Migraine is a disabling disease in pediatric and adult population and it is distinguished in Migraine Without Aura (MwA) and With Aura (MA). Rare complications of migraine include status migrainous, persistent aura without migraine infarction, and migraine infarction [1].

Migraine aura status is a rare complication of migraine attack. It is diagnosed by the presence of at least 3 episodes of migrainous aura over a period of 3 days. The pain and/or associated symptoms are often disabling. It was also confirmed in the appendix of the subsequent classification, International Classification Headaches Disorders 3rd (ICHD-3) of 2018, which modified the diagnostic cut-off, setting it at at least 3 aura episodes in 3 days. However, the proposed criteria are based on insufficient empirical evidence, especially for the precise cut-off in terms of the frequency of attacks required for diagnosis [2, 3].

Cortical laminar necrosis (CLN), also described as pseudolaminar necrosis (PLN), is a degeneration of neurons in the cerebral cortex in conditions where the supply of oxygen and glucose is inadequate to meet local needs. It can be observed during events that compromise the patient’s ability to supply the brain with sufficient nutrients to meet its needs [4].

A clarification must certainly be made on the terminology commonly used since the two definitions, CLN and PLN, although often used interchangeably, have different histological meanings: Cortical laminar necrosis involves all layers of the affected cerebral cortex, whereas pseudo laminar necrosis selectively affects the middle and deep layers [5, 6].

Here we describe a rare pediatric migraine aura status associated to the cortical laminar necrosis and report a narrative review of the two disorders.

Table 1 shows the diagnostic criteria of ICHD-3 [2] regarding the definition of Migraine Aura Status.

We present the clinical case of a 14-year-old male patient with a negative personal history of headache or neurological disorders, who came to our observation in the Pediatric Neuropsychiatry Unit, referred by the Emergency Department of the same hospital, for the onset of acute and persistent symptoms. While playing volleyball with friends, without any evident cranial or extracranial trauma, the boy experienced a sudden psychomotor slowdown, with aphasia and dysarthria. The mother, who rushed to provide first aid, reported marked pallor, sweating, and a blank and distant stare. He repeatedly touched the left side of his head and was unable to articulate words. During transport from his hometown to the emergency department and after triage, the state persisted, but aphasia gradually resolved. During hospitalization, in a pain-free phase of wellness, the patient described his experience to clinicians: “I felt like in a dream, I wanted to say something, but I couldn’t pronounce the words. I could hear what they were saying, but I didn’t understand. I was sweating and felt hot, like being inside a bubble because I couldn’t interact with the outside world, and I couldn’t communicate.” At that stage, he reported the onset of severe headache localized to the left parieto-occipital region and major visual function deficits, initially a right homonymous hemianopsia lasting about one hour, followed by transient blurred vision and phosphenes. He also reported disorientation and objective vertigo. On admission, general examination revealed a prostrated appearance, anxiety, and a tendency to touch the left side of the head due to a stabbing and pulsating pain. Neurological examination showed: normal cranial nerves, ocular motility, and coordination; positive Romberg with leftward deviation; mild right-sided weakness; no language disturbance at the time of formal assessment.

During inpatient stay, the patient experienced two further critical episodes. The first one (nighttime) was characterized by abrupt awakening with confusion, fear, disorientation, and failure to recognize roommate. He claimed that there were people in the room who were not really there (“there are doctors here, they have to operate on my ears”), suggesting a possible hallucinatory episode lasting a few minutes. Resolution was followed by severe left parieto-occipital headache and right upper limb paresthesias starting in the hand and extending proximally. The second episode (wakeful rest) was characterized by confusional state with possible phosphenes (“I see like when I rub my eyes”), lasting about 4 minutes, associated with paresthesias in the right hand. It was followed by a severe left parieto-occipital headache too, responsive to oral corticosteroid.

About six months before admission episodes were reported—at least two within two consecutive months—with poorly defined semiological characteristics, not clearly migrainous. During these episodes, the patient was described as agitated and frightened, with facial pallor, profuse sweating, and significant tachycardia with palpitations. During the second episode, nausea with vomiting was reported.

Due to the severity and sudden onset of symptoms, an urgent Brain computed tomography (CT) (General Electric Aascent FCE 128, Milano, Italy), was performed two hours after onset. It showed a millimetric hypodensity in the left parieto-occipital cortical-subcortical area, requiring further characterization.

