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Normal pressure hydrocephalus (NPH) is divided into idiopathic (iNPH) and secondary causes such as tumors, infections, trauma, or intracranial hemorrhage. More people are now overweight, and BMI correlates with CSF pressure so many patients have higher CSF pressure making the “normal” in NPH a partial misnomer. The dominant symptom is gait impairment often accompanied by cognitive and urinary difficulty. NPH requiring treatment is relatively rare but potentially underdiagnosed. A clinical approach to NPH should start with a differential diagnosis of the gait difficulty, as in early stages, patients may not have the full symptom triad. Those with anomia may have Alzheimer’s dementia or primary progressive aphasia, and there can be additional patients with concomitant NPH and other disorders such as Alzheimer’s, Parkinson’s, and cervical myelopathy that may require treatment of both pathologies. The most useful diagnostic tests in the workup of NPH include magnetic resonance imaging (MRI) of the brain and a CSF drainage trial, with either high volume lumbar puncture or lumbar drain trial, with the videotaping of the gait before and after the CSF removal. The primary treatment remains a CSF diversion procedure with placement of a shunt, and several shunt surgical advances have resulted in less mortality and morbidity.
Cerebrospinal fluid diversion via ventriculoperitoneal (VP) shunting is the mainstay treatment for hydrocephalus. Traditionally, neurosurgeons place the abdominal catheter through abdominal incision, which has become an efficient and standardized technique. This approach carries a 10–30% complication rate, including infection, catheter obstruction, misplacement, hemorrhage and post-operative pain. Laparoscopic assistance (LA) is an emerging alternative to mini-laparotomy, with potential benefits including reduced distal catheter malplacement and shorter operative times. Most investigations on LA are limited to single centers, with no data from Canada. This study aims to identify practice patterns in VP shunting within a Canadian context.
Methods:
Practicing Canadian neurosurgeons were surveyed using a modified Delphi methodology. The survey was distributed to practicing neurosurgeons via the Canadian Neurosurgical Society and the Canadian Neurosurgery Research Collaborative.
Results:
Across two rounds, 36 neurosurgeons participated, representing all provinces with academic neurosurgical centers. Consensus was reached on five out of eight topics. Findings revealed that 65.5% of respondents had experience with LA, and 93% believed it reduced distal catheter malposition. Infection (77.8%), distal catheter obstruction (82.9%) and proximal obstruction (69.4%) were identified as the most common complications, each occurring in up to 10% of cases. In total, 71% anticipated eventual reduced operative times with increased LA experience.
Conclusion:
Canadian neurosurgeons did not identify major barriers to LA beyond personal preference. LA is expected to improve distal catheter placement, though its broader benefits remain uncertain. Patient comorbidities were considered a greater risk factor for complications than surgical technique alone.
Normal pressure hydrocephalus (NPH) is characterized by pathologically enlarged ventricles without elevated cerebrospinal fluid (CSF) pressure along with a triad of clinical symptoms including gait disturbances, urinary incontinence, and cognitive impairment. NPH is evaluated with lumbar drain trials (LDTs) where CSF is removed over several days to determine if patients would benefit from ventricular shunting. Candidate selection and success for these surgeries remains challenging because other diseases such as Alzheimer’s disease (AD) share common features with NPH in cognitive impairment and enlarged ventricles. Prior research has found that 20%-40% of presumed NPH cases have AD pathology as determined by brain biopsy or autopsy. CSF biomarkers of AD can be altered in NPH and are not always conclusive, complicating the interpretation of results when formulating diagnoses and prognoses. Studies to refine the analyses of AD CSF biomarkers in NPH are needed. We aimed to examine the frequency of CSF biomarker results among patients presenting for NPH evaluations with LDTs.
