Ep. 193: Understanding GBS and other inflammatory neuropathies across ages

00:00:00: Welcome to EINcast, your weekly source for education research and updates from the European Academy of Neurology.

00:00:15: Hello!

00:00:16: And welcome to EIncast Weekly Neurology.

00:00:20: My name is Francesco Germano.

00:00:22: I'm a neurologist and neurophysiologist working with both children and adults... ...and i currently practice at Galliera Hospital in general Italy where I represent the residents and their research fellows section.

00:00:41: Today, i have the pleasure perhaps even a challenge of wearing two hats will be moderating these epitodes while also taking part in this discussion as one of guests.

00:00:53: This episode is part of podcast series Our Children Small Haddles A question that's simple on surface yet deeply complex in daily clinical practice.

00:01:05: Through this series we aim to explore neurological disease across the lifespan, focusing on what truly changes with age.

00:01:13: What remain the same and why these distinctions matter for patient care?

00:01:18: In today's episode We will be focussing on Guillain-Barre syndromes And other inflammatory neuropathies across ages.

00:01:26: These disorders are particularly well suited To this discussion I'll throw.

00:01:31: they share a common immunological mechanism.

00:01:34: Their clinical presentation, disease curves, diagnostic challenges and management strategies can differ markedly between pediatric and adult patients.

00:01:44: Subtle differences in symptoms, electrophysiological findings or recovery patterns may have a major impact on clinical decision especially acute and emergency settings.

00:01:57: To explore this aspect I'm very pleased to welcome a colleague and friend also joining us from Genoa, Dr.

00:02:05: Matteo Cataldi.

00:02:06: that is a child neuropsychiatrist with strong expertise in clinical neurophysiologies and neuromuscular emergencies.

00:02:14: his work lies at the crossroad of pediatric neurology acute neuromascular disorders and electrophysiology making him an ideal partner for today's discussion.

00:02:25: together we will discuss Our inflammatory neuropathies present at different ages.

00:02:32: Where pediatric and adult perspective align, where they clearly diverge?

00:02:37: Our goal is to offer clinically relevant insights encourage dialogue between pediatrics and adult neurologists And ultimately contribute a more integrated life-span oriented approach to the neurologist.

00:02:52: Matteo Thank you very much for joining us today and welcome to the EINcast.

00:02:57: Thank You Francesco, it's really a pleasure to be here!

00:03:03: And I really like the framing of this series because in neurology we often say that children are not just small headwoods but In practice We still tend think in headwood potters then try to adapt them.

00:03:18: GBS is perfect example.

00:03:20: The immunological process may be similar across ages, but the way children present deteriorate and ricotta can be very different.

00:03:29: And those differences matter especially in acute settings!

00:03:34: So I am happy to take part of this discussion and explore where pediatric and adult perspectives truly overlap... ...and where they really don't.

00:03:46: Amazing Matteo.

00:03:47: So, before we drive into a mechanism and management let's take more concrete look at epidemiology because sometimes it is boring math.

00:03:58: numbers, patterns & real life differences across ages matter when you are actually seeing patients.

00:04:06: If your on call an patient presents with acute or subacute weakness.

00:04:11: inflammatory neuropathies may not be the most common diagnosis but they are common enough that missing them as real consequences.

00:04:21: So starting with the most frequent entity Guillain-Barre syndrome, large population based studies consistently report at overall incidence of about one to two cases per hundred thousand per year in heddles ,with a clear increase roughly zero dot four to one per hundred thousand per year, but GBS still represents the most common case of acute flat sheet paralysis in pediatric population.

00:04:58: In high income countries.

00:05:00: so while you will see it less often in children when acute weakness appears gbs remained a priority diagnosis.

00:05:10: there are also age-related differences.

00:05:13: In most European court, occult inflammatory demilinizing polyneuropathy is the dominant form across all ages.

00:05:22: However, axonal variants such Aiman and Anstan appear relatively more frequent in children than young adults in southern and eastern Europe compared with northern Europe.

00:05:33: This not just an academic detail because axonal forms often have different prognosis and recover trajectory.

