Adult T-cell leukemia-lymphoma complicated by Takotsubo cardiomyopathy and HTLV-1-associated myelopathy after treatment with the anti-CCR4 antibody mogamulizumab.

Adult T-cell leukemia-lymphoma (ATL) caused by human T-lymphotropic virus type 1 (HTLV-1) is generally associated with poor prognosis. The anti-CCR4 antibody mogamulizumab is one of the options for refractory or relapsed ATL. Mogamulizumab is intravenously administered as a single agent at a dose of 1.0 mg/kg once a week for 8 weeks. The overall response rate is reported to be 50%. Reported common adverse events after administration of mogamulizumab are leukocytopenia, infusion reaction, and skin rash. Although rare, mogamulizumab-associated cardiomyopathy has also been reported. HTLV-1 is also associated with a number of diseases, such as HTLV-1-associated myelopathy (HAM). Approximately 0.25% of infected individuals are estimated to develop HAM. HAM is an inflammatory disease of the central nervous system, which is characterized by slowly progressive spastic paresis of bilateral lower limbs and bladder-rectal disorder. Coexistence of ATL and HAM has rarely been reported. Here we present the case of a patient with ATL in whom Takotsubo cardiomyopathy and HAM developed after mogamulizumab administration.

New-onset schizophrenia in an adolescent after COVID-19.

Schizophrenia develops during adolescence. Maternal infections during the fetal period increase the incidence of schizophrenia in children, which suggests that the pathogenesis involves neuroinflammation. Here, we report a case of new-onset schizophrenia in a 16-year-old boy after COVID-19. After developing COVID-19, he entered a catatonic state 4 days later and was hospitalized. Benzodiazepines alleviated his catatonia, but hallucinations and delusions persisted. Encephalitis and epilepsy were excluded by magnetic resonance imaging (MRI), encephalography, and cerebrospinal fluid examination. Psychosis persisted after the virus titer declined and the inflammatory response subsided. Moreover, the patient exhibited delusions of control-a Schneider's first-rank symptom. Schizophrenia was diagnosed, and olanzapine improved his symptoms. He had a brief history of insomnia before COVID-19 but his symptoms did not satisfy the ultra-high-risk criteria. However, COVID-19 may have facilitated development of schizophrenia through neuroinflammation and volume reduction in the gray matter of the right medial temporal lobe. This case demonstrates that infectious diseases in adolescents should be carefully managed, to prevent schizophrenia.

A Case of Unilateral Recurrent Nerve Palsy with Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disease in which peripheral sensory and motor nerves of the four limbs are impaired due to autoimmune mechanism-induced demyelinating changes through a 2-month or longer chronic course. The incidence of complication by cranial neuropathy has been reported to be 15%, but there have been very few reports on disorder of the vagus nerve and its branch, the recurrent nerve. We report a patient who developed left recurrent nerve palsy with CIDP. The patient was a 48-year-old male. The disease developed as progressive muscle weakness and numbness of the four limbs 3 years before and was diagnosed as CIDP. The symptoms had been improved by high-dose intravenous gamma-globulin therapy. However, from 2 months before he became aware of breathy hoarseness, and bilateral decreased grip strength and sensory disturbance of the upper and lower limbs recurred and progressed. On laryngoscopy disorder of left vocal fold movement and glottal closure incompetence during phonation were observed, and neurogenic changes were detected in the left thyroarytenoid muscle by needle electromyography for the intrinsic laryngeal muscles. High-dose intravenous gamma-globulin therapy was performed and left vocal fold movement recovered with recovery of bilateral grip strength and sensory disturbance of the upper and lower limbs, and phonation was also normalized.

Neuromyelitis optica with rapid respiratory failure: a case report.

BACKGROUND: Neuromyelitis optica is an inflammatory demyelinating disease of the central nervous system and is characterized by severe optic neuritis and transverse myelitis. CASE PRESENTATION: The patient was a 74-year-old man with pneumonia. On admission, he exhibited lower limb weakness and rapid respiratory deterioration in the form of tachypnea. Subsequently, he was transported to the Emergency Center of our hospital. High-signal lesions were observed from the cervical spinal cord to the thoracic spinal cord on T2-weighted spinal magnetic resonance images. Neuromyelitis optica was suspected, and the patient received steroid pulse therapy and immunoadsorption plasmapheresis. Serum samples obtained upon transfer were positive for anti-aquaporin-4 antibodies, which confirmed the diagnosis of neuromyelitis optica. Thereafter, the patient was transferred to a rehabilitation hospital. CONCLUSION: Rapid respiratory failure in neuromyelitis optica is rare, and care is needed while treating these cases.

