Utility of transoral motion-mode ultrasonography to detect tongue fasciculation in patients with amyotrophic lateral sclerosis.

INTRODUCTION: Increasing evidence suggests the utility of the submandibular approach for ultrasonography to detect tongue fasciculation in amyotrophic lateral sclerosis (ALS). We hypothesized that transoral motion-mode ultrasonography (TOMU) would be useful to detect tongue fasciculation in patients with ALS. METHODS: Patients with sporadic ALS showing clinically definite tongue fasciculation were enrolled, and the ultrasonography findings of patients' tongues on TOMU and ultrasonography by the conventional submandibular approach were analyzed. RESULTS: Six patients with clinically definite ALS were enrolled in this study. Although small, irregular muscle movements of 5 to 10 mm in amplitude and 0.1 to 0.2 second in duration were detected in all patients by TOMU, similar muscle movements were detected in only two of the six patients by the submandibular approach. DISCUSSION: TOMU appeared to be useful for detecting tongue fasciculation in ALS patients. Further study is needed to better determine its role as a diagnostic tool for ALS.

The Risk Factors for Death within 6 Months After Ischemic Stroke in Patients with Cancer.

OBJECTIVES: While the intravenous recombinant tissue plasminogen activator (rt-PA) therapy for acute ischemic stroke patients with cancer is recommended when survival of ≥ 6 months is expected, the risk factors for death and stroke recurrence within 6 months after stroke are not well known. Thus, we aimed to identify markers for death and recurrence risks within six months from stroke onset in patients with cancer. MATERIALS AND METHODS: In a retrospective cohort study, the subjects comprised acute ischemic stroke patients with cancer hospitalized at St. Marianna University hospital from 2008 through 2019. To evaluate the associations between the clinical factors within 24 h of the initial stroke and death or stroke recurrence events within 6 months from stroke onset, Logistic analysis and Cox proportional hazards regression analysis was used respectively. Next, the optimal cutoff point of markers for different mortality groups was determined using the receiver operating characteristic curve analysis and cumulative outcome rate of each group was compared using the Kaplan-Meier method. RESULTS: Among 194 patients with cancer who developed acute stroke, 167 were ultimately selected for analysis. 47 subjects (28.14%) passed away within 6 months following stroke onset, and 20 subjects (11.98%) had stroke recurrence. High D-dimer levels, low fibrinogen levels, high Glasgow prognostic scores (GPS), and multiple vascular territory infarctions was independently associated with death, where higher death rate was significantly confirmed in the group with D-dimer levels of ≥3.95 mg/dl, fibrinogen levels <277.5 mg/dl and GPS scores of 2. Low fibrinogen level, lack of antithrombotic therapy, and the presence of metastasis were associated with stroke recurrence. CONCLUSIONS: When patients with cancer suffer stroke, D-dimer levels, fibrinogen levels, GPS, and multiple vascular territory infarctions would be associated with the risk of death within 6 months. Low fibrinogen levels, lack of antithrombotic therapy, and the presence of metastasis correlated with high risk of stroke recurrence.

Association of Progressive Cerebellar Atrophy With Long-term Outcome in Patients With Anti-N-Methyl-d-Aspartate Receptor Encephalitis.