00:05:42: If we move to GBS variant, Miller Fisher syndromes accounts for roughly five-ten percent of GBS cases in Europe and tends be more frequent in adults than children while very young children may present with incomplete or atypical forms that are easy to miss.

00:06:01: Now chronic inflammatory neuropathies show a much sharper age gradient.

00:06:06: Chronic inflammatory demyelinating polyneuroid egopathy have a prevalence in adults ranging from one to nine per hundred thousand depending on diagnostic criteria and study design.

00:06:19: In contrast, pediatric CIDP is rare with prevalence estimates well below one-per-hundred-thousand children.

00:06:28: When it does occur It often presents earlier with gait disturbance proximal weakness or relapsing curses features that can overlap with inherited neuropathies and delayed diagnosis.

00:06:40: Some inflammatory neuropaties are in practice almost exclusively adult disease, multifocal motor neuropathy for example as a prevalence of about zero point five per hundred thousand and is especially rare in children.

00:06:54: anti-mag associated neuropaty essentially confined to adult or older reflected its strong association with monoclonal gamopathies.

00:07:04: Similarly, vasculitic neuropathies predominantly affect adults and elderly patients... ...and are rarely incurrent in pediatric neurology.

00:07:15: On the other hand certain immuno-mediated neuropatic presentation is proportionally more relevant to children.

00:07:22: Acute post infection neuropathy including GBS or some painful narrating syndrome following viral illness.. ..are a classic pediatric scenario.

00:07:32: Antesident infections are identified in up to two first of pediatric GBS cases, compared with lower proportion in adults where comorbidities and systemic disease play a larger role.

00:07:44: European epidemiology also reminds us that geography matters.

00:07:48: Northern European courts tend to report older age at Olsen for many inflammatory neuropathies, while southern and eastern regions often show younger onset in the slightly higher proportion of axonal forms.

00:08:02: These differences likely reflect a complex interaction between infections genetics environmental factors and health care access.

00:08:11: so already Just be considering age and context, we are not only estimating probability.

00:08:17: We're shaping our differential diagnosis Our diagnostic strategy And our expectation for outcome.

00:08:25: So when you ask whether children are small-headed inflammatory neuropathies give us a clear answer Not really The same diagnostic label may hide different epidemiology Different triggers and different clinical challenge.

00:08:41: And with this overview in mind, I think that is the perfect moment to move from how often and in whom these diseases occur.

00:08:50: To all they are actually present... ...and now we recognize them early which is where Matteo's expertise will guide us next.

00:09:00: Thank you Francesco!

00:09:03: The real challenge is that in pediatrics GBS does not look like classic GBS especially in infants and young children.

00:09:14: So, how do children present?

00:09:16: And How is this different from

00:09:18: adults?".

00:09:19: In school-age children the story can still sound familiar A prevedly well child develops leg pain then starts limping or refusing to walk.

00:09:29: Over a few days you begin see symmetric weakness and reduce or absent reflexes.

00:09:36: But pain plays much bigger role in pediatrics than adults.

00:09:40: Heddles often report peresthesia early.

00:09:44: Younger children may not be able to describe sensory symptoms at all, so you see behavioral equivalence, irritability.

00:09:52: crying with movement, refusal to stand or parance saying it looks like their legs hurt.

00:09:59: Progression can be fast.

00:10:00: some children reach nada within a few days, cranial nerve involvement is not rare and when barber's symptoms appear dysphagia, a weak or nasal voice that immediately becomes a safety issue not just a diagnostic clue.

00:10:17: Autonomous dysfunctional some matters.

00:10:19: heart rate instability blood pressure swings even arrhythmias can be substantial especially in severe cases.

00:10:30: Neurological examination in infants and young children.

00:10:33: where are the main pitfalls?

00:10:35: This is where things really get tricky.

00:10:39: Infants don't give you GBS on command.

00:10:42: You can test strife compassionally, so the exam becomes observational and developmental.

00:10:49: The first question is always has a child lost milestones?

00:10:54: Less skating, reduced anti-gravity movements New head lag, decreased spontaneous activity These are often the earliest signs Feeding his another critical window.

