Iodine-123-Iomazenil Single-Photon Emission Computed Tomography Revealed Recovery of Neuronal Viability in Association With Improvement of Cognitive Dysfunction After Revascularization in Moyamoya Disease.

BACKGROUND AND OBJECTIVES: Recent studies indicate that 123I-iomazenil (123I-IMZ) single-photon emission computed tomography (SPECT) can demonstrate neuronal viability. Although cognitive dysfunction has been recognized as an important issue in adult patients with moyamoya disease (MMD), no standard neuroradiological methods to define such conditions have been established. We examined the relationship between cognitive function and 123I-IMZ SPECT before and after revascularization in patients with MMD. METHODS: The study participants were 16 adult patients with MMD whose cerebrovascular reactivities were decreased only on the surgical sides of combined revascularization. Cognitive function was examined using the Mini-Mental State Examination (MMSE; cutoff: 27) and the Frontal Assessment Battery (FAB; cutoff: 16) before and at 3 to 6 months after surgery. 123I-iodoamphetamine (123I-IMP) SPECT with acetazolamide challenge and 123I-IMZ SPECT were performed concurrently while evaluating cognitive function. The radioreactivities of 123I-IMZ SPECT in regions with decreased cerebrovascular reactivities on 123I-IMP SPECT were investigated using affected-to-contralateral side asymmetry ratio (IMZ-ACR). RESULTS: Twelve patients showed normal cognitive function (MMSE: 29.8 ± 0.4, FAB: 18 ± 0) before surgery. No evident laterality of 123I-IMZ uptake was seen (IMZ-ACR: 0.98 ± 0.04). Neither cognitive function nor 123I-IMZ SPECT worsened after surgery (MMSE: 29.8 ± 0.3, FAB: 18 ± 0, IMZ-ACR: 1.00 ± 0.04). By contrast, 4 patients presented cognitive dysfunction (MMSE: 24.3 ± 3.9, FAB: 14.8 ± 2.7) before revascularization. Preoperative imaging of these patients showed decreased 123I-IMZ uptake, and their IMZ-ACRs (0.83 ± 0.08) were significantly lower than those of the normal group. After revascularization, cognitive functions and 123I-IMZ uptake tended to ameliorate (MMSE: 27.5 ± 1.7, FAB: 16.3 ± 2.2, IMZ-ACR: 0.94 ± 0.09). CONCLUSION: Preoperative cognitive function was associated with 123I-IMZ uptake in adult patients with MMD. After revascularization, cognitive function could be recovered in the viable areas of the brain, which is consistent with 123I-IMZ SPECT findings.

Spinal glomus AVM presenting solely with groin pain: illustrative case.

BACKGROUND: Spinal glomus arteriovenous malformations (AVMs) are rare and can cause neurological morbidity due to spinal hemorrhage, venous hypertension, or mass effect. OBSERVATIONS: The authors presented a rare case of spinal glomus AVM presenting with groin pain due to nerve root compression by a feeder aneurysm. A 41-year-old woman was referred to the hospital with initial right groin pain that had worsened over 2 months. Magnetic resonance imaging showed intra- and extramedullary abnormal flow voids at the T11-12 level, and spinal angiography revealed an intramedullary AVM, with extramedullary protrusion of an aneurysm on the feeder vessel, which arose from the sulcal artery of the anterior spinal artery. Because compression of the right L1 nerve root by the aneurysm was the likely cause of the patient's pain, endovascular embolization was performed. The feeder aneurysm disappeared after partial n-butyl 2-cyanoacrylate embolization, and the groin pain disappeared immediately after treatment. Her clinical status has been stable with no recurrence during 1 year of follow-up. LESSONS: This is the first report of glomus-type AVM presenting with radiculopathy alone. One should not overlook the possibility of spinal AVM among patients with groin pain.

PepYLCIV and PepYLCAV resistance gene Pepy-2 encodes DFDGD-Class RNA-dependent RNA polymerase in Capsicum.