IMPORTANCE: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an immune-mediated disorder that occurs with IgG antibodies against the GluN1 subunit of NMDAR. Some patients develop reversible diffuse cerebral atrophy (DCA), but the long-term clinical significance of progressive brain and cerebellar atrophy is unknown. OBJECTIVE: To report the long-term clinical implications of DCA and cerebellar atrophy in anti-NMDAR encephalitis. DESIGN, SETTING, AND PARTICIPANTS: A retrospective observational study and long-term imaging investigation was conducted in the Department of Neurology at Kitasato University. Fifteen patients with anti-NMDAR encephalitis admitted to Kitasato University Hospital between January 1, 1999, and December 31, 2014, were included; data analysis was conducted between July 15, 2015, and January 18, 2016. EXPOSURES: Neurologic examination, immunotherapy, and magnetic resonance imaging (MRI) studies were performed. MAIN OUTCOMES AND MEASURES: Long-term MRI changes in association with disease severity, serious complications (eg, pulmonary embolism, septic shock, and rhabdomyolysis), treatment, and outcome. RESULTS: The clinical outcome of 15 patients (median age, 21 years, [range, 14-46 years]; 10 [67%] female) was evaluated after a median follow-up of 68 months (range, 10-179 months). Thirteen patients (87%) received first-line immunotherapy (intravenous high-dose methylprednisolone, intravenous immunoglobulin, and plasma exchange alone or combined), and 4 individuals (27%) also received cyclophosphamide; 2 patients (13%) did not receive immunotherapy. In 5 patients (33%), ovarian teratoma was found and removed. Serious complications developed in 4 patients (27%). Follow-up MRI revealed DCA in 5 patients (33%) that, in 2 individuals (13%), was associated with progressive cerebellar atrophy. Long-term outcome was good in 13 patients (87%) and poor in the other 2 individuals (13%). Although cerebellar atrophy was associated with poor long-term outcome (2 of 2 vs 0 of 13 patients; P = .01), other features, such as DCA without cerebellar atrophy, serious complications, ventilatory support, or prolonged hospitalization, were not associated with a poor outcome. Five patients with DCA had longer hospitalizations (11.1 vs 2.4 months; P = .002), required ventilatory support more frequently (5 of 5 vs 4 of 10 patients; P = .04), and developed more serious complications (4 of 5 vs 0 of 10 patients; P = .004) compared with those without DCA. Although DCA was reversible, cerebellar atrophy was irreversible. CONCLUSIONS AND RELEVANCE: In anti-NMDAR encephalitis, DCA can be reversible and does not imply a poor clinical outcome. In contrast, cerebellar atrophy was irreversible and associated with a poor outcome. This observation deserves further study to confirm progressive cerebellar atrophy as a prognostic marker of poor outcome.

[Life-threatening airway obstruction accompanied by vocal cord paralysis due to indwelling nasogastric tube in malnourished elderly patients: a report of four cases].

We report 4 cases of elderly patients with abrupt onset of serious airway obstruction that is presumed to be due to indwelling nasogastric tube. 2 cases are patients of cerebral infarction and 2 cases are patients of Parkinson disease. The average number of days until NGTS is 17.8 days. In all cases, fiber-optic examination revealed complete loss of adduction in both vocal cords. Infection in the posterior cricoid region caused by ulcerative lesions at the upper end of the esophagus has been implicated as a pathophysiological mechanism of this syndrome, but it was not possible to confirm in the 4 cases. Because it is difficult to exactly diagnose with NGTS in clinical practice, there is a need to consider the inducing factor and response. Body mass index is very low in each of the 4 cases, ranging from 14.2 to 18.0, implying a severely malnourished or immunocompromised state, and may represent a high risk factor for this syndrome. Whenever this life-threatening syndrome is suspected, direct vocal cord examination and removal of the tube are recommended. In addition, the clinicians should not hesitate about doing intubation or tracheotomy in emergency.

Vim thalamotomy in a patient with Holmes' tremor and palatal tremor - Pathophysiological considerations.

BACKGROUND: We peformed a ventral intermediate nucleus (Vim) thalamotomy in a patient with Holmes' tremor and palatal tremor. The frequencies of these movement disorders were 4 Hz and 3 Hz, respectively. Vim thalamotomy stopped the Holmes' tremor but not the palatal tremor. Our observations suggest different mechanisms for these two involuntary movements. CASE PRESENTATION: A 57-arm 11 months after a pontine hemorrhage. Transoral carotid ultrasonography revealed periodic motion of her posterior pharyngeal wall with a frequency of 3 Hz. Recording of neuronal activities in the thalamus revealed a 4Hz rhythmic discharge time that was associated with her tremor in the contralateral arm. A left Vim thalamotomy was performed. The resting tremor of the upper limb stopped, but the kinetic tremor recurred 6 months after the thalamotomy. No effect was observed on her palatal tremor. CONCLUSIONS: The different effects of Vim thalamotomy on the Holmes' tremor and palatal tremor suggest different oscillation sources for these two involuntary movements.

[A case report of cerebral amyloid angiopathy-related inflammation treated with cyclophosphamide].