00:11:07: A weak suck, early fatigue during feets or feats that suddenly take much longer than usual can be the first signs of a bulbarium bulb note.

00:11:17: Often well before respiratory compromise is obvious At bad site tone and reflexes are still your most reliable hankers.

00:11:27: Hypotonia with hyporexia remains the core pattern but only if child is calm.

00:11:34: Crying and distress can transiently increase tone and mask weakness.

00:11:39: Pain complicates everything, neuropathic or radicular pain can make children miserable and inconsolable.

00:11:46: They may look encephalopathic which easily misleads clinicians toward meningitis or encefalitis and delays the correct diagnosis.

00:11:56: And then there's the track of early normal tests.

00:11:59: CSF albuminocytological dissociation is often absent in the first week.

00:12:05: Nerve conduction studies can also be no diagnostic early on, so an early normal lumbar puncture or EMG does not exclude GBS.

00:12:16: When presentation is atypical especially with asymmetry and very abrupt progression spinal hemorrhoid becomes essential Not to prove GBS but to rule out conditions like acute flaccid myelitis or transverse myelitus.

00:12:34: So which pediatric red flags help avoid misdiagnosis and identify danger early?

00:12:41: I usually group red flags into two categories, first red flag is suggesting an alternative diagnosis.

00:12:50: marked asymmetry extremely abrupt onset a clear sensory level or early sphincter does.

00:12:56: this function should immediately point towards spinal cord disease rather than classic GBS.

00:13:03: Altered mental status, persistent high fila or seizures are not compatible with GBS and should redirect your diagnostic thinking.

00:13:14: Second red flags for severity within GBS bulb at dysfunction rapidly, rapidly progressive weakness and autonomic instability mean you should escalate monitoring early, and seriously consider ICU-level care.

00:13:30: In children the margin for error is smaller, deterioration can be fast & respiratory failure may arrive before your field diagnostically comfortable.

00:13:41: So... The key takeaway in pediatrics GBHAS rarely follows textbook.

00:13:49: developmental stage pain and limited cooperation often obscure early science.

00:13:55: The key is recognizing the bedside pattern, produce progressive weakness with hyperreflexia ,and using MRI and repeat neurophysiology when initial tests are on revealing.

00:14:09: Thank you so much Matteo!

00:14:11: Really really interesting !

00:14:13: Now let's go home And Let's keep an eye on management.

00:14:18: So When we talk about Management This is where guidelines stop and bedside decision begin.

00:14:26: Because once the diagnosis has made, The real question isn't what's recommended in general but What Is the right treatment for this patient?

00:14:35: And this age Right now.

00:14:38: Inflammatory neuropathies clearly show why management must be age-adapted.

00:14:43: The tools may look similar But how we use them and what we aim For changes substantially across lifespan.

00:14:51: Let's start with acute inflammatory neuropathies, particularly Guillain-Barré syndrome.

00:14:56: Across children adults and endodilipatients.

00:14:59: first line immunotherapy remains intravenous immunoglobulin or plasma exchange but their application differs.

00:15:07: In children EVG is usually the preferred option.

00:15:11: it easier to administer better tolerated and associated with fewer complications.

00:15:17: Day-key management issue in pediatric is often early recognition and monitoring.

00:15:23: Respiratory function, autonomic instability, pain & early rehabilitation.

00:15:29: Children often recover well but only if deterioration is anticipated rather than reacted to.

00:15:37: In adults both EVG and plasma exchange are valid and evidenced based.

00:15:42: Timing is crucial and disease severity of the guides destroys ear-reventing treatment.

00:15:48: related complication is an important as treating the neuropathy itself.

00:15:54: Moving to chronic inflammatory neuropathies, CIDP is where age-related tailoring becomes most evident.

00:16:02: CIDp is predominantly an adult disease.

00:16:05: in children it is rare frequently atypical and often confused with inherited neuropaties.

00:16:11: from a management perspective pediatrics CID p aims for disease control while minimizing long-term toxicity.

00:16:20: Steroids can be effective but their impact on growth and bone health makes EVG the preferred long term strategy in many, many pediatric cases.

