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Cortical damage in areas such as the frontal lobe is reported in amyotrophic lateral sclerosis (ALS). However, aside from executive dysfunction, the pathological significance of this cortical damage has yet to be clarified. The present study investigated the effects of cortical damage on vestibular function in ALS.Subjects comprised 18 ALS patients and 18 age- and sex-matched healthy controls. Cold air caloric stimulation was performed in all subjects to induce vestibular nystagmus, which was analysed to evaluate vestibular function. Visual suppression testing to investigate the suppressive effects of visual stimuli on vestibular nystagmus was expressed as suppression rate (SR, %). Executive function was tested using the frontal assessment battery (FAB).Suppression rate and FAB score were significantly lower in the ALS group than in the control group (p 0.01 each). A positive correlation was also observed between SR and FAB score (R = 0.65, p = 0.023).Visual suppression testing showed significant damage to the central nervous system vestibular control mechanisms, which utilize visual information in the ALS group and a positive correlation between SR and FAB score suggest a relationship between frontal lobe damage and impaired vestibular control. A simple vestibular function test may be useful as a tool to objectively monitor the progression of cerebral lesions in ALS.

CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an inflammatory central nervous system disorder that gives rise to brainstem symptoms such as diplopia and articulatory disorders. It shows characteristic "salt-and-pepper" enhancing brainstem lesions on magnetic resonance imaging (MRI) scans. We present an unusual case of CLIPPERS, in which a 29-year-old man initially presented with a seizure. Brain MRI revealed faint open-ring enhancements in the right frontal lobe, and a brain biopsy was performed to make a definitive diagnosis. Immunohistological tests showed perivascular infiltration by primarily CD3- and CD20-positive cells, albeit including a few CD68-positive cells, which confirmed a diagnosis of CLIPPERS. Both the symptoms and the MRI findings were markedly improved by 3 courses of steroid (methylprednisolone) pulse therapy, followed by oral steroid (prednisolone) administration. This case shows that CLIPPERS can present as a phenotype of cortical lesions and seizures as the first signs. A brain biopsy should therefore be actively carried out for differential diagnosis, especially when other, cortically based inflammatory disorders are suspected.

We report a case of superficial siderosis (SS) of the central nervous system that developed approximately 8 years after the treatment of cerebral arteriovenous malformation (AVM). A 17-year-old boy was diagnosed with cerebral AVM in the right frontal lobe, which was treated with embolization and radiation therapy. Radiation-induced encephalopathy occurred 5 years later, and the lesion was resected. Although the patient could walk with a cane following the surgery and rehabilitation, he exhibited complete paresis of the left upper extremity. In the course of the next 9 years, he showed cognitive function decline, paresis of the lower right extremity, and the inability to walk. He also exhibited dysarthria and paresis of the right arm. Respiratory failure developed at age 39 years, necessitating mechanical ventilation. Cerebral angiography revealed no recurrence of cerebral AVM; however, brain and spinal magnetic resonance imaging revealed broad hemosiderin deposition, which is characteristic of SS, and the presence of a postoperative cyst that was suspected to be the bleeding source. While eliminating the source of the bleeding is considered the best line of treatment for SS, this was not pursued due to the patient's irreversible neurological condition. Thus, clinicians should consider SS as a differential diagnosis for patients that exhibit gradual and progressive neurological defects several years after successful AVM treatment. Keywords: Superficial siderosis, Radiation-induced encephalopathy, Arteriovenous malformation