An improvement in efficacy treatment and development of the social support system has led to many patients with neurological disease being able to reach adulthood. Therefore health care for life from pediatrics to adulthood has become necessary. The Special Committee for Measures Against Transition from Pediatric to Adult Health Care of the Japanese Society of Neurology officially started to examine the current situation and issues of transition from pediatric to adult health care in July 2020. Pediatric neurologists and adult neurologists have an awareness of this issue of constructing a better transition from pediatric to adult health care. However, there are some tasks that need to be resolved in the medical system. We intend to improve the understanding of transition and assessment of medical service fees for transition in cooperation with the Japanese Society of Neurology and the Japanese Society of Child Neurology.

Background: Intravenous immunoglobulin (IVIg) have been administrated for the long time in patients with several autoimmune neuromuscular diseases. Eczematous eruption has been described as IVIg-induced adverse effect. Objective: The purpose of this study is to clarify the incidence and characteristic of IVIg-induced eczematous eruption in autoimmune neuromuscular disease. Methods: We retrospectively collected the data from 92 patients with autoimmune neuromuscular diseases, including 35 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 8 patients with multifocal motor neuropathy (MMN), 25 patients with myositis, 15 patients with Guillain–Barré syndrome (GBS), and 9 patients with myasthenia gravis (MG), who have administrated IVIg in Yamaguchi University Hospital. Results: There are 10 patients (6 CIDP/4 MMN), who had an eczematous skin reaction after IVIg infusion. The frequencies of IVIg-induced eczematous eruption were significantly higher in patients with multifocal acquired demyelinating sensory and motor (MADSAM) and MMN than in patients with GBS, myositis, and MG. In addition, corticosteroids or immunosuppressive drugs had been administrated before IVIg treatment more frequently in patients with myositis and MG than in those with MADSAM and MMN. Conclusion: MADSAM or MMN patients had more frequently IVIg-induced eczematous eruption than other autoimmune neuromuscular diseases. Pathophysiology of MADAM and MMN is considered to be cell-mediated immunity against the peripheral nerve and the accumulation of IgG in both epidermis and dermis of the hand after IVIg may induce the infiltration of inflammatory cells around the vessels in the skin, causing eczematous eruption in MADSAM and MMN.

The case is a 65 years old man. He noticed muscle weakness of lower limbs from 3 years ago. Dysesthesia was developed, He came in our hospital in X year. He was detected muscle weakness, sensory disturbance in distal lower limbs predominance and detected radicular significant swelling in spinal cord MRI and diagnosed it with chronic inflammatory demyelinating polyneuritis (CIDP). In the follow-up purpose of other diseases regularly, it was confirmed that nerve root was gradually swelling from around 7 years before the onset. The radicular swelling is one of the characteristics in CIDP, supports the diagnosis. This case was the valuable case that was able to chase image progress before the onset.

We report a 62-year-old woman with thymoma associated myasthenia gravis (MG). She had significant dysphagia and was treated with corticosteroids, intravenous immunoglobulin (IVIG), immunoadsorption plasmapheresis (IAPP), and immunosuppressive drugs, and the extended thymectomy. Her symptoms gradually improved, but 3 weeks after thymectomy, her bulbar symptoms recurred. Although she was treated with repeated IVIG and IAPP, her symptom remained. Finally, after starting eculizumab did her symptoms go into complete remission. This case suggests the efficacy of anti-complement therapy for postoperative exacerbation of MG.

The patient was a 14-year-old boy with two previous episodes of self-remitting right ophthalmoplegia with right temporal pain at ages 9 and 12. In 2019, he developed right eyelid ptosis and diplopia 2 days after a pulsating right-sided temporoparietal headache. Recurrent headaches with ophthalmoplegia responded to high-dose steroid therapy, and the clinical features resembled recurrent painful ophthalmoplegic neuropathy (RPON). RPON generally presents with MRI findings of hypertrophy and inflammation at the root of the oculomotor nerve, a vulnerable site of the blood-brain barrier. However, the imaging features in this case were different from those in typical cases of RPON, and oculomotor nerve inflammation was found in the cavernous sinus. The order of onset of headache and oculomotor nerve palsy differed in each recurrence, suggesting that both autoimmune and vascular mechanisms may have been involved in the onset of the disease in our case.

