Carpal tunnel syndrome is the most common initial manifestation of systemic wild-type amyloid transthyretin (ATTR) amyloidosis, a systemic and lethal form of amyloidosis, which can alternatively present as a focal disease. Herein, we present a case series of six men who underwent the removal of a portion of the transverse carpal ligament during carpal tunnel release surgery and were diagnosed with ATTR amyloidosis among a cohort of 38 cases. In addition, one of the six patients with ATTR amyloidosis had cardiac and gastric amyloidosis. Extraction of part of a ligament during carpal tunnel release surgery is a straightforward procedure that can help diagnose ATTR amyloidosis at an early stage using a simple approach. Considering the epidemiology of carpal tunnel syndrome and systemic wild-type ATTR amyloidosis, amyloidosis can be ruled out during carpal tunnel release surgery at least for men.

  Background : As carpal tunnel syndrome (CTS) produces non-specific symptoms similar to those seen in other conditions, such as cervical spondylosis or intracranial disease, patients with CTS are often referred to neurosurgeons. At our department, surgical treatment of CTS was started in January 2014. Herein, we summarize the surgical cases of CTS over 5 years since January 2014.

  Materials and Methods : Fifty-one hands of 42 patients with CTS were retrospectively reviewed based on medical records, outpatient visits, or telephone interviews. Patients with CTS were divided into two groups : FV group, including those who first visited our outpatient department ; and OP group, including those who had been followed-up on an outpatient basis. Self-assessment reports were used to assess the postoperative alleviation of symptoms and degree of patient satisfaction (satisfactory, acceptable, or dissatisfactory). The factors that caused dissatisfaction were analyzed.

  Results : The mean follow-up period was 24.9±16.1 months. CTS was diagnosed in 33 of 51 hands (64.7%) in the FV group and 18 of 51 hands (25.3%) in the OP group. Of the 33 patients in the FV group with CTS, 24 (72.7%) had been referred for sensory disturbance of the hand. The self-satisfaction assessment revealed patient dissatisfaction with 13 of 51 hands (25.3%). Of the 13 hands with a dissatisfactory outcome, 10 had stenosing tenosynovitis while two had pillar pain.

  Conclusion : Regardless of the medical history, CTS should be considered in patients with sensory impairment of the hands. Careful postoperative follow-up for 6 months is important, and clinicians must respond appropriately if stenosing tenosynovitis or pillar pain develops.

  Introduction : Neural tube defects are usually observed at the brain and lumbosacral levels. We report a case of adult cervical spina bifida occulta that led to myelopathy.

  Case presentation : A 66-year-old woman presented with 5-month history of pain from the right occipital region to the upper limb, dullness of the bilateral upper limbs, numbness of the bilateral lower limbs, and skill movement disturbance. Neurological examination revealed distal muscle weakness of the bilateral upper limbs, grasping power weakness of the right hand, hyperalgesia of the radial side of the right upper limb, and hyperreflexia of the lower limbs. Cervical spinal radiography indicated a vertebral arch defect and nonunion spinous process at the C6 level. Dynamic cervical spinal radiography revealed the dislocation of the left free-floating part of the dysraphism of the C6 spinous process. Cervical spinal extension lateral radiography revealed that the free-floating bony structure had moved forward. Cervical spinal computed tomography (CT) illustrated the rostral tip of the free-floating bony structure inserting in the spinal canal at the C5/C6 level. Cervical spinal magnetic resonance imaging detected a spinal canal stenosis and high-intensity lesion in the spinal cord at the C5/C6 level. The patient underwent fenestration of the C5/C6 vertebral arch and removal of the free bone inserting in the spinal canal. The postoperative course was uneventful, and her neurological symptom improved.

  Conclusion : We report an extremely rare case of myelopathy due to dynamic stress of the left free-floating part of the dysraphism of the C6 spinous process in an adult patient with cervical spina bifida occulta.

  Both lumbar canal stenosis (LCS) and peripheral arterial disease (PAD) can cause groin pain. Although differentiating the two pathologies is essential, it can be more challenging in patients with groin pain. We report the case of a 71-year-old woman with PAD who presented with left groin pain after surgery for LCS at L4/5. Before the surgery, she had paresis of her left toe and intermittent claudication (IC) caused by pain in the left groin and L5 dermatome. Magnetic resonance imaging (MRI) showed a lateral recess stenosis with slippage at L4/5. As the arterial pulses in the lower extremities were palpable with normal skin color, we diagnosed a neurogenic IC due to LCS despite a low ankle brachial index (ABI) of 0.54 (≤0.9). A decompressive surgery of the L5 nerve root completely relieved preoperative symptoms; however, IC with left groin pain recurred 3 months after the surgery. Physical examination, selective L5 nerve root block and imaging studies of the lumbar, hip joint, and pelvis could not identify the cause. Finally, a cardiologist noticed the poor color tone of her left sole and diagnosed a symptomatic PAD (left common iliac artery occlusion) with an ABI of 0.29. She underwent stent placement with dual antiplatelet therapy, resulting in the prompt disappearance of the vascular IC. A close review of the initial plain radiograph and computed tomography confirmed severe calcifications of the abdominal aorta and iliac arteries. Spine surgeons tend to overlook the possibility of a coexisting PAD in patients with degenerative changes corresponding to LCS, and physical findings can be insufficient to differentiate them. Therefore, PAD should be screened in every IC patient using the ABI. In the case of an ABI less than 0.9, further vascular examinations should be done.

  Introduction : Clinically significant intradural extramedullary hemangioblastomas are extremely rare in patients without von Hippel-Lindau disease (VHL), and a few reports have been published on the angiographic findings in such tumors.

  Case presentation : An 80-year-old man with no history of VHL presented with progressive gait disturbance. Magnetic resonance imaging showed an occupational lesion in the spinal canal at the thoracic level. The thoracic spinal cord was squeezed to the left ventral side. Vascular flow voids were found near the lesion, and the lesion showed a strong contrast effect. The feeding artery of the tumor was the pial branch of the posterior spinal artery through the left T3 intercostal artery, as confirmed by angiography. Operative findings revealed a clear margin between the tumor and the spinal cord. In addition, the tumor was adhered to the right T5 nerve root. The patient could walk without assistance after the removal of the tumor.

  Discussion : While considering tumor removal, it is important to identify its origin and feeding artery. Previous reports and our experience suggest that the origin of the intradural extramedullary hemangioblastoma is associated with the pia mater and that tumor grows outside the spinal cord along the posterior nerve root. Angiography is useful for clarifying the relationship among tumor, feeding arteries, and draining veins and for assessing the origin of the tumor.

  Conclusion : Intradural extramedullary hemangioblastoma is a rare disease. Therefore, it is necessary to accumulate case reports focusing on the origin and feeding artery of such tumors.