00:16:31: In adults CIDP often requires long-terms therapy using EVG corticosteroid plasma exchange or immunosuppressants.

00:16:40: In elderly patients that challenge is avoiding over treatment.

00:16:44: sometimes maintaining function with the lowest effective intensity is best outcome.

00:16:50: This also where new targeted therapies are reshaping field, in adults F-Gartigymod and FCRN antagonists has shown efficacy in reducing relapse risk in CIDP And it's now approved for adult patients.

00:17:07: For pediatrics Robust trial data are still lacking and use remaining of flabber or investigational.

00:17:15: However, pediatric investigation plans are underway and this signal has shifted toward more age-inclusive neuroimmunology research.

00:17:26: Another critical group to mention is Paranodopphatis particularly antibody mediated diseases such as antineurofascin neuropathy.

00:17:35: These patients both adults or children, often respond purely to intravenous immunoglobulin and behave differently from classing CIDP.

00:17:46: Importantly case reports and small pediatric series show responses to Rituximac especially in treatment resistant children.

00:17:55: the practical message so is simple if a CID P patient is EVG refractory Multifocal motor neuropathy is another condition, where management is very specific.

00:18:11: Intravenous imoglobulins remains the only evidence-based therapy across ages.

00:18:17: Steroids and plasma exchange are generally ineffective or harmful.

00:18:22: Rituximab has been explored but evidence does not currently support routine use.

00:18:27: Optimizing EVG remains the constant.

00:18:31: Finally vasculity neurophaties rare in children more frequent in adults, require a completely different approach.

00:18:39: These are systemic disease until proven otherwise often requiring corticosteroids and immunosuppressive agent in close collaboration with rheomatology.

00:18:51: Early recognition here is critical because delayed treatment can lead to irreversible nerve damage.

00:18:59: across all of this condition multidisciplinary care is not optional.

00:19:05: So, when managing inflammatory neuropathies the key question is NOT which drug but which strategy for each patient at which stage of life and that's where truly personalized neurology begins!

00:19:23: Let's move on with the next chapter.

00:19:28: Prognosis & Outcomes Across Age Groups.

00:19:32: Over all, children tend to recover better than adults from GBS but better does not mean benign.

00:19:41: Prognosis still depends on severity, subtype and early complications And Children need careful follow-up just as much as adults do.

00:19:51: How does prognosis differ between children and adults?

00:19:55: If we look at the big picture The difference is quite clear.

00:20:01: Most children with Gb has regaining independent walking within weeks to a few months and the long-term neurological outcome is generally favorable.

00:20:11: Heddles, especially holder heddles have as lower an often less complete recovery.

00:20:16: They are more likely to have persistent weakness fatigue pain and functional limitations at six to twelve months.

00:20:24: Mortality is low in both groups with moderate care but it remains higher in levels mainly due to respiratory failure and severe autonomic complications.

00:20:35: That said, pediatric is not a free pass.

00:20:38: even when children recover motor function many continue experience fatigue pain or reduced endurance.

00:20:46: that affects daily life school participation sports at overall quality of the live.

00:20:51: these aspects are often underestimated if we focus only on working.

00:20:57: What does really drive long-term outcome?

00:21:00: Across all age groups, the strongest prognostic factors are remarkably consistent.

00:21:06: First initial severity Children who cannot walk at NEDA Who develop respiratory failure or who show early bulbar involvement have a higher risk of prolonged recovery and receivable deficits.

00:21:21: Second autonomic dysfunction Significant arrhythmias or marked blood pressure instability are not just acute management issues, they're markers of more severe disease and worse overall outcome.

00:21:35: Third, electrophysiology – finding suggesting hexanal injury patterns consistent with hexal variants predict slower and less complete recovery compared to poorly emanating forms.

00:21:49: where Age comes into play is slightly different in adults versus children.

00:21:55: In adults, age itself is a strong independent negative prognostic factor and it's built into most outcome prediction tools.

00:22:04: In Children, age is less straightforward.

00:22:07: very young children don't necessarily do worse biologically but they often have more complex clinical courses.