During the second day of hospitalization, a contrast-enhanced brain magnetic resonance imaging (MRI) (Milano -Italy General Electric SIGNA EXPLORER 1.5 Tesla, GE Healthcare, Milano, Italy) was performed with T1weight (T1w) spin echo (SE), T2weight (T2w), Fast Spin Echo (FSE), Fluid Attenuated Inversion Recovery (FLAIR), Gradient Recalled Echo (GRE) and Diffusion Weighted Imaging (DWI) sequences.

It showed a slight cortical thickening and signal alteration of the left supramarginal gyrus characterized by hyperintensity in T2/Flair sequences, diffusion weighted imaging (DWI)/apparent diffusion coefficient (ADC) restriction and no post-contrast enhancement (see Figs. 1,2,3,4). Moreover, a 3D time-of-flight (TOF) angio sequence was also performed to evaluate the intracranial arterial circulation showing no abnormalities.

Fig. 1.

Axial CT basal scan at the onset; slight millimetric hypodensity in parieto-occipital region (white arrow). CT, computed tomography.

Fig. 2.

Axial FSE T2W MRI performed one day after the debut, shows swollen edematous appearance of the cortex of the left supramarginal gyrus (arrowhead). FSE, Fast Spin Echo; MRI, magnetic resonance imaging.

Fig. 3.

In diffusion weighted imaging/apparent diffusion coefficient (DWI/ADC) sequence the lesion shows restriction of water molecule diffusivity due to cytotoxic edema (a,b).

Fig. 4.

Control MRI examination performed twenty days later. Axial SE T1W basal image (4a) and axial FSE T2W (4b) respectively show appearance of cortical hyperintensity, suggestive of laminar necrosis (white arrow) and edema reduction (arrowhead). Hyperintensity is slightly reduced but still appreciable in DWI (4c), in the absence of signs of restriction in ADC (4d). SE, spin echo.

These results were compatible with cytotoxic edema of uncertain pathogenesis (vascular, epileptic, or inflammatory).

Based on the clinical presentation, diagnostic hypotheses included: migraine with aura, epileptic seizure, acute cerebrovascular event, or infectious meningo-encephalitic condition. Toxicological urine screening was negative, excluding substance abuse. Clinical and electroencephalogram (EEG) findings, along with the absence of semiological features typical of seizures, excluded an epileptic pathogenesis. Similarly, the absence of systemic inflammatory signs, meningeal irritation, EEG abnormalities, or imaging evidence made meningoencephalitic or infectious causes unlikely. Serological tests for neurotropic viruses and neuroimmunological markers were also negative, excluding viral or immune-mediated encephalopathies. Due to the onset of hemiparesis during aura attacks, sporadic FHM could also be suspected, but genetic testing was not performed, both because there were no other relatives with a suspicion of hemiplegia and because one of the separated parents lived far away.

Follow-up brain MRI, performed twenty days later and compared with prior exam, showed reduced T2W signal alterations in the left parietal cortex and no restriction in DWI/ADC sequences. At the site of the lesion, mild hyperintensity was appreciated on T1W on the external cortical surface of the supramarginal gyrus, compatible with laminar necrosis.

Table 2 shows a clinical case summary (Supplementary Material-Care checklist).

The authors declare that written informed consent was obtained for publication.

Clinical semeiology of our case is strongly suggestive of migraine aura status, an infrequent condition that must be distinguished from hemicranius status [2] based on clinical differential diagnostic parameters, and the occurrence of vascular damage, radiologically demonstrated and described as laminar cortical necrosis.

It seems appropriate to describe the characteristics of these manifestations and to review the literature, both in general and in developmental age, regarding the possible correlations between migraine and cerebrovascular damage, with specific attention to descriptions of clinical cases in which the brain damage takes on the anatomopathological characteristics of laminar cortical necrosis. Furthermore, because of the presence of recurrence of migraine aura status within a short period of time in the patient in our study, a review of the literature regarding migraine aura status appears useful.

The literature review was conducted by searching Medline/PubMed and the Cochrane Library from 1980 to 2025, using the following terms: “Migraine Aura Status”, “Aura Status”, “Migrainous Infarction”, “laminar Necrosis”, “Cortical laminar Necrosis”, “Cortical laminar Necrosis and Migraine”.

