We report the case of a 73-year-old right-handed female with a right parietal cerebral infarction and presented symptoms that were challenging to differentiate between alien hand sign (AHS) and sensory ataxia. She presented to our emergency department with chief complaints of abnormal involuntary movements and a feeling of foreignness on her left upper limb. The first neurological examination revealed left spatial neglect, left-side sensory impairment that included superficial and deep sensations, left limb-kinetic apraxia, and left limb ataxia. Furthermore, her symptoms and complaints had characteristics of AHS that includes a sensation that her left upper limb dose not belong to herself and an abnormal behavior of left hand that is contrary to her own intent. Brain MRI revealed an acute cerebral infarction confined to the right postcentral gyrus. This case highlights that sensory ataxia due to the disturbance of deep sensation might present symptoms similar to AHS. Previous studies suggested the involvement of the disturbance of somatosensory pathway in posterior-variant AHS. Therefore, a precise distinction between AHS and sensory ataxia, especially in posterior-variant AHS, is imperative to avoid confusion regarding the term “alien hand sign.”
A 62 year-old man, who was taking prednisolone for nephrotic syndrome, was diagnosed with herpes zoster of the trigeminal nerve and treated with oral valacyclovir. One month later, he reported pain from the right side of the head and vomiting. MRI revealed an acute infarction in the right frontal lobe and dissection of the internal carotid artery of the right cervix. Trauma or other potential triggers were not observed. In consideration of the preceding condition of varicella zoster virus infection, acyclovir was administered in addition to unfractionated heparin, but an intramural hematoma emerged in the left internal carotid artery. Furthermore, evidence showing progression of these lesions was found. On the fifth day, prednisolone was increased to 1 mg/kg/day, and progression of vascular lesions was not observed. This case may prove valuable because it suggests a relationship between cervical artery dissection and herpes zoster.
We report a case of acute disseminated encephalomyelitis (ADEM) concomitant with polyneuropathy associated with anti-lactosylceramide antibody. A 68-year-old man was admitted to our hospital with ophthalmoparesis, bulbar palsy, tetraplegia after suffering from upper respiratory infection and headache. Subsequently, he developed respiratory failure requiring mechanical ventilation. Fluid-attenuated inversion recovery (FLAIR) MRI showed high intensities in the pons and medulla, and a nerve conduction study revealed motor-dominant axonal polyneuropathy. Although the laboratory tests revealed the presence of anti-lactosylceramide antibody in his serum, he was diagnosed with acute disseminated encephalomyelitis concomitant with polyneuropathy. Whereas the intensive treatment with corticosteroids, plasmapharesis, and high-dose intravenous immunoglobulin (IVIg) brought a moderate improvement, his tetraparesis continued to exist.
A 32-year-old woman showed transient central type facial nerve palsy and bulbar symptoms. Brain MRI revealed high intensity signals in the cerebral white matter, splenium of corpus callosum, and posterior limb of internal capsule. Two elder brothers of the patient had distal dominant peripheral neuropathies in four limbs. In this family, the point mutation of GJB1 gene, encoding connexin 32, was revealed and X-linked Charcot-Marie-Tooth disease (CMTX1) was diagnosed. The presented case was a heterozygote of this mutation. She showed severe transient central nervous system (CNS) symptoms and subclinical demyelinating peripheral neuropathy. The CNS symptoms and alterations of brain images were very similar among three siblings. There are many reports on male patients with CMTX1 who show associated CN symptoms, but female patients are very rare. There has been no previous report of a CMTX1 patient similar to the patient presented here. The trigger factors have been recognized at the onset of transient CN symptoms in these cases. The prevention of these factors is important for the management of such patients.
