CONTINUUM Lifelong Learning in Neurology最新文献

Objective: This article highlights important concepts of caring for patients with childhood-onset hydrocephalus across the lifespan, emphasizing recent advances and current areas of controversy.

Latest developments: There are numerous causes of symptomatic hydrocephalus in neonates, infants, and children, and each etiology predicts the typical clinical course across the lifespan. Because the major cellular components essential for CSF dynamics develop predominantly during the third trimester and first 6 months after term birth, directly overlapping with the onset of many types of fetal, neonatal, and infantile hydrocephalus, the timing of onset impacts lifelong management. The treatment of childhood-onset hydrocephalus varies dramatically worldwide due to the differing etiologies and access to neurosurgical interventions. The surgical options for CSF diversion have advanced over the past decade, with a better appreciation of which techniques are more successful for the various types of childhood-onset hydrocephalus. These advances include using temporary shunt devices to delay permanent CSF diversion and endoscopic third ventriculostomy, at times with choroid plexus cauterization.

Essential points: Etiology and age of onset impact the lifelong management of individuals living with childhood-onset hydrocephalus, with a rapid pace of evidence-based changes in clinical practice.

Objective: This article reviews the most common presenting symptoms and radiographic findings of atraumatic cranial CSF leaks, surgical management of skull base leaks, and postoperative management of idiopathic intracranial hypertension (IIH).

Latest developments: IIH is now widely accepted as a causative factor in the development of thinning of the bone of the anterior and lateral skull base and formation of encephaloceles or arachnoid granulations through the skull base with resulting CSF leaks into the aerated sinuses or middle ear and mastoid cavities. Demographic characteristics and presenting symptoms may differ slightly between patients with IIH and those with CSF leaks, but in both populations, management of elevated intracranial CSF pressure is required. Dural venous stenosis is associated with both conditions, and stenting of transverse or sigmoid sinus stenotic segments is now considered a treatment option for IIH.

Essential points: Atraumatic cranial CSF leaks present with clear fluid either dripping from the nose or accumulating in the middle ear and mastoid because of bony and dural defects in the anterior or lateral skull base. Cranial CSF leaks are often associated with IIH, and treatment requires both repair of the skull base defects and management of IIH. Timely diagnosis and treatment of CSF leaks and IIH are necessary to prevent meningitis and decrease the risk of recurrence of leaks or sequelae of IIH. Patients with these CSF leaks should be evaluated for IIH by imaging or postoperative lumbar puncture with opening pressure because pressure may increase further once the CSF leak is repaired. Medication, weight loss, and CSF shunting are management options for elevated CSF pressure, but imaging for intracranial venous stenosis should be considered with venous stenting for treatment of elevated venous pressure.

Objective: This article reviews the preoperative considerations, postoperative complications, and ongoing management of shunts for normal pressure hydrocephalus.

Latest developments: The radiographic pattern of disproportionately enlarged subarachnoid space hydrocephalus (DESH) predicts a positive response to permanent CSF diversion. MRI-safe programmable shunt valves allow for fluid drainage adjustment based on patients' symptoms and radiographic images. Endoscopic third ventriculostomy is a viable alternative to shunting for patients with normal pressure hydrocephalus due to aqueductal stenosis.

Essential points: Approximately 75% of patients with normal pressure hydrocephalus improve after shunt surgery regardless of shunt type or location. Clinical benefit and complication rates are similar for frontal and parietal ventricular catheter approaches. Acetazolamide is not an effective treatment for normal pressure hydrocephalus.

Objective: Headache is the most common symptom of intracranial hypertension and hypotension and may not remit after normalization of intracranial pressure. This article reviews the clinical presentation, mechanism, differential diagnosis, treatment, prognosis, and monitoring of persistent headache after normalization of intracranial pressure in the setting of idiopathic intracranial hypertension and spontaneous intracranial hypotension.

Latest developments: Erenumab, a monoclonal antibody to the calcitonin gene-related peptide receptor, was shown to reduce headache frequency in the first-ever prospective study of headache treatment in patients with idiopathic intracranial hypertension in ocular remission. Similar avenues remain to be explored for spontaneous intracranial hypotension even though it has been shown that some patients continue with headache despite radiographic resolution of CSF leaks.

Essential points: Headache is the most common symptom to herald an intracranial pressure disturbance and may not resolve despite normalization of pressure. Neurologists must be aware that persistent headache does not automatically imply abnormal intracranial pressure in patients with previous disorders of CSF dynamics and informed of the possible alternative headache etiologies in these populations.

Objective: Neuro-ophthalmic deficits can significantly impair quality of life. This article reviews neuro-ophthalmic symptoms and their impact on activities of daily living and discusses therapies that can improve quality of life.

Latest developments: Loss of central vision, peripheral visual field deficits, and diplopia can result in loss of driving privileges, restricted ambulation, impaired reading, and poor social interaction. Poor vision is associated with worse cognition and mental health deterioration in older patients, which can be reversed by ophthalmic interventions such as cataract surgery. Ophthalmic conditions such as dry eye syndrome and convergence insufficiency are common in patients with neurologic diseases such as Parkinson disease and can affect daily activities such as reading and watching television. Low-vision occupational therapy can improve quality of life by helping patients adjust to visual disturbances from neuro-ophthalmic disorders. Vision-related quality of life is an important outcome measure in studies of neuro-ophthalmic diseases. It is most frequently measured using the National Eye Institute Visual Function Questionnaire.

Essential points: Neurologists should screen vision, visual symptoms, and vision-related quality of life when managing patients with neurologic diseases. Patients must be encouraged to consult an eye care provider to manage common ophthalmic conditions such as refractive error, dry eyes, and cataracts. Neurologists should partner with low-vision occupational therapy services to improve vision, cognition, mental health, and quality of life in patients with these conditions.

Objective: This article describes how ocular imaging techniques and electrophysiology studies aid in the localization, diagnosis, and management of disorders affecting vision.

Latest development: The retina and optic nerve are functionally and embryologically part of the central nervous system, and they are the exceptional parts of the brain that can be directly examined by the neurologist or ophthalmologist (through the fundus examination). Ocular imaging techniques including fundus photography, optical coherence tomography (OCT), retinal angiography, and orbital ultrasound allow more detailed assessments of the retina and optic nerve. In some settings, nonmydriatic fundus photography can provide more accurate assessments than direct ophthalmoscopy. OCT allows high-resolution imaging of retinal and optic nerve structures. Combined with automated segmentation algorithms to allow for the measurement of retinal nerve fiber layer and macular ganglion cell layer thicknesses, clinicians and investigators can determine how these layers are affected in optic neuropathies and some neurodegenerative disorders. Electrophysiologic studies provide complementary information to ocular imaging modalities. Electroretinography assesses the function primarily of photoreceptor rods and cones, whereas visual evoked potentials assess primarily optic nerve function.

Essential points: Ocular imaging and electrophysiology techniques aid in diagnosing and managing neuro-ophthalmic disorders and are increasingly accessible to neurologists.