTS: The level of spinal biopsy was cervical in nine patients (2%), thoracic in 123 (31%), lumbar in 164 (42%), and sacral in 96 (25%). The overall diagnostic accuracy of CTGSB was 89%, with a false-negative rate of 11%. Biopsy of lytic lesions yielded an accurate diagnosis in 93% (220 of 236). Despite technical challenges inherent to biopsy of sclerotic lesions, diagnostic accuracy was 76% (63 of 83), although more importantly, 24% (20 of 83) of the results in sclerotic lesions were falsely negative. CONCLUSION: CTGSB of osseous spinal lesions is an important tool in the workup of patients with known or suspected underlying neoplastic disease. However, a negative result must be confirmed with either close follow-up or, preferably, open biopsy, especially in cases of sclerotic lesions for which diagnostic accuracy is decreased and the false-negative rate is high.