Case Reports in Neurology最新文献

A 63-year-old woman under treatment of autoimmune hepatitis presented with headache, memory loss, and somnolence. Three months before admission, the patient experienced liver inflammation relapse after prednisolone (PSL) cessation. Consequently, PSL was resumed and then tapered. Cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with remarkably reduced glucose and elevated angiotensin-converting enzyme and soluble interleukin-2 receptor levels. Magnetic resonance imaging (MRI) revealed prominent bilateral periventricular white-matter lesions, hydrocephalus, ischemic stroke with gadolinium enhancement of frontoparietal and basilar meninges on contrast-enhanced fluid-attenuated inversion recovery. Magnetic resonance angiography (MRA) showed narrowing of the bilateral middle cerebral arteries. Based on these findings, we diagnosed the patient with neurosarcoidosis. Re-increment of PSL improved the neurological symptoms, CSF findings, and abnormalities found on MRI and MRA. This case suggests that neurosarcoidosis may occur as a complication of some autoimmune diseases during immunotherapy administration.

Ofatumumab is the first fully human anti-CD20 monoclonal antibody that, on March 26, 2021, was approved by the EMA to treat patients with relapsing multiple sclerosis. This paper aimed to present a case confirming the ability to produce and maintain anti-SARS-CoV-2 antibodies in a patient treated with ofatumumab for over 4 years. The course of the infection was moderate, and the patient did not require hospitalization. Antibody measurements were performed five times post-COVID-19 infection. The first test was performed in the fourth month (131 days), and the last, over 1 year after the infection. To date, only 2 cases have been published describing the ability of a patient treated with the same drug to produce antibodies against SARS-CoV-2, although the observation was conducted over a shorter period. In our case study, we have 15-month follow-up data. The patient was not vaccinated and additionally received suppressive steroid therapy due to the relapse. We observed an increase in antibodies up to 10 months from the infection. The case under analysis suggests that patients treated with ofatumumab, despite complete peripheral B-cell depletion, can produce and maintain a long-lasting immune response.

A 64-year-old man presented with left upper limb weakness and dysesthesia for 4 months. Magnetic resonance imaging demonstrated swelling from the 6th-8th left cervical nerve roots to the left brachial plexus. The serum IgG4 level was elevated (762.7 mg/dL). ¹⁸F-FDG-PET showed high uptake in the mediastinal lymph nodes, and biopsy revealed infiltration of IgG4-positive plasma cells. We diagnosed IgG4-related neuropathy, and steroid therapy administration improved the symptoms. IgG4-related disease should be considered in the differential diagnosis of peripheral nerve swellings. If biopsy of the disordered nerves is difficult, lymph nodes or other organs should be considered.

A 55-year-old woman with a history of allergic sinusitis was being administered cyclosporine for ptosis and diplopia due to myasthenia gravis since age 46 years. She developed painful dysesthesia that began in her feet and later spread to her palms, leading to difficulty in walking. Eosinophils were markedly increased in the peripheral blood. Nerve conduction studies revealed mononeuritis multiplex. Nerve biopsy showed the infiltration of eosinophils in the superior neurovasculature. Based on these findings, eosinophilic granulomatous polyangiitis was diagnosed. Methylprednisolone pulse therapy was followed by oral prednisolone. Two weeks after treatment, the patient could do normal daily activities without assistance. In patients with myasthenia gravis having a history of allergic diseases, considering EGPA as a complication and monitoring prior changes in blood data are necessary for early detection before apparent tissue damage.

Perimesencephalic subarachnoid hemorrhage (PMSAH) is a group of diseases characterized by bleeding around the brainstem. In recent years, it has been suggested that PMSAH is associated with the venous system. We report a case of PMSAH caused by stenosis of the junction of the vein of Galen (VG) and the rectus sinus (RS). A 39-year-old man presented with restlessness at work. He was administered diazepam, and a few minutes later, his consciousness became clear. Imaging showed subarachnoid hemorrhage (SAH) around the right midbrain, occlusion of the right sigmoid sinus and the right transverse sinus, stenosis of the junction of the VG and RS, T2 hyperintensity in the right middle temporal gyrus, and obstruction of the right vein of Labbe. The location of the SAH coincided with stenosis of the junction of the VG and RS. PMSAH was diagnosed due to the increase in intracranial venous pressure caused by the patient's sinus obstruction in addition to the stenosis of the junction of the VG and RS. It is necessary to pay attention to venous return when PMSAH is observed.

Although it is generally recognized that pure autonomic failure (PAF) is a progressive neurodegenerative disease selectively involving the autonomic nervous system, its mode of progression remains to be settled. A 57-year-old man presented with sweat reduction on the left side during previous 3 years. The thermoregulatory sweat test revealed left-sided multi-segmental hypohidrosis more markedly on the face. Pharmacological sweating was relatively preserved except for the face. During the subsequent 8 years, he developed erectile dysfunction and overt orthostatic hypotension. Plasma norepinephrine was markedly reduced without reactive increase during the tilt-table test. The heart to mediastinum ratio in ¹²³I-meta-iodobenzylguanidine cardiac scintigraphy was reduced. Over the following 3 years, he showed progressive and generalized postganglionic sudomotor impairment without cognitive impairment or somatic nervous dysfunctions. Present observations suggest that in some patients with PAF, pathological process might start mainly at the central level and later extends to the peripheral level.