00:22:14: Why?

00:22:15: Because early detection is harder symptoms are less explicit and respiratory or bulbar compromise can be missed until later.

00:22:24: That delay alone can influence outcome.

00:22:28: Follow-up is crucial.

00:22:29: if a child fails to improve within the expected time window, who worsens?

00:22:33: after initial stabilization you have to reconsider diagnosis.

00:22:38: Acute onset CIDP is rare but it exists and misdiagnosis at start can masquerade as poor recovery.

00:22:47: What might change how we think about prognosis in the future?

00:22:52: There are two major directions that may reshape prognosive thinking in the coming years.

00:22:57: The first is the treatment, as was clearly illustrated in Francesco's previous talk beyond high V-high G and plasma exchange newer therapies targeting compliant activation or accelerating pathogenic high GG removal are being explored.

00:23:15: Early trials have shown mixed results on short-term handpoints, but there are signals suggesting potential long term functional benefit and the pipeline is very active.

00:23:25: The second is prediction & personalization.

00:23:28: We're moving toward better pediatric specific prognostic model based on large international cohorts.

00:23:34: The goal isn't just to predict walking But to identify early ways at risk of persistent fatigue pain cognitive or psychosocial difficulties and to intervene sooner.

00:23:45: Importantly, outcome measures are also evolving.

00:23:49: recovery is no longer just can the child walk.

00:23:53: it's participation in school physical endurance emotional well-being and quality of life.

00:23:59: those domains matter enormously to families and they should matter to us.

00:24:05: so the take home message is this pediatric gbhassis usually a good recovery story but it becomes an even better one when we identify severe phenotypes early, monitor respiratory and autonomic risk aggressively ,and ensure structure rehabilitation along term follow-up.

00:24:23: Thank you so much Matteo for your valuable point of view .

00:24:28: And for sharing with us your great experience.

00:24:32: So We have reached almost the end of our chat.

00:24:35: But before we close today's episode Let me briefly highlight a few take-home messages and then say goodbye.

00:24:44: First of all, inflammatory neuropathies remind us very clearly that age matters.

00:24:51: Children, adults and elderly patients may share similar disease mechanisms but they differ in frequency presentation treatment priorities and outcomes.

00:25:02: asking are children our small huddles?

00:25:06: It's not just a philosophical question, it is practical one that affects how we diagnose treat and support our patients every day.

00:25:17: Second management is increasingly moving toward tailored patient-centered strategies from established therapists such as EVIG and plasma exchange to emerging targeted treatments.

00:25:31: the future of inflammatory neuropathies lies in personalization.

00:25:36: This brings me to a broader point, we are living an era of extreme subspecialisation in neurology.

00:25:44: while this allows us reach remarkable levels of expertise it also makes collaboration more and more important than ever.

00:25:53: complex neurological disorders do not respect artificial boundaries between age groups subspeciabilities or countries.

00:26:02: Progress happens when we talk to each other, share perspectives and engage in real brainstorming across disciplines and across borders.

00:26:13: In this sense the European Academy of Neurology play a crucial role.

00:26:19: The EEN is not only as scientific society but it's truly European community that brings together neurologists at all career stages from all subspeciabilities and from all parts of Europe.

00:26:33: Through its educational programs, scientific initiatives working groups and opportunities for young neurologists the EEN actively foster dialogue collaboration and innovation.

00:26:47: With that I would like to thank you for listening And once again thanks my colleague For this stimulating discussion.

00:26:55: We hope this episode is giving you new insights, practical reflection and perhaps a few questions to take back your daily clinical practice.

00:27:06: So thank-you for joining us on the EINcast Weekly Neurology!

00:27:10: And until next time.

00:27:12: let's keep the conversation going.

00:27:15: Thank You again Francesco.

00:27:16: It has been real pleasure collaborating with you today.

00:27:20: Thanks everyone for listening.

00:27:30: This has been EINCast Weekly Neurology.

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00:28:07: E&Cast Weekly Neurology is your unbiased and independent source of educational research-related neurological content, although all content provided by experts in their field should not be considered official medical advice.