Effect of brain atrophy in quantitative analysis of 123I-ioflupane SPECT.

OBJECTIVE: Dopamine transporter (DAT) imaging such as 123I-ioflupane (123I-FP-CIT) SPECT is a useful tool for the diagnosis of parkinsonism and dementia. The Southampton method is the quantitative method for evaluating 123I-FP-CIT SPECT and is less affected by the partial volume effect of the striatum. The method may be vulnerable to contamination by low-uptake areas of cerebrospinal fluid in whole brain, and the threshold of voxel value (threshold method, TM) was developed to correct the contamination. The purpose of this study is to evaluate the TM in the patients with neurological disease. METHODS: We studied 99 subjects, including 39 patients with Alzheimer's disease (AD), 15 patients with Parkinson's disease (PD) and 10 patients with dementia with Lewy bodies (DLB). Each subject had undergone 123I-FP-CIT SPECT. We calculated the SBR with and without the TM. The SBR laterality was assessed using the asymmetry index (AI). We investigated the relationship between the SBR change with TM and brain atrophy, which were assessed using Evans index (EI), sylvian index (SI) and cerebral atrophy index (CAI). Cutoff value for EI was 0.3, and cutoff values for SI and CAI were the first quartile, respectively. RESULTS: The SBR with TM was 0.53 percentage points lower than the SBR without TM overall (p < 0.01). Positive and negative reversal of AI increased with age. The rate of the SBR change with TM was tended to be lower in groups with brain atrophy. The number of voxels excluded by TM in striatal volumes of interest (VOIs) was larger with high groups for EI, SI and CAI than in low groups. The number of voxels excluded using TM in reference VOIs was related to SI. CONCLUSIONS: The SBR was decreased using TM. The effect of TM on the SBR tended to be small in the subjects with severe brain atrophy. The effect of brain atrophy in the TM is larger in the striatal VOIs than in the reference VOIs. Even if quantitative analyses are available, visual assessment of 123I-FP-CIT SPECT is essential for diagnosis.

A case of headache attributed to otitis media chronica cholesteatomatica with cerebral sigmoid sinus thrombosis.

Otitis media chronica cholesteatomatica with cerebral sigmoid sinus thrombosis is an important differential diagnosis in the evaluation of headache. We describe a 31-year-old Filipino man with chief complaints of headache, otalgia, vomiting, and vertigo, and no significant past medical history. Two years before admission he stuffed tissues into the right external auditory canal because of a noise in the street on the night of the New Years festival and sometimes had right ear discharge. One month before admission he had a right occipital headache with right otalgia and fever. One day before admission he vomited. Vertigo developed on the day of admission. On physical examination at admission, the patient was somnolent and had a body temperature of 36.9 degrees C, and meningeal signs were obvious. Magnetic resonance of the brain revealed right otitis media chronica cholesteatomatica and right cerebral sigmoid sinus thrombosis. Computed tomography of the skull base revealed that the tympanic cavity and mastoid air cells were filled with a mass of soft-tissue density invading toward the sigmoid sinus. Cerebrospinal fluid examination showed a cell count of 32/3 mm3. The patients level of consciousness and symptoms improved after administration of ampicillin and ceftriaxone sodium. A diagnosis of "headache attributed to disorder of ears," with The International Classification of Headache Disorders, 2nd edition (ICHD-II) code 11.4, was made on the basis of symptoms and imaging findings. Otitis media chronica cholesteatomatica had invaded toward the sigmoid sinus and was thought to be the cause of cerebral sigmoid sinus thrombosis and meningitis. Six weeks after admission the patient underwent mastoidectomy and tympanoplasty to cure the cholesteatoma and prevent recurrence of inflammation. The postoperative progress was satisfactory. In cases of headache with otalgia, "headache attributed to disorder of ears" should be considered.

