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A 54-year old man was presented to the Emergency Department (ED) with a long-time history of migraine without aura. He developed neurological symptoms gradually. He complained that the desktop icon of his computer screens appeared nearer, moving towards his right visual field. These visible symptoms appeared for 10 minutes, and then he developed sensory aphasia that lasted almost an hour and disappeared shortly. All these symptoms were transient and grew gradually. These symptoms were further followed by pulsating, intense headache accompanied by nausea and photophobia. He was diagnosed with AIWS due to a glioblastoma located in the left temporal– occipital junction, and to date, it is the first case of AIWS caused by glioblastoma.
What will be most likely explanation for the pathogenesis of Alice in Wonderland Syndrome in the present case?
Glioblastoma multiforme (GBM) is a highly malignant form
of a brain tumour presented with focal neurological symptoms and signs of
raised intracranial pressure as a reduced level of consciousness, seizures and
headache.
The patient did not show any medical history for related diseases
except episodic migraines occurring irregularly in the previous years with a
mean of two attacks per month. He had a family history of gliomas. Brain MRI
was performed three years before to exclude secondary headaches were normal.
The patient was
admitted to ED, and a neurological exam was performed. Standard
electroencephalography (EEG) showed normal results. Unenhanced brain computed
tomography (CT), cerebrospinal fluid and laboratory blood examination showed no
alterations. The patient was shifted to the Neurology Department. The
neurological exam remained unchanged even after one day, and the patient
confirmed that headache following the transient symptom was similar to his
usual migraine. It was the first time he experienced an aura-like phenomenon.
Brain MRI revealed a 2.4 × 2.5 cm cortical-subcortical temporal–occipital
lesion in the left hemisphere, positive in diffusion-weighted imaging (DWI),
with inhomogeneous gadolinium enhancement. The examination was completed by MR
spectroscopy and tractography. The space-occupying lesion was identified as a
possible high-grade glioma.
The patient underwent surgery, and the glioma mass was removed en
bloc with a laser. No postoperative complications were reported. Histological
examination showed an incidence of glioblastoma with primitive neuroectodermal
tumour (PNET) component.
He was advised to start antiepileptic therapy (valproate 1000 mg
daily) immediately after the surgery. The patient recovered after a few days,
and he was discharged after a few days in good condition without neurological
deficits. Outpatients clinic visits were planned to administrate temozolomide
and radiotherapy. The patient underwent neurosurgery again after one year for
removing a relapse of glioma in the same site. A secondly generalised seizure
preceded him with language disturbance without visual alterations. He reported
no features of AIWS after the first episode. Levetiracetam (1000 mg daily) was
added to valproate. Twenty months after the first AIWS episode, the patient is
alive, with no evidence of disease and in good clinical condition.
It is the first reported case of AIWS associated with GBM. The
patient had a transient visual disorder characterised by kinetopsia (objects
appearing moving while they are still) and pelopsia (objects appearing nearer
than they are) as initial clinical presentation of a GBM in the left
occipital-temporal area. These symptoms were followed by sensory aphasia and
headache, indicating the diagnosis of migraine with aura. It was the first
episode; therefore, a definite diagnosis of migraine with aura cannot be made.
Migraine is the prevalent neurological disease, affecting about 15% of the global
population. Among these, 30% of migraineurs experienced transient neurological
deficits as migraine aura. The differential diagnosis between migraine aura and
its mimics may be challenging because aura features are variable among migraine
patients. A brain MRI was performed due to the occurrence of symptoms of
migraine with aura. It showed the presence of brain lesion in the
temporal-occipital carrefour. This area is altered in most of the cases
reporting AIWS following localised damage. The lesions affect the right
hemisphere in most of the cases, but abnormalities on the left side have also
been reported. The direct effect of intracranial space-occupying lesion
involving the temporal-parietal-occipital multimodal area can be described as
the most reasonable hypothesis that can trigger AIWS. The patient reported only
one episode of AIWS, which did not appear in the disease-free period after
removal of GBM nor during the tumour relapse. Another explanation can be the
occurrence of a seizure caused by irritation of cortex induced by GBM, but this
possibility seems less probable. Apart from negative EEG at the admission,
symptoms developed slowly with a gradual symptom-to-symptom progression lasting
for about 1 hr.
Moreover, symptoms were followed by a headache of migrainous
characteristics in due time. Headache accompanied by an epileptic seizure is a
rare event, and it lacks typical features of migraine. The occurrence of
cortical spreading depolarization is an intriguing hypothesis. There is no data
on the relationship between brain tumours and CSD; the latter can be induced in
animal models by an increase of extracellular potassium, a direct consequence
of neural damage. Therefore, the neuronal compression and the damage caused by
GBM may trigger an aura-mimic mechanism leading to AIWS symptoms. Lesional AIWS
seems to be related to a dysfunction in the parietal-temporal-occipital region,
so attention should be paid in the area in AIWS cases occurring without signs
of systemic alteration (e.g., encephalitis, drug abuse, etc…)
A careful diagnostic workup should always be suggested in
the first-ever occurrence of migraine aura with symptoms related to the
inclusion of multimodal areas and also in patients with a long history of
migraine so that the aura mimicking phenomenon or underlying pathologies such
as high-grade gliomas could be excluded.
Neurocase.2018; 24: 5-6.
Temporal-occipital glioblastoma presenting with Alice in Wonderland Syndrome in a patient with a long-time history of migraine without aura
Mastria, G. et al.
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