Participants and Methods:
62 patients presented for LDTs upon physician recommendations. CSF specimens were sent to Mayo Clinic Laboratories for Alzheimer Disease Evaluation (ADEVL) that utilizes Elecsys (Lenexa, KS) CSF electrochemiluminescence immunoassays (Roche Diagnostics, Basel, Switzerland) to measure levels of amyloid-beta 42 (Aβ42), total tau (t-tau), and phosphorylatedtau (p-tau), and p-tau:Aβ42 ratio. Results were classified based on interpretation through the Amyloid/Tau/Neurodegeneration (ATN) framework1: 1) AD - biomarker profile consistent with AD pathologic change, 2) non-AD profile - biomarker levels normal or inconsistent with AD pathologic change, or 3) indeterminate - biomarkers were incongruous with only one or two abnormal levels of Aβ42, t-tau, p-tau, or ptau: Aβ42. Indeterminate cases may represent altered protein levels due to CSF dynamics or AD-related pathologic change. In reviewing recent research on CSF dynamics and AD biomarkers in NPH2 a p-tau threshold of 15 pg/mL was derived and implemented such that cases with Aß42 <=1026 pg/mL and p-tau <15 pg/mL were designated as suspected non-AD, and those with Aß42 <=1026 pg/mL and p-tau >15 pg/mL were designated suspected AD.
Results:
Of the 62 LDT cases, 12 (19.35%) were classified as AD, 31 (50%) were indeterminate and 22 (35.48%) were non-AD. Of the 31 indeterminate cases, 21 (33.87% of the overall sample) were suspected non-AD and 7 (11.29% of the full sample) were categorized as suspected AD.
Conclusions:
Our findings show that 20%-30% of patients presenting for LDT showed evidence for AD-type pathologic change, consistent with prior reports of AD pathology in cases of possible NPH. Half of all LDT cases had indeterminate AD CSF biomarker results, the interpretations of which were confounded by the potential alterations of CSF biomarkers levels due to NPH independent of AD. Our findings emphasize the need to establish better approaches to interpreting CSF AD biomarkers in evaluating NPH. Future research should examine the discriminative utility of CSF AD biomarkers and the selected p-tau threshold in indeterminate cases for predicting response to LDT and shunting.
Central nervous system (CNS) infections with the dematiaceous fungus Cladophialophora bantiana (C. bantiana) are extremely rare, with approximately 120 confirmed cases reported as of 2016. C. bantiana is a highly neurotropic and thermotolerant fungus found in soil worldwide. The mode of entry into the CNS remains unknown, but inhalation of fungal spores or subcutaneous trauma have been suggested. Entry of fungal spores can cause cerebral phaeohyphomycosis with the main clinical manifestation of a brain abscess. Symptoms are non-specific and can include headache, fever, hemiparesis, aphasia, visual disturbances, and confusion. C. bantiana cerebral phaeohyphomycosis occurs in both immunocompetent and immunocompromised individuals, with a slightly higher prevalence in immunocompetent males for unknown reasons. Diagnosis is often delayed due to its nonspecific presentation and prevalence in individuals without pre-existing immunological disease. Prognosis is poor, with mortality rates of approximately 70% despite aggressive treatment. Treatment is not standardized but may include several anti-fungal agents and surgical intervention. Case reports documenting the variability seen with cerebral phaeohyphomycosis by C. bantiana can provide valuable insight into this emerging disease. C. bantiana’s neurotropic propensity also warrants cognitive investigation of the disease; however, there are currently limited descriptions of cognitive findings in published case reports of C. bantiana CNS infections.
Participants and Methods:
Here, we describe a case of a 35-year-old immunocompetent, college educated male with a CNS C. bantiana infection, presumably following a fall while biking in Costa Rica. First symptoms included left sided facial palsy, headache, and hand weakness, prompting extensive diagnostic workup, with diagnosis of C. bantiana infection confirmed 8 months after symptom onset. Initial treatment included anti-fungal agents and steroids, but his course of infection was complicated by infectious vasculitis with posterior circulation infarcts and obstructive hydrocephalus requiring ventriculoperitoneal shunt placement two years following the fungal infection diagnosis. The most recent brain MRI revealed encephalomalacia in global periventricular areas, two small masses, likely representing small fungal phlegmons, and enhancing lesions in the upper cervical spinal canal.
Results:
The patient reported cognitive changes following the infarcts and shunt placement including difficulties with spatial navigation, following directions, and articulating thoughts. Memory concerns and lapses in judgment were also reported. Results from a neuropsychological evaluation revealed high average baseline intellectual abilities with decrements in visuospatial processing, processing speed, executive functioning, and aspects of memory stemming from his executive dysfunction. At the time, his cognitive profile suggested parietal and frontosubcortical systems disruption meeting criteria for mild cognitive impairment. Two years later, the patient reported continuing cognitive difficulties prompting a follow-up neuropsychological evaluation. Results were similar to his first evaluation, revealing deficits in aspects of visuospatial processing, decreased verbal and visual learning, bradyphrenia and processing speed deficits, and difficulties with visual planning and organization. Minimal anxiety and depression, but increased apathy and executive dysfunction were endorsed on self-report measures.
