Diagnosis and Treatment of the Central Disorders of Hypersomnolence

The central disorders of hypersomnolence (CDH) are lifelong conditions that cause an irrepressible need for sleep during waking hours. Some of these disorders are due to medications or other conditions, but narcolepsy type 1 (NT1), narcolepsy type 2 (NT2), and idiopathic hypersomnia (IH) occur as primary conditions. In each disorder excessive daytime sleepiness (EDS) may be accompanied by ancillary features such as sleep paralysis, hypnagogic/hypnopompic hallucinations, sleep drunkenness, or, in the case of NT1, cataplexy. Polysomnography and a multiple sleep latency test are used for diagnosis. Diagnostic lumbar puncture may be performed to differentiate NT1 from NT2. Although the mainstay of treatment for CDH is currently pharmacotherapy, management may also involve behavioral approaches such as sleep and nap scheduling, dietary modifications, and cognitive-behavioral therapy. Pharmacologic treatment can include traditional medications such as modafinil, methylphenidate, or sodium oxybate. Novel therapeutic agents such as pitolisant, clarithromycin, flumazenil, and melatonin may be useful in relieving EDS and associated symptoms.

Normal pressure hydrocephalus: Diagnostic delay

Introduction: Normotensive hydrocephalus is a differential diagnosis in the evaluation of the dementia syndrome. The diagnostic protocols would allow detecting this pathology that has more effective treatment than other dementias.Objective: To describe a population with clinical suspicion of normal pressure hydrocephalus evaluated in a Colombian psychiatric hospital and discuss the possible reasons for its diagnostic and therapeutic delay.Materials and methods: We conducted a retrospective study of medical records to identify patients with suspected normal pressure hydrocephalus during a 5-year period.Results: Thirty-five patients with suspected normal pressure hydrocephalus underwent diagnostic lumbar puncture and five of them were considered candidates for a peritonealvenous shunt, but none underwent this surgical procedure. After three to six months of the lumbar puncture, the gait pattern improved in 22.8% of the patients, cognition in 22.8%, and sphincter control in 11.4%. Improvement was not sustained in the long term (1 year) in any of them.Conclusion: This study suggests the poor implementation of the protocols for evaluating patients with cognitive deficits and delays in the diagnosis of normal pressure hydrocephalus. A small number of patients were identified as candidates for treatment.Normal pressure hydrocephalus is a potentially reversible clinical entity with the placement of a peritoneal ventricular shunt, but delays in diagnosis and treatment have deleterious consequences for patients and their families.

It Is Just a Blood Patch: Considerations for Patients with Preexisting Intracranial Hypertension

Epidural blood patches are routine procedures interventional pain physicians perform for postdural puncture headaches (PDPH), whether it be due to the inadvertent wet tap from an epidural or a diagnostic lumbar puncture. Typically, these patients are relatively healthy and an epidural is relatively straightforward. However, there are cases complicated by a neurologic history such as benign intracranial hypertension. Here, we present a case of a patient with benign intracranial hypertension (BIH) that suffered a postdural puncture headache after a diagnostic lumbar puncture, with no documented opening pressure, continued on acetazolamide. There have only been a small number of documented cases of BIH complicated by PDPH. We discuss the medical management of BIH, how it can exacerbate a postdural puncture headache, our definitive management with an epidural blood patch, and our concerns of rebound intracranial hypertension. We demonstrate that treatment of PDPH in BIH is best managed with image-guided blood patches, with smaller volume of autologous blood, and at a slower rate.

Decreased Cerebrospinal Fluid Orexin-A (Hypocretin-1) Concentrations in Patients after Generalized Convulsive Status Epilepticus

The effects of status epilepticus on the orexin/hypocretin system have yet to be investigated. The present study aimed to assay orexin-A/hypocretin-1 in the cerebrospinal fluid (CSF) of patients after generalized convulsive status epilepticus (GCSE). The study groups included 20 GCSE patients, 24 patients diagnosed with epilepsy but remaining in remission (ER), and 25 normal controls (CTR). Diagnostic lumbar puncture was performed in GCSE patients within 3–10 days of seizure cessation, as well as in the ER and to CTR subjects. Among all GCSE patients, the outcome was graded according to the modified Rankin Scale (mRS) at 1-month follow-up. Orexin-A levels were measured in unextracted CSF samples, using a commercial radioimmunoassay. There was a significant overall difference in median CSF orexin-A concentrations between GCSE, RE, and CTR patients (p < 0.001). The lowest concentrations were noted in the GCSE group compared to ER (p < 0.001) or CTR (p < 0.001). CSF orexin-A levels in GCSE patients inversely correlated with clinical outcome as assessed on the mRS at 1-month follow-up (r = −0.55; p = 0.1). In conclusion, CSF orexin-A levels may serve as a biomarker of increased turn-over of the peptide or post-SE neuronal damage, and implicates the orexin system in the pathogenesis of SE.

Influence of Optic Nerve Appearance on Visual Outcome in Pediatric Idiopathic Intracranial Hypertension

ABSTRACT:Objectives:To determine whether optic disc hemorrhages (ODH) and cotton wool spots (CWS) at presentation are associated with worse visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH).Methods:Retrospective institutional review of 100 eyes of 50 consecutive pediatric IIH patients (aged 16 years or less) who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardized manner. Visual function was assessed using visual acuity (VA) and visual field grade (VFG).Results:At least one ODH was found in 41% of eyes, at least one CWS was found in 27% of eyes, and 20% of eyes had both ODH and CWS. At presentation, Frisén grade was associated with the presence of CWS (p = 0.013) and showed no association with ODH (p = 0.060). When controlling for Frisén grade, ODH and CWS were not associated with worse VA or VFG at final follow-up. Severe ODH were associated with worse VA and VFG at presentation (p < 0.03), but not at final follow-up. Severe CWS at presentation was strongly associated with a worse Humphrey mean deviation of 5.0 dB (95% confidence interval 1.6–8.3) at final follow-up (p = 0.002).Conclusion:When controlling for the severity of papilledema, ODH do not provide any additional prognostic value in pediatric IIH patients. Frisén grade and severe CWS at presentation were independently associated with worse visual outcomes at the final follow-up.

Post-Lumbar Puncture Headache—Does Hydration before Puncture Prevent Headache and Affect Cerebral Blood Flow?

Headache is a common complication after diagnostic lumbar puncture (DLP). We aimed to check whether hydration before puncture influences the incidence of post-lumbar puncture headache (PLPH) and affects cerebral blood flow. Ninety-nine patients enrolled for puncture were assigned to a group with (n = 40) or without hydration (n = 59). In the hydration group, 1000 mL 0.9% NaCl was infused and a minimum of 1500 mL oral fluids was recommended within the 24 h before puncture. A Transcranial Doppler (TCD) was performed before and after DLP. Mean velocity (Vm) and pulsatility index (PI) were measured in the middle cerebral arteries (MCAs). PLPH occurred in 28 patients (28.2%): six (15.4%) from the hydrated and 22 (37.3%) from the non-hydrated group (p < 0.023). Patients with PLPH were younger (p < 0.014) and with headaches in their histories (p < 0.036) compared with the non-headache group. Vm values in both MCAs after puncture were significantly lower than before puncture in all patients. In the PLPH group, Vm in MCAs before puncture were significantly higher and the PI was lower than in the non-headache group. Our findings suggest that hydration of patients within 24 h before puncture prevented PLPH. Twenty-four hours after puncture, significant decreases in Vm were observed in the MCAs of all patients. Low baseline values of PI and high Vm predisposed patients to PLPH.