A 78-year-old woman was admitted to another hospital with progressive cognitive dysfunction and right hemiparesis. Magnetic resonance imaging (MRI) of the brain revealed a high-intensity area in bilateral frontal lobes on T2-weighted imaging (T2WI) and fluid-attenuated inversion recovery (FLAIR). Brain biopsy revealed no significant findings. She was transferred to our hospital for further examination. Although the etiology of the white matter lesion was not specified, the lesion and symptoms improved with steroid pulse therapy. Three months later, disturbance of consciousness and right hemiparesis worsened again, and the white matter lesion was expanded on MRI. Steroid pulse therapy was again performed, but her condition gradually deteriorated and akinetic mutism developed. T2-based imaging showed microbleeds and analysis of the apolipoprotein E (ApoE) genotype showed ε4/ε4; therefore, cerebral amyloid angiopathy-related inflammation was diagnosed. Two cycles of cyclophosphamide pulse therapy were performed, resulting in improvement of the white matter lesion, disorder of consciousness, and hemiparesis after the first cycle. The patient was discharged from our hospital in an ambulatory state. Steroid therapy for cerebral amyloid angiopathy-related inflammation is relatively well-known in Japan, but cyclophosphamide therapy appears useful for steroid-resistant amyloid angiopathy-related inflammation in the early stage of the disease.

A case of rhabdomyolysis in which levetiracetam was suspected as the cause.

Several studies have reported rhabdomyolysis induced by various drugs but not by the antiepileptic drug levetiracetam. We present a case of suspected levetiracetam-induced rhabdomyolysis. A 29-year-old woman was hospitalized for generalized tonic-clonic seizure and given levetiracetam for the first time. One day after starting levetiracetam, she developed myalgia, particularly backache, and weakness in both lower limbs. Based on her clinical symptoms and blood test results indicating hyperCKemia, our diagnosis was levetiracetam-induced rhabdomyolysis. Withdrawal of levetiracetam immediately improved the clinical symptoms and hyperCKemia. This first report of suspected levetiracetam-induced rhabdomyolysis provides important information for treating patients early in levetiracetam administration.

Rest-induced suppression of anterior horn cell excitability as measured by F waves: comparison between volitionally inactivated and control muscles.

To test the hypothesis that the anterior horn cells become hypoexcitable in the absence of central drive, we recorded F waves simultaneously from the first dorsal interosseous (FDI) and the abductor digiti minimi (ADM) before and after volitionally inactivating one muscle (target) while periodically contracting the other muscle (control). In 14 healthy subjects, F waves recorded from the target muscle showed a progressive decrease in persistence and amplitude (whether counting all 100 trials or only detectable responses) after muscle relaxation for 1, 3, and 6 hours, followed by a quick recovery upon brief muscle contraction. We conclude that volitional inactivation suppresses the F waves of the target muscle without equally affecting the control muscle innervated by the same nerve. The history of activity of a muscle should therefore be taken into account in clinical testing, especially when the study of a paretic muscle shows abnormal F-wave excitability.

[A case of ventriculitis with bacterial meningitis occurred during the treatment of liver abscess].

A 47-case-year old male was admitted to our hospital because of high fever and general fatigue. He had no immune deficiency, and had no other disease in his past history. On admission, the white blood cell count and C-reacted protein were severely elevated (18,700/microliter, 27.7 mg/dl, respectively) and abdominal CT revealed multiple low density, From these results, he was diagnosed as liver abscess. Intravenous MINO and SBT/CPZ injection were started. On the fifth hospital day, he suffered from headache and nuchal rigidity. The clinical data revealed the cerebro-spinal fluid (CSF) counting 8,336 cells/mm3 (mononuclear 8,000,) protein at 119 mg/dl, and sugar 42 mg/dl. CSF cultures were negative, but Klebsiella was recognized in the blood culture and drainage fluid in liver abscess. This condition was diagnosed as bacterial meningitis and antibiotics were changed to intravenous CTRX and MEPM. Furthermore we administered oral PSL and intravenous steroid-pulse therapy. After these combination therapies his condition improved gradually. After 40 hospital day, however, he suddenly had double vision, Axial FLAIR (SE6,000/120) image revealed with high signal intensity at 4th ventricle. Intravenous MEPM was administered again. On the 60th hospital day, double vision was gradually improved and abnormal intensity at 4th ventricle was almost disappeared. This case may provide us a considerable suggestion on the treatment of bacterial meningitis.