Transcranial Doppler (TCD) was introduced in 1982 to assess intracranial arteries noninvasively, since when it has been widely used to assess and monitor cerebrovascular hemodynamics. The clinical applications of TCD are broadening to include other fields that require monitoring of the cerebral blood flow. TCD has fewer temporal and spatial restrictions than other methods, can be performed on less-compliant patients, and causes no harm to the body. However, its reliance on high levels of examiner skill and experience, as well as a lack of standardized scanning protocols are obstacles that still need to be overcome. In this report we review TCD examination techniques and interpret their findings for several conditions.

Citations

• Manual of Transcranial Doppler Ultrasonography