We report the case of a 50-year-old female survivor of Hodgkin lymphoma (HL), who developed dropped head syndrome (DHS). The patient was diagnosed with HL at 20 years of age, and underwent chemo-radiotherapy, which led to complete remission. Undergoing supplemental therapy for post-radiation hypothyroidism, she had twin babies. She noticed white stains on her neck at the age of 30, and the decolored area gradually expanded. Sixteen years after the radiotherapy (RT), her posterior neck muscle strength began to decline. She had to make considerable efforts to keep her neck straight, and came to experience a severe pain in the neck and shoulders. The patient visited our department due to DHS at the age of 50. She had leukoderma, muscle weakness, and muscle atrophy in the neck and para-spinal region, which were consistent with the area of RT. The strength was preserved in the other parts of the muscle, including the proximal upper limbs. Sensory nerve disorder was not detected. The serum creatine kinase level was slightly elevated. Cervical spine or cervical cord disease that can cause DHS was not detected by MRI examination. The MRI and CT images revealed marked atrophy in the posterior neck and para-spinal muscles. The electromyogram revealed myopathic changes, and the cause of her DHS was diagnosed as radiation-induced myopathy. DHS is a well-known late-onset radiation injury, and Japanese cases have been reported in elderly persons with laryngeal or lung cancer. However, there have been no Japanese case reports of radiation-induced DHS due to RT against HL in younger persons. The patient had visited several clinics and hospitals before she came to our hospital, but RT-induced DHS had been overlooked. Greater recognition and consideration is required for DHS as one of the various issues arising after long passage of HL survivors.
A 72-year-old man was admitted to our hospital because of right upper limb monoplegia 8 hours after the initial intravitreal injection of aflibercept, which is an inhibitor of vascular endothelial growth factor. Magnetic resonance diffusion-weighted images showed recent ischemic lesions in the left corona radiata and the right superior frontal gyrus. Laboratory findings showed mild hyperfibrinolysis. A patent foramen ovale was diagnosed on transesophageal echocardiography; however, lower-extremity ultrasonography did not detect deep vein thrombosis. The source of embolism remained unknown. A possible mechanism of cerebral emboli in the present case was a rapidly induced hypercoagulative state due to transfer of aflibercept from the vitreous body to the systemic circulation.
A 15-year-old girl presented with non-progressive sensorineural hearing impairment in her right ear since her early childhood. She had normal intellectual development. When she was 15 years old, small deep white matter lesions around the lateral ventricles were incidentally detected in brain MRI studies through further investigation of auditory organs. Laboratory data including cerebrospinal fluid analysis and antibodies to aquaporin-4 or myelin-oligodendrocyte glycoprotein were normal. She was diagnosed as a congenital cytomegalovirus (CMV) infection based on the detection of CMV DNA from preserved umbilical cord tissue by real-time polymerase chain reaction. It should be kept in mind that a case of congenital CMV infection with normal intelligence may be underdiagnosed and that sensorineural hearing impairment from early childhood and deep white matter abnormalities can be key features giving rise to suspicion on congenital CMV infection.
Patient 1 was a 59-year-old woman receiving prednisolone for idiopathic hypereosinophilia. Brain MRI of patient 1 disclosed slight gadolinium enhancement at lesions, indicating inflammation. Patient 2 was a 32-year-old woman with systemic lupus erythematosus under immunosuppressive therapy. Brain biopsy of patient 2 showed balanced infiltration of CD8+ and CD4+ T lymphocytes at the sites of lesions. Both subjects were diagnosed as having progressive multifocal leukoencephalopathy (PML) shortly after the onset of neurological symptoms and were treated with a combination of mefloquine, mirtazapine, and risperidone. Both patients remain alive with improved neurological symptoms even after long-term follow-up (24 months in patient 1 and 45 months in patient 2). Although the prognosis of PML is very poor, our findings suggest that pharmacotherapy may be effective for patients with well-controlled immune reactions against the JC virus.
A 77-year-old woman with Parkinson’s disease presented with left chest pain. Physical examination revealed tenderness at her second left sternocostal joint. There was no skin rash. Chest CT revealed hyperostosis of the sternocostal joint, and cervical MRI showed vertebral osteosclerosis and osteolysis. 99mTc-MDP bone scintigraphy showed an increased activity in the sternocostal joint and vertebral column. The patient was diagnosed with SAHPO syndrome according to the diagnostic criteria. Her chest pain was relieved after oral administration of nonsteroidal anti-inflammatory drugs. Although pain is a common non-motor symptom of Parkinson’s disease, chest pain is relatively rare, according to a previous reports. When patients with Parkinson’s disease complain of chest pain, physicians should make an appropriate differential diagnosis after excluding emergent cardiovascular disease. To the best of our knowledge, this is the first report of Parkinson’s disease associated with SAPHO syndrome. The relationship between the two diseases is unclear. However, peripheral inflammation is known to exacerbate ongoing neuronal damage in neurodegenerative diseases, such as Parkinson’s disease. Therefore, systemic inflammation of SAPHO syndrome may affect the disease course of Parkinson’s disease.