The diagnostic evaluation of this patient was guided by the need to exclude acute conditions capable of producing this complex neurological phenotype.

We suggest a migrainous origin. Supporting factors included a first-degree family history of migraine, frequent symptomatic recurrences without interictal manifestations, and the absence of permanent deficits or cognitive decline. The age of the minor, the absence of general and cardiac risk factors favouring an ischemic lesion, the associated severe headache, and the reported march of the migraine aura support, in our opinion, the hypothesis that the aura status induced the cortical laminar necrosis. However, the alternative hypothesis—that the onset of the ischemic lesion induced a cortical spreading depression which caused the appearance of the characteristic clinical symptoms—cannot be unequivocally excluded [27].

The case is noteworthy for its unusual aura presentation. Migrainous aura usually arises from cortical spreading depression (CSD) [28], typically beginning in the occipital cortex and spreading anteriorly, producing transient visual, sensory, and sometimes language symptoms [29]. However, it can also present with other rarer symptoms, which make it extremely fascinating to study. Viana et al. (2016–2017) [30, 31] highlight the potential clinical variability of this disorder.

In our patient the sequence and characteristics of symptoms varied across three distinct episodes:

- The first attack began with language impairment, suggesting a frontal onset (Broca’s area), followed by visual symptoms consistent with posterior spread;

This variability suggests that CSD is not restricted to an occipital onset, but may arise in extra-occipital regions, with multidirectional spread. Such phenomena have been described in isolated adult cases [32], for first Raieli et al. [33] have documented a small series of atypical aura in pediatric patients across multiple episodes.

Because the patient experienced three aura episodes within three days, he meets the ICHD-3 criteria for aura status. This entity is rarely reported and poorly characterized, with limited data on its association with cerebrovascular complications.

A second remarkable feature of this case was the cerebrovascular complication. The recurrent CSD likely induced a state of diffuse cortical hyperexcitability and hypermetabolism, creating a mismatch between metabolic demand and cerebral perfusion. This mismatch may more easily render vulnerable areas at the border between grey and white matter or at the transition between the occipital cortex and neighbouring areas [34]. The involvement of extraoccipital cortical areas in all six reported cases of CLN and migraine supports these pathophysiological considerations.

To our opinion, for first time a pediatric case has described in the literature and long-term follow-up remains essential to assess potential developmental impact.

Certainly, individual case reports do not allow for in-depth conclusions, especially when there are very few reports in the literature, and therefore, it is necessary to accumulate further data. However, the increased use of accurate functional MRIs, serialized over short periods of time, EEG spectral analysis, and other neurophysiological methods may provide further information in the future study of other similar cases [35].

This report therefore, highlights an unusual presentation of migraine with aura, complicated by cortical laminar necrosis in a pediatric patient. It underscores the need for greater awareness of atypical aura patterns, recognition of migraine aura status, and further investigation into the potential cerebrovascular risks associated with migraine in childhood.

So, the case appears interesting for:

- The absolutely infrequent finding of laminar cortical necrosis in a situation suggestive of a migraine aura state, with atypical aura;

As can be seen from the literature review, previously mentioned, to date, this patient represents the first ever described case of laminar cortical necrosis following migraine with aura in pediatric age.

No new data were created, and clinical data are unavailable due to privacy or ethical restrictions. The datasets used and analyzed during the current study are available from the corresponding author on reasonable request.

FC and VR: conception and design. CG, GA, GC: neuroradiological data analysis; FC, LMM, CM, EC and VR: clinical data analysis; CG, CM, EC and VR: migraine aura status literature review; CG, GA and GC: Cortical laminar necrosis literature review; FC, CG, GA, GC, CM, EC, LMM and VR: drafting and revising of the article. All authors read and approved the final manuscript. All authors have participated sufficiently in the work and agreed to be accountable for all aspects of the work.

Informed written consent was obtained from the parents to use clinical data anonymously for scientific publications. All the data were part of the patient’s standard medical files and for this reason, according to local ethical policies, the study was deemed as exempt from formal approval by the hospital ethics committee. The study was carried out in accordance with the guidelines of the Declaration of Helsinki.

Not applicable.

This research received no external funding.

The authors declare no conflict of interest.

Supplementary material associated with this article can be found, in the online version, at https://doi.org/10.31083/RN47684.