Journal Of The Neurological Sciences[JOURNAL]

Idiopathic intracranial hypertension (IIH) and Chiari I malformation (CM-I) are traditionally considered distinct entities, yet accumulating evidence reveals substantial overlap in their clinical presentations and imagin... Idiopathic intracranial hypertension (IIH) and Chiari I malformation (CM-I) are traditionally considered distinct entities, yet accumulating evidence reveals substantial overlap in their clinical presentations and imaging features. Patients with IIH may develop acquired tonsillar herniation mimicking CM-I, while patients with CM-I may harbor unrecognized intracranial hypertension. Failure to identify the primary condition risks unnecessary posterior fossa decompression in patients whose tonsillar descent is pressure-driven, or untreated intracranial hypertension in patients mislabeled as having congenital CM-I. Thus, despite their overlapping presentations, identifying distinct traits in each condition is critical in delivering effective treatment. In this review, we synthesize the neuroimaging features of each condition, identifying their distinct features and their overlap, present illustrative cases spanning the IIH-Chiari diagnostic spectrum, and propose a framework for differentiating the primary pathology to guide management.

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Challenges in the diagnosis of spinocerebellar ATAXIA 27B.

Pons NC, Quirós AP, Planas-Ballvé A … +3 more , Del Toro PL, Sendra IH, Rivera AÁ

J Neurol Sci · 2026 Jul · PMID 42090775 · Publisher ↗

OBJECTIVE: To characterize the clinical, radiologic, and genetic spectrum of patients with (GAA) repeat expansions in FGF14 gene and to analyze diagnostic challenges associated with spinocerebellar ataxia type 27B (SCA27... OBJECTIVE: To characterize the clinical, radiologic, and genetic spectrum of patients with (GAA) repeat expansions in FGF14 gene and to analyze diagnostic challenges associated with spinocerebellar ataxia type 27B (SCA27B). METHODS: We retrospectively evaluated a Spanish cohort of 53 adults with idiopathic late-onset cerebellar ataxia. The intronic FGF14 repeat locus was examined by fluorescent long-range PCR to determine allele size, by bidirectional repeat-primed PCR to verify the presence and nature of the expansion. Clinical data, neuroimaging findings, and response to 4-aminopyridine were collected. RESULTS: Among 53 ataxia patients, 39.62% (21/53) carried uninterrupted >200 (GAA) repeats in FGF14, all in heterozygosity. Of these, 71.42% (15/21) exhibited >250 repeats, corresponding to 28.30% of the total cohort, and 73.33% (11/15) had >300 repeats, representing 20.75% overall. Additionally, 11.32% (6/53) of patients carried (GAA) 200-249 repeats, four of whom presented phenotypes compatible with SCA27B. Neuroimaging showed cerebellar atrophy in 7/21 and superior cerebellar peduncle hyperintensity in MRI of 7/17, including two patients with 200-249 (GAA) repeats. Seventeen patients received 4-aminopyridine; 11 continued therapy and 9 reported clinical improvement. CONCLUSIONS: SCA27B is a frequent cause of idiopathic late-onset cerebellar ataxia. Downbeat nystagmus, episodic symptoms, and superior cerebellar peduncle abnormalities increase diagnostic suspicion. Expansions >250 (GAA) repeats are highly predictive, whereas 200-250 may be pathogenic in compatible phenotypes. Early identification is difficult but crucial given the potential therapeutic benefit of 4-aminopyridine.

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A response to the Comment on "The effect of end-tidal carbon dioxide levels on dynamic cerebral autoregulation and clinical outcome in acute ischemic stroke: an INFOMATAS study".

Walker LM, Nogueira RC, Vasilopoulos T … +6 more , Ince J, Levis B, Mueller M, Panerai RB, Robinson TG, Minhas JS

J Neurol Sci · 2026 Jul · PMID 42090774 · Publisher ↗

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Population and brain diversity are missing from the integrated framework of MS susceptibility.

Rudroff T

J Neurol Sci · 2026 Jul · PMID 42085964 · Publisher ↗

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Revisiting the association between stroke preventive strategies and long-term cognitive outcomes.

