Ultrasound-Guided Botulinum Toxin Injection with Factor VIII Administration for Post Stroke Spasticity in a Hemophilia A Patient

Chemodenervation with botulinum toxin (BTX) has been recommended for focal spasticity. BTX injection should be performed with caution in patients with bleeding disorders and/or receiving anticoagulation therapy. We present a case of BTX injection for post-stroke spasticity in a patient with hemophilia A who could not take oral spasmolytics due to chronic hepatitis C. To minimize the bleeding risk, we replaced factor VIII intravenously in accordance with the World Federation of Hemophilia guidelines for minor surgery. FVIII (3,000 IU) was administered 15 minutes before BTX injection. One day later, 2,000 IU was administered, and 2 days later, another 2,000 IU was administered. We performed the real-time Ultrasound-guided BTX injection three times, then spasticity and upper extremity function improved without adverse events. BTX injection can be considered as a treatment option for spasticity among patients with hemophilia.

Anatomical Correlates of Neuropsychological Deficits Among Patients With the Cerebellar Stroke

OBJECTIVE: To investigate the anatomical correlates of the neuropsychological deficits in patients with the cerebellar stroke. METHODS: We screened patients who were admitted to the National Rehabilitation Center with the cerebellar stroke between October 2012 and November 2016. The patients with the cerebellar stroke who underwent neuropsychological testing for which the Seoul Neuropsychological Screening Battery (SNSB) or the SNSB-II were enrolled. The neuropsychological function capacities were compared in accordance with the stroke type (hemorrhagic vs. ischemic) and the location (right/left anterior, right/left posterior intermediate, right/left posterior lateral lobe, and vermis). Mean z-scores were computed to compare the patient performances with the population averages. RESULTS: Twenty-six patients (15 with ischemic stroke and 11 with hemorrhagic stroke) with a mean age of 54.8±16.6 years were assessed 8.8±9.2 months after the stroke. Differences in the neuropsychological functioning according to the stroke type were not observed. All of the numerical subtests of the stroke patients showed significantly poorer performances compared with the population averages (mean z-score < 0), and some of the subtests revealed abnormal performances in attention-, visuospatial function-, memory-, and frontal/executive function-related tasks (mean z-score <−1). The patients with the presence of a lesion in the right posterior intermediate lobe of the cerebellum showed a poorer performance in the subtests evaluating the executive function including the Korean-version Stroop Test (p=0.04), the Digit Symbol Coding Test (p=0.01), and the Korean-version Trail Making Test (p=0.02) compared with the patients without that lesion. CONCLUSION: The present study confirms that the cerebellar stroke affects the neuropsychological functioning which is associated with the anatomical site of stroke.

Anatomical Correlates of Neuropsychological Deficits Among Patients With the Cerebellar Stroke

OBJECTIVE: To investigate the anatomical correlates of the neuropsychological deficits in patients with the cerebellar stroke. METHODS: We screened patients who were admitted to the National Rehabilitation Center with the cerebellar stroke between October 2012 and November 2016. The patients with the cerebellar stroke who underwent neuropsychological testing for which the Seoul Neuropsychological Screening Battery (SNSB) or the SNSB-II were enrolled. The neuropsychological function capacities were compared in accordance with the stroke type (hemorrhagic vs. ischemic) and the location (right/left anterior, right/left posterior intermediate, right/left posterior lateral lobe, and vermis). Mean z-scores were computed to compare the patient performances with the population averages. RESULTS: Twenty-six patients (15 with ischemic stroke and 11 with hemorrhagic stroke) with a mean age of 54.8±16.6 years were assessed 8.8±9.2 months after the stroke. Differences in the neuropsychological functioning according to the stroke type were not observed. All of the numerical subtests of the stroke patients showed significantly poorer performances compared with the population averages (mean z-score < 0), and some of the subtests revealed abnormal performances in attention-, visuospatial function-, memory-, and frontal/executive function-related tasks (mean z-score <−1). The patients with the presence of a lesion in the right posterior intermediate lobe of the cerebellum showed a poorer performance in the subtests evaluating the executive function including the Korean-version Stroop Test (p=0.04), the Digit Symbol Coding Test (p=0.01), and the Korean-version Trail Making Test (p=0.02) compared with the patients without that lesion. CONCLUSION: The present study confirms that the cerebellar stroke affects the neuropsychological functioning which is associated with the anatomical site of stroke.

An Infantile Case of Sandhoff Disease Presenting With Swallowing Difficulty

Infants with Sandhoff disease typically appear normal until 3–6 months of age. As the disease progresses, they present with symptoms such as loss of motor skills, exaggerated startle response to loud noise, seizures, visual loss, and paralysis. We encountered a rare case of a 22-month-old girl with Sandhoff disease characterized by progressive motor weakness and dysphagia, who initially showed signs of aspiration at 20 months of age. The major problems related to dysphagia were oromotor dysfunction and abnormal feeding posture. Within 3 months of identification of difficulty in swallowing, the patient showed a significant decrease in food intake, with rapid deterioration of nutritional status. We report our case with a review of the literature.