We report a patient with myelin oligodendrocyte glycoprotein (MOG) antibody positivity who manifested myelitis with right optic perineuritis (OPN) 6 years following left OPN. A 45-year-old man treated 6 years previously for left OPN developed ascending numbness in both legs, urinary dysfunctions, and constipation. Neurologic examination disclosed bilateral hypesthesia extending downward over the chest from the T8 level. No motor weakness was evident. Visual field testing showed dense peripheral constriction with intact central vision on the right and a smaller superior scotoma on the left. Visual acuity and funduscopic findings were normal. Results of routine serologic investigations and autoimmune antibody titers, including those of anti-aquaporin 4 antibody, were within normal limits, except that both serum and cerebrospinal fluid were positive for anti-MOG antibody. MRI displayed a longitudinal cord lesion extending from T2 to T9, as well as optic nerve sheath enhancement characteristic of OPN. The patient was diagnosed with myelitis in addition to OPN, both resulting from MOG antibody-associated demyelination. Patients with myelitis, require careful assessment of visual acuity and visual fields to detect possible accompanying OPN and ON. We suspect that OPN in some other patients may likewise be caused by anti-MOG antibody.

A 75-year-old man with a history of hypertension developed weakness and sensory disturbance in the extremities 1 week after upper respiratory tract infection and faced difficulty walking. Screening at the time of hospital admission revealed an incidental positive SARS-CoV-2 PCR test, and COVID-19 was diagnosed. Neurological findings showed dysarthria, dysphagia, absence of deep tendon reflexes in the extremities, distal-dominant muscle weakness, sensory disturbance, urinary retention and constipation. Nerve conduction studies showed prolonged distal latency, decreased conduction velocity, and poor F-wave response, leading to a diagnosis of COVID-19-associated Guillain–Barré syndrome (GBS). The patient was treated with intravenous immunoglobulin, and his neurological symptoms improved without the need of a ventilator. Anti-ganglioside autoantibodies were negative. The patient developed GBS during the infectious period of SARS-CoV-2 and was treated in the isolation ward by clinical staff with personal protective equipment. Because COVID-19-associated GBS can develop during the infectious period of SARS-CoV-2, it is important for neurologists to consider GBS and other neurological disorders as being potentially COVID-19-related, and to treat patients with COVID-19 accordingly.

A 68-year-old woman with Parkinson’s disease, who had previously undergone Roux-en-Y gastrojejunostomy for early gastric cancer, complained of wearing-off and troublesome dyskinesia that had progressed over 7-years. After the introduction of levodopa-carbidopa intestinal gel therapy (LCIG) by nasojejunal tube, she had a good clinical response. Percutaneous endoscopic gastrostomy with a jejunal extension tube was difficult in this case, due to lack of gastrostomy site and fibrous postoperative adhesion. We introduced LCIG by direct percutaneous endoscopic jejunostomy (D-PEJ) which offers a less invasive procedure to operative tube placement. The factors affecting the success of D-PEJ could interfere with transillumination, abdominal thickness and the location of other organs. We determined the optimum site of catheter insertion with the assistance of real-time 3D reconstruction CT-jejunography. She was discharged home on postoperative day 14 without any procedure-related complications. Real-time 3D reconstructive CT-jejunography guided D-PEJ is a useful method for a patient who benefit from LCIG with prior gastrojejunostomy.

A 9-year-old girl was admitted to our hospital with severe plantar pain, 7 days after the onset of Campylobacter jejuni enteritis. On admission, extremity strength and the deep tendon reflex were normal; however, there was difficulty in walking owing to plantar pain. Motor nerve conduction test showed no abnormalities. No spinal cord protein cell dissociation. Lumbar spine-enhanced MRI showed a 4th and 5th lumbar vertebrae nerve root contrast-enhanced effect. Gabapentin was effective in minimizing her pain, eventually enabling the patient to walk. Antiganglioside antibody tests on admission showed multiple positive results. Six months after the initial onset of symptoms, she had recovered completely. She was suspected with sensory Guillain–Barré syndrome (GBS). GBS subsequent to Campylobacter jejuni enteritis has been recognized as an acute motor axonal neuropathy; hence, this report is considered to be valuable.