Qiu Y, Wang F, Kong W

J Neurol Sci · 2026 Jul · PMID 42085963 · Publisher ↗

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Comment on "The effect of end-tidal carbon dioxide levels on dynamic cerebral autoregulation and clinical outcome in acute ischemic stroke: An INFOMATAS study".

Padhi S, Kokiwar PR, Patel J … +1 more , Kalra A

J Neurol Sci · 2026 Jul · PMID 42085962 · Publisher ↗

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Response to "comment on 'evidence for compromised function of the glymphatic system in pediatric acquired demyelinating disorders'".

Ontiveros F, Mattson WI, Nelson EE

J Neurol Sci · 2026 Jul · PMID 42085961 · Publisher ↗

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Reconsidering peripheral immune markers in Parkinson's disease: Methodological reflections on recent findings.

Sahin A, Ismah, Herawati AA … +2 more , Mubarak MA, Pautina AR

J Neurol Sci · 2026 Jul · PMID 42070469 · Publisher ↗

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Methodological considerations in evaluating lifetime estrogen exposure and neurodegeneration in multiple sclerosis.

Sahin A, Ismah, Herawati AA … +2 more , Mubarak MA, Pautina AR

J Neurol Sci · 2026 Jul · PMID 42070468 · Publisher ↗

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Beyond vascular risk control: Expanding mechanistic and multidomain perspectives on post-stroke cognitive impairment.

Dai J, Sun Y, Zhou X

J Neurol Sci · 2026 Jul · PMID 42070467 · Publisher ↗

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Letter to the editor: Viral load and pathophysiology-based framework in varicella-zoster virus meningitis: Focus on altered consciousness and outcomes.

Peng J, Sun K, Zhu Y

J Neurol Sci · 2026 Jul · PMID 42068659 · Publisher ↗

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Letter to the editor: OSA treatment lowers the risk of declining daily activities.

Putra BJ

J Neurol Sci · 2026 Jul · PMID 42068658 · Publisher ↗

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Comment on "The kynurenine pathway and markers of neurodegeneration and cerebral small vessel disease: The Maastricht study".

Aravind P, Meyyammai CT, Devasirvadam V … +1 more , Ravi N

J Neurol Sci · 2026 Jul · PMID 42068657 · Publisher ↗

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Response to "Letter to the editor: Viral load and pathophysiology-based framework in varicella-zoster virus meningitis: Focus on altered consciousness and outcomes".

Yabumoto T

J Neurol Sci · 2026 Jul · PMID 42068656 · Publisher ↗

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Comment on "Improving care for amyotrophic lateral sclerosis with artificial intelligence and affective computing".

Adhyapak SU, Mishra P, Basavaraj M

J Neurol Sci · 2026 Jul · PMID 42067481 · Publisher ↗

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No interferon-beta antibodies in natalizumab-treated multiple sclerosis patients with progressive multifocal leukoencephalopathy.

Gelissen LMY, Wijburg MT, Strijbis EMM … +5 more , de Jong BA, van Oosten BW, Deisenhammer F, van Kempen ZLE, Killestein J

J Neurol Sci · 2026 Jul · PMID 42066588 · Publisher ↗

BACKGROUND: Interferon beta therapy (IFN-β) can induce production of antibodies (NAbs) that may neutralize both therapeutic IFN-β and endogenous IFN-β, potentially impairing antiviral immunity. OBJECTIVES: To investigate... BACKGROUND: Interferon beta therapy (IFN-β) can induce production of antibodies (NAbs) that may neutralize both therapeutic IFN-β and endogenous IFN-β, potentially impairing antiviral immunity. OBJECTIVES: To investigate whether NAbs against IFN-β occur in multiple sclerosis patients that developed natalizumab-associated progressive multifocal leukoencephalopathy (NTZ-PML). METHODS: Seven NTZ-PML patients were matched with natalizumab-treated patients with prior IFN-β exposure. All were tested for NAbs against IFN-β in serum. RESULTS: NAbs against IFN-β were absent in all NTZ-PML cases, but present in three matched patients. CONCLUSIONS: These findings do not suggest a contributing role for IFN-β NAbs in natalizumab-associated PML.

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Comment on "Stroke preventive strategies and long-term cognitive function: A population-based study".

Song H

J Neurol Sci · 2026 Jul · PMID 42066587 · Publisher ↗

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Journal Of The Neurological Sciences[JOURNAL]

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