Effect of glycerol on ischemic cerebral edema assessed by magnetic resonance imaging.

The aim of this study is to assess the anticerebral edema effect of glycerol on a large cerebral infarction with magnetic resonance imaging (MRI). Glycerol, which is widely used as an osmotic agent against cerebral edema, could exacerbate brain tissue shift, since it has been suggested that glycerol might shrink a noninfarcted hemisphere and worsen the mass effect after a large hemispheric cerebral infarction. To investigate these issues, changes in a large hemispheric infarction with cerebral edema were studied using MRI before and after glycerol administration. Infarct volumes, normal brain tissue volumes and lateral ventricle volumes, in addition to signal intensities of T(2)-weighted images, were measured in six patients before and after administration of 300 ml of glycerol. Ventricle volumes were significantly increased (p=0.0015) and the T(2) signal intensity of the post-treatment ischemic region decreased after glycerol administration. In contrast, no significant differences in either cerebral volume or T(2) signal intensity were seen in the noninfarcted hemisphere before and after administration. Our data suggest that glycerol does not exacerbate the mass effect on a large hemispheric infarction.

Efficacy of tracheostomy for central alveolar hypoventilation syndrome caused by lateral medullary infarction.

Central alveolar hypoventilation syndrome (CAHS) is a rare and potentially fatal condition. However, respiratory care for patients with CAHS caused by lateral medullary infarction (CAHS-LMI) remains an important unsolved problem. We describe 2 patients with CAHS-LMI and review the case reports for 17 previously described patients. Patient 1 was a 78-year-old man who was referred to our hospital because of dizziness. After admission, Wallenberg syndrome developed. Magnetic resonance imaging showed left LMI. He had hypercapnia and respiratory acidosis the next afternoon and temporarily received mechanical ventilation. A tracheotomy was performed on the 12th hospital day, and the patient was weaned from the ventilator on the 18th hospital day. Patient 2 was 72-year-old man who was referred to our hospital because of dizziness and gait disturbance. Wallenberg syndrome was diagnosed after admission, and magnetic resonance imaging showed right LMI. His consciousness deteriorated, and hypercapnia developed on the ninth hospital day. The patient received ventilatory support, and a tracheotomy was performed on the 12th hospital day. He was weaned from the ventilator by the 16th hospital day. Consistent with our findings, most previously reported cases of CAHS-LMI were initially associated with mild symptoms, which subsequently worsened. Five of the 19 patients (26.3%) died within 1 month after onset, and 7 patients (36.8%) died within 1 year. Tracheotomy was performed in 12 patients, 2 of whom died 1 month after onset (16.7%); another patient died of chronic renal failure after 2 years. Tracheotomy seemed to be an effective procedure in patients with CAHS-LMI. We speculate that tracheotomy assists alveolar ventilation by reducing dead space ventilation. Closure of the tracheotomy should, therefore, be avoided in patients with CAHS-LMI, even if respiratory status is good.

Thrombosis in Japanese patients with Fabry disease.

Fabry disease is an X-linked lysosomal storage disease resulting from deficient activity of the enzyme alpha-galactosidase (alpha-Gal) A. It has been postulated that the accumulation of globotriaosylceramide in the endothelial cells of blood vessels may lead to thrombosis of the brain and other tissues. Recently, enzyme replacement therapy (ERT) for Fabry disease is available. A high incidence of thrombotic accidents in Fabry disease has been postulated. However, a systemic study on thrombosis in cases of Fabry disease has not been undertaken. To clarify the incidence of thrombosis in Fabry disease, we screened 65 patients with Fabry disease (49 hemizygotes and 16 heterozygotes) from 39 unrelated Japanese families. We found that ten patients with Fabry disease (7 hemizygous males and 3 heterozygous females) had experienced thrombotic accidents, under 45-years-old in 8 cases. These 10 patients showed the gene mutations of classical Fabry disease. Nine of these thrombotic patients developed brain infarctions, one man who had the complication of recurrent thrombophlebitis, and the remaining woman showed central retinal artery occlusion and thrombophlebitis. We demonstrated a high incidence of thrombosis in Fabry disease (15%). ERT should be performed in patients not only in hemizygous males but also in heterozygous females and started at their early ages.