[Development of pure alexia due to a lesion in the left fusiform gyrus in a patient with hypertrophic pachymeningitis].

We present the case of a patient with pure alexia due to a restricted lesion in the left fusiform gyrus. A 59-year-old right-handed female, with a 7-year history of rheumatoid hypertrophic pachymeningitis suddenly developed reading and writing difficulties. Neuropsychological examinations revealed the presence of alexia for both Japanese kanji (morphograms) and kana (phonograms); mild agraphia predominantly for kanji; and word-finding difficulty. Brain magnetic resonance imaging (MRI) revealed a high signal intensity lesion in the left fusiform gyrus on fluid attenuated inversion recovery (FLAIR) images in addition to marked thickness of the left cerebellar tentorium on contrast-enhanced T1-weighted images. The abnormal intensity lesion in the left fusiform gyrus was recognized as representing a cerebral edematous change due to venous insufficiency associated with dural thickness. After high-dose methyl-prednisolone therapy, there was a rapid improvement in the reading and writing abilities of the patient, and 5 days later all the symptoms had disappeared. Three months later, a repeat MRI showed that the abnormal intensity lesion in the left fusiform gyrus had disappeared completely. The present case suggests that damage to the left fusiform gyrus alone can cause pure alexia and mild agraphia. Furthermore, this case raises the possibility that the fusiform gyrus is a part of the writing center.

Improvement in Tc-99m HMPAO brain SPECT findings during donepezil therapy in a patient with pure akinesia.

A 58-year-old man presented with a history of disturbance in initiating gait. His history revealed meningoencephalitis five years prior to admission. Neurological examination included gait disturbance as difficulty in initiation and a hesitating speech with many freezing episodes and micrographia Magnetic resonance imaging (MRI) showed diffuse hyperintensity of frontal subcortical white matter on T2 weighted images. He was diagnosed with PA. L-Dopa up to the dosages of 1000 mg/ day and selegiline 10 mg/day were given. First brain SPECT using technetium-99m labeled D,L-hexamethylpropylene amine oxime (Tc-99m HMPAO) was performed when he was taking L-dopa and selegiline. In visual evaluation, hypoperfusion in bilateral frontoparietal cortex was seen (Fig. 2). Treatment with L-dopa and selegiline produced no benefit. Donepezil 10 mg/day was begun. This therapy regimen resulted in dramatic clinical improvement within several days that was confirmed by blinded raters who watched the patient's video recordings. During this response second brain perfusion SPECT study was repeated during donepezil therapy. Markedly increased perfusion in bilateral frontoparietal cortex was observed. This is the first case of PA to develop possibly after an episode of bacterial pneumococcal meningoencephalitis and who responded to donepezil as documented by changes in clinical findings and Tc-99m HMPAO brain SPECT studies.