Conclusions:
This case report highlights neurological sequela resulting from CNS infection with C. bantiana, -with a course complicated by subsequent strokes, hydrocephalus, and cognitive impairment-, and contributes additional insight into the relatively limited existing reports of an extremely rare but emerging disease.
Nontraumatic intracerebral hemorrhage affects more than one million per year worldwide and accounts for 10% of strokes in the United States. Aneurysm rupture is the most common cause of nontraumatic subarachnoid hemorrhage and is often associated with significant morbidity and mortality. Subdural and epidural hemorrhages may be induced by head trauma and can be life-threatening if not closely monitored and treated. The widespread use of systemic anticoagulation agents for cardiac and prothrombotic conditions raises the risk of all types of intracranial hemorrhage and presents unique challenges in acute management. Treatment of intracranial hemorrhage is geared toward minimizing hematoma expansion, reducing increased intracranial pressure, and surgically treating aneurysms, vascular malformations, and herniation syndromes.
This chapter reviews epidemiological, clinical, and pathological aspects of benign external hydrocephalus, a medical condition that is a risk factor for development of subdural haematoma, and that frequently is mistaken for abusive head trauma (AHT). For infants, there are striking epidemiological similarities regarding gender and age between external hydrocephalus, subdural haematoma (SDH), and AHT/SBS. There is a marked male preponderance, in most infants the symptom debut occurs during the first 6 months, and prematurity appears to be more frequent. External hydrocephalus is known to predispose for development of SDH. Most infants with external hydrocephalus are born with a close-to-normal head circumference (HC) that starts to grow abnormally fast during the first postnatal months; most of these infants reach HC values compatible with hydrocephalus at the age of 2 to 3 months, the peak age at which AHT/SBS most often is diagnosed. Both in infantile SDH and AHT/SBS, the subdural fluid collections appear to be chronic, not acute as one would expect after a traumatic event. There are reasons to assume that external hydrocephalus often has been and will be misdiagnosed as AHT/SBS.
The radiological characteristics of abusive head trauma (AHT) appear to be vaguely defined. A literature search during the period 2008-21 identified 63 articles presenting 172 illustrations with subdural hematomas described as representative of AHT. We evaluated these for signs of benign external hydrocephalus (BEH) or expansive acute subdural haematoma (ASDH). Signs of BEH were widened interhemispheric distance, preserved subarachnoid space and preserved cortical relief despite an overlying SDH, and absence of ventricle compression or midline shift. Signs of an ASDH were hyperattenuating SDH combined with compression of the subarachnoid space, the cortical relief, and ventricles, as well as midline shift. Radiological findings suggesting BEH were detected in 59 illustrations (34.3%). A weaker suspicion of BEH was raised in 32 images. An expansive ASDH was detected in 24 images, and 57 illustrations showed neither signs of BEH nor expansive ASDH or were inconclusive. Males were overrepresented in all groups. Mean age was 4.2 months in the BEH-like group and 17.6 months in the ASDH-like group. BEH complicated by SDH may have been misdiagnosed as SBS/AHT in many of these articles.
Among medical therapies, osmotherapy with colloidal agents (mannitol, glycerol) or crystalloid agents (hypertonic saline) is reasonable for patients whose condition is deteriorating due to mass effect and herniation from large anterior circulation hemispheric or cerebellar infarcts, especially as bridging therapies to definitive surgical intervention. Hyperventilation and hypothermia may also be reasonable, though are of less certain benefit. Corticosteroids have not been found helpful. Agents that block sulfonylurea receptor-mediated cellular swelling, such as intravenous glyburide, have shown promise but require pivotal trial testing. Ventriculostomy is useful to treat non-communicating hydrocephalus arising from obstruction of CSF flow pathways by swollen brain infarcts. But in select large cerebellar infarcts, ventricular drainage alone may exacerbate upward herniation of swollen cerebellar tissues, and suboccipital craniectomy along with ventricular drainage is preferred. Suboccipital craniectomy, often with resection of infarcted tissue, is recommended for massive cerebellar infarcts that may herniate directly into the brainstem, on the basis of observational evidence. Hemicraniectomy for brain oedema associated with large anterior circulation hemispheric infarction is life-saving, but survivors often have severe disability, especially when over age 60. If pursued, hemicraniectomy is generally best performed within 48 h of onset and before development of advanced herniation and neurological deterioration.
Acute non-traumatic intracerebral haemorrhage (ICH) has a poor prognosis and is the least treatable form of acute stroke.Although less common than acute ischaemic stroke, ICH causes greater premature loss of productive life years on a global scale due to its predilection to affect people at younger ages with devastating consequences.Prognosis from ICH is related to location, initial volume and speed of expansion of the haematoma, and associated intraventricular haemorrhage. Care in a specialised stroke or neurointensive care unit improves outcome. Surgical haematoma evacuation should be pursued as for patients with cerebellar haemorrhage with neurologic deterioration, hydrocephalus, orsd brainstem compression. Haematoma evacuation may be considered, as a life-saving measure, in patients with coma or large haematoma with mass effect.Minimally-invasive surgery in stable patients is of uncertain benefit and is being evaluated in RCTs.Clinicians should not routinely use haemostatic therapies where there is no evidence of coagulopathy or anticoagulant use.When coagulopathy is present, early corrective measures should be taken. Early moderate intensity BP lowering to a systolic BP target of 140 mmHg is reasonable.Medical therapies to reduce mass effect and intracranial pressure should not be used routinely, but hyperventilation and hypertonic saline or colloidal osmotic agents are reasonable in patients with imminent herniation as a bridge to definitive neurosurgical intervention. Corticosteroids should be avoided.Novel neuroprotective approaches hold promise.
Cognitive impairment and apathy are well-documented features of idiopathic normal pressure hydrocephalus (iNPH). However, research examining other neuropsychiatric manifestations of iNPH is scant, and it is unknown whether the neuropsychiatric presentation differs for iNPH patients with comorbid Alzheimer’s disease (AD) versus iNPH without AD. This study aims to advance our understanding of neuropsychiatric syndromes associated with iNPH.
Methods:
Fifty patients from Butler Hospital’s Normal Pressure Hydrocephalus Clinic met inclusion criteria. Caregiver ratings on the Frontal Systems Behavior Scale (FrSBe) were examined to appraise changes in apathy, executive dysfunction, and disinhibition. Patients also completed cognitive tests of global cognition, psychomotor speed, and executive functioning. AD biomarker status was determined by either amyloid-beta (Aβ) positron emission tomography (PET) imaging or cerebrospinal fluid (CSF) total tau to Aβ-42 ratio.
Results:
Results revealed clinically significant elevations on the FrSBe’s apathy and executive dysfunction scales and modest correlations among these scales and cognitive measures. Of the 44 patients with available neuroimaging or CSF draw data, 14 presented with comorbid AD. Relative to the iNPH-only group, the iNPH + AD group showed a larger increase from pre-illness to current informant ratings on the executive dysfunction scale, but not the apathy or disinhibition scales.
Conclusions:
These results replicate and extend prior research by identifying apathy and executive dysfunction as prominent neuropsychiatric symptoms of iNPH and suggest comorbid AD exacerbates dysexecutive behaviors. Future research is warranted to examine the effects of comorbid AD pathology in response to shunt surgery for iNPH, neuropsychiatric symptom changes, and resultant caregiver burden.
This chapter, provides an overview for midgestational approach to neural tube defects. The authors describe pathophysiology and associated anomalies related to neural tube defects. The surgical approaches and related anesthetic implications are discussed.
In this chapter, the common pediatric conditions of Myelomeningocele and Hydrocephalus are reviewed. The pathophysiology of spina bifida is discussed in relation of both post-natal and fetal surgical management. The author covers the surgical interventions and the related complex anesthetic considerations for both the primary repair of myelomeningocele as well as for ventriculoperitoneal shunt placement. The presentation of patients with raised intracranial pressure in the setting of hydrocephalus is considered.
Flanged ventricular catheters are now used infrequently. Many patients with longstanding hydrocephalus still harbor these catheters, either as their current ventricular catheter, or as a retained catheter from a prior implant. The removal of flanged ventricular catheters is sometimes necessary, and may be challenging due to intraventricular adhesions. We describe the use of an endoscopic technique for the successful retrieval of flanged ventricular catheters in two patients. The technique described in this report may be helpful for patients that have flanged ventricular catheters that must be removed.