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Background Heterogenous central nervous system (CNS) neurologic manifestations of polyarteritis nodosa (PAN) are underrecognized. We review three cases of patients with PAN that illustrate a range of nervous system pathology, including the classical mononeuritis multiplex as well as uncommon brain and spinal cord vascular manifestations. Case presentation Case 1 presented with mononeuritis multiplex and characteristic skin findings. Case 2 presented with thunderclap headache and myelopathy due to spinal artery aneurysm rupture. Both patients experienced disease remission upon treatment. Case 3 presented with headache and bulbar symptoms due to partially thrombosed intracranial aneurysms, followed by systemic manifestations related to visceral aneurysms. She demonstrated clinical improvement with treatment, was lost to follow-up, then clinically deteriorated and entered hospice care. Conclusions Although the peripheral manifestations of PAN are well-known, PAN association with CNS neurovascular disease is relatively underappreciated. Clinician awareness of the spectrum of neurologic disease is required to reduce diagnostic delay and promote prompt diagnosis and treatment with immunosuppressants.

Objective To assess primary caregiver understanding of the term epilepsy. Study design A cross-sectional telephone survey evaluated understanding of the term epilepsy among primary caregivers of children diagnosed with epilepsy at an urban referral center during a 24-month period. Three measures of primary caregiver understanding were used: (1) identifying if their child had a seizure disorder, epilepsy, or both; (2) providing an open-ended definition of epilepsy; and (3) selecting from a multiple-choice definition of epilepsy. Caregivers with 3 correct answers were assigned the greatest knowledge score. Associations with possible predictor variables were analyzed. Results Caregivers for 75 of 116 eligible patients were contacted successfully. Of those, 55 of 75 met eligibility criteria; 45 of the eligible caregivers completed the survey. Twenty-six of 45 caregivers (58%) identified that their child had both a seizure disorder and epilepsy, 5 of 45 (11%) provided a correct open-ended definition of epilepsy, and 16 of 45 (36%) selected the correct multiple-choice definition. Fifteen caregivers (33%) had no correct answers. Seventeen (38%) answered 1, 9 (20%) answered 2, and 4 (9%) answered all 3 measures correctly. Caregivers with greater self-rated understanding had greater epilepsy knowledge scores (P = .008). Having a child neurologist as the first person to discuss the diagnosis with the caregiver also predicted a greater epilepsy knowledge score (P = .04). Conclusions Most primary caregivers of children with epilepsy have a poor understanding of the term epilepsy. Changes are needed in how we educate caregivers about the meaning of this term.

Acute ataxia in a pediatric patient poses a diagnostic dilemma for any physician. While the most common etiologies are benign, occasional individuals require urgent intervention. Children with stroke, toxic ingestion, infection, and neuro-inflammatory disorders frequently exhibit ataxia as an essential-if not the only-presenting feature. The available retrospective research utilize inconsistent definitions of acute ataxia, precluding the ability to pool data from these studies. No prospective data exist that report on patients presenting to the emergency department with ataxia. This review examines the reported causes of ataxia and attempts to group them into distinct categories: post-infectious and inflammatory central and peripheral phenomena, toxic ingestion, neurovascular, infectious and miscellaneous. From there, we synthesize the existing literature to understand which aspects of the history, physical exam, and ancillary testing might aid in narrowing the differential diagnosis. MRI is superior to CT in detecting inflammatory or vascular insults in the posterior fossa, though CT may be necessary in emergent situations. Lumbar puncture may be deferred until after admission in most instances, with suspicion for meningitis being the major exception. There is insufficient evidence to guide laboratory evaluation of serum, testing should be ordered based on clinical judgement-recommended studies include metabolic profiles and screening labs for metabolic disorders (lactate and ammonia). All patients should be reflexively screened for toxic ingestions.

Na/K-ATPase pumps generate outward current during ATP-driven stoichiometric exchange of three intracellular Na ions for two extracellular K ions. At acidic pH, in the absence of extracellular Na and K ions, an uncoupled current flows through wild-type Na/K pumps at large negative membrane potentials, believed carried by protons. Both currents are abolished by the specific Na/K pump inhibitor ouabain. In Xenopusα1 pumps made less sensitive to ouabain by mutations Q120R/N131D or C113Y we observed a similar uncoupled current in the absence of extracellular Na and K ions even at physiological pH (7.6). After deletion of the last five (∈″KESYY), or two (∈″YY), COOH-terminal residues of those pumps, uncoupled current was recorded also in extracellular Na. We used two-microelectrode recording to measure uncoupled, and coupled Na/K transport, currents as those inhibited by 10-30 mM ouabain in Xenopus oocytes expressing C113Y or C113Y-∈″YY Na/K pumps. To investigate whether the uncoupled current traverses the same principal pathway followed by transported Na and K ions we used two methods to close that pathway. In one method, we formed stable BeFX-Na/K-pump complexes, trapped in an E2P-like state with closed cytoplasmic-side gates, by injecting oocytes with 1mM BeFX. In the other, we closed the extracellular access pathway by modifying a Cys substituted for T806, at the outer end of TM6, with 1 mM extracellular MTSET. Both methods abolished coupled Na/K pump transport current, as well as Na current flow through palytoxin-bound C113Y Na/K pump-channels. But neither method diminished uncoupled current at −180 mV, suggesting either that the responsible ions do not traverse the principal pathway shared by transported Na and K ions or, if they do follow that route, that they do not travel as hydrated cations. [NIH HL36783]