KEY MESSAGE: A begomovirus resistance gene Pepy-2 encoding the DFDGD-Class RNA-dependent RNA polymerase 3a was identified in pepper (C. annuum) through the forward and reverse genetic analyses. In several countries throughout the world, the whitefly-transmitted begomovirus causes massive yield losses in pepper (Capsicum spp.) production. Although introgression of the genetic resistance against begomovirus to commercial cultivars is strongly required, the recently discovered recessive resistance gene pepy-1, which encodes the messenger RNA surveillance factor Pelota, is the only begomovirus resistance gene identified in Capsicum so far. In this study, we fine-mapped another begomovirus resistance gene from PG1-1 (C. annuum), which is resistant to pepper yellow leaf curl Indonesia virus (PepYLCIV) and pepper yellow leaf curl Aceh virus (PepYLCAV), to further speed up the marker-assisted breeding of begomovirus resistance in peppers. A single dominant locus, Pepy-2, conferring resistance against PepYLCIV in PG1-1 was identified on chromosome 7 by screening recombinants from the F2 and F3 segregating populations derived from a cross between PG1-1 and begomovirus susceptible SCM334. In the target region spanning 722 kb, a strong candidate gene, the RNA-dependent RNA polymerase 3a (CaRDR3a), was identified. The whole-genome and transcriptome sequences of PG1-1 and SCM334 revealed a single Guanine (G) deletion in CaRDR3a first exon, causing a frameshift resulting in loss-of-function in SCM334. In addition, multiple loss-of-function alleles of CaRDR3a were identified in the reference sequences of C. annuum, C. chinense, and C. baccatum in the public database. Furthermore, virus-induced gene silencing of CaRDR3a in PG1-1 resulted in the loss of resistance against PepYLCIV. PG1-1 and the DNA marker developed in this study will be useful to breeders using Pepy-2 in their breeding programs.

Endoscope-Integrated Fluorescence Video Angiography for the Surgery of Ventrally Located Perimedullary Arteriovenous Fistula at Craniocervical Junction.

BACKGROUND: Intraoperative confirmation of the vascular anatomy and blood flow contributes to the safety of the surgery for perimedullary arteriovenous fistulas (PAVF). However, because the PAVF at the craniocervical junction (CCJ) is mainly located on the ventral spinal cord surface, it is difficult to observe the entire pathology by a conventional surgical approach. To achieve increased viewing angle and visualization of real time blood flow, we introduced endoscope-integrated fluorescein video angiography in the treatment for PAVF at the CCJ for the first time. CASE DESCRIPTION: A 63-year-old man presented with subarachnoid hemorrhage due to rupture of PAVF at the CCJ, fed by both the right C1 radiculomedullary artery and the anterior spinal artery (ASA). Suboccipital craniotomy and C1 hemilaminotomy was performed and microscopic observation revealed partial anatomy of the PAVF covered by subarachnoid clots on the ventrolateral surface at the right C1 nerve root level. However, pathology ventral to the C1 nerve root was obscure and an endoscope-integrated fluorescein video angiography was introduced, which clearly demonstrated the PAVF components and the ASA. CONCLUSIONS: According to these findings, the PAVF was coagulated and the ASA was preserved. Endoscope-integrated fluorescein video angiography allowed to visualize its real-time blood flow, leading to a safe and reliable treatment.

Iodine-123-Iomazenil SPECT Revealed Recovery of Neuronal Viability in Association with Improvement in Symptoms Following Treatment for Obstructive Hydrocephalus due to a Giant Posterior Cerebral Artery Aneurysm.

BACKGROUND: Early and late images of 123I-iomazenil (123I-IMZ) single-photon emission computed tomography (SPECT) are considered to show cerebral blood flow and neuronal activity, respectively, and this modality may demonstrate temporal dysfunction of the frontal lobes in obstructive hydrocephalus. In this report, we examined 123I-IMZ SPECT in a patient with chronic obstructive hydrocephalus owing to compression of the aqueduct by a partially thrombosed aneurysm of the left posterior cerebral artery for the first time. CASE DESCRIPTION: A woman aged 77 years presented with progression of cognitive decline, gait disturbance, and urinary incontinence. She had a medical history of epilepsy and subarachnoid hemorrhage due to a ruptured left posterior cerebral artery aneurysm, treated conservatively when she was age 56 years. Magnetic resonance imaging revealed a mass lesion in the pineal region, which showed a target sign with gadolinium-based contrast agents, causing obstructive hydrocephalus owing to compression of the cerebral aqueduct. A right vertebral angiogram confirmed the presence of a partially thrombosed giant aneurysm at the left posterior cerebral artery. To rule out the involvement of nonconvulsive status epilepticus in her pathology, we performed 123I-IMZ SPECT, and both early and late images demonstrated low uptake in the bilateral frontal cortex. After surgical trapping of the parent artery and resection of the aneurysm, hydrocephalus was relieved, and the symptoms disappeared along with improvement in early and late 123I-IMZ SPECT images. CONCLUSIONS: The findings in the present case indicate that 123I-IMZ SPECT can detect reversible cerebral blood flow reduction and neuronal viability in the frontal lobes, which may affect the clinical manifestation of obstructive hydrocephalus.