Spontaneous intracranial hypotension is an increasingly recognized cause of postural headache. However, appropriate management of obtundation caused by intracranial hypotension is not well defined.A 43-year-old man presented with postural headache followed by rapid decline in mental status. Imaging findings were consistent with the diagnosis of spontaneous intracranial hypotension, with bilateral subdural hematomas, pachymeningeal enhancement, and caudal displacement of posterior fossa structures and optic chiasm.Despite treatment with lumbar epidural blood patch, worsening stupor necessitated intubation and mechanical ventilation. Contrast-enhanced magnetic resonance imaging and computed tomographic myelography of the spine failed to demonstrate the site of cerebrospinal fluid fistula. The enlarging subdural fluid collections were drained, and a ventriculostomy was performed. Postoperatively, the patient remained semicomatose. To restore intraspinal and intracranial pressures, intrathecal infusion of saline was initiated. After several hours of lumbar saline infusion, lumbar and intracranial pressures normalized, and the patient's stupor resolved rapidly. Repeat computed tomographic myelography accomplished via C1-C2 puncture demonstrated a large ventrolateral T1-T3 leak, which was treated successfully with a thoracic epidural blood patch. Follow-up magnetic resonance imaging demonstrated resolution of intracranial hypotension, and the patient was discharged in excellent condition.Spontaneous intracranial hypotension may cause a decline of mental status and require lumbar intrathecal saline infusion to arrest or reverse impending central (transtentorial) herniation. This case demonstrates the use of simultaneous monitoring of lumbar and intracranial pressures to appropriately titrate the infusion and document resolution of intracranial hypotension. Maneuvers aimed at sealing the cerebrospinal fluid fistula then can be performed in a less emergent fashion after the patient's mental status has stabilized.
Optical coherence tomography (OCT) is a biomedical imaging technique with high spatial-temporal resolution. With its minimally invasive approach OCT has been used extensively in ophthalmology, dermatology, and gastroenterology1-3. Using a thinned-skull cortical window (TSCW), we employ spectral-domain OCT (SD-OCT) modality as a tool to image the cortex in vivo. Commonly, an opened-skull has been used for neuro-imaging as it provides more versatility, however, a TSCW approach is less invasive and is an effective mean for long term imaging in neuropathology studies. Here, we present a method of creating a TSCW in a mouse model for in vivo OCT imaging of the cerebral cortex.
Abstract Fragile X Syndrome (FXS) is a monogenetic form of intellectual disability and autism in which well-established knockout (KO) animal models point to neuronal hyperexcitability and abnormal gamma-frequency physiology as a basis for key disorder features. Translating these findings into patients may identify tractable treatment targets. Using source modeling of resting-state electroencephalography data, we report novel findings in FXS, including 1) increases in localized gamma activity, 2) pervasive changes of theta/alpha activity, indicative of disrupted thalamocortical modulation coupled with elevated gamma power, 3) stepwise moderation of these abnormalities based on female sex, and 4) relationship of this physiology to intellectual disability and neuropsychiatric symptoms. Our observations extend findings in Fmr1 -/- KO mice to patients with FXS and raise a novel role for disrupted thalamocortical modulation in local hyperexcitability. This systems-level mechanism has received limited preclinical attention but has significant implications for understanding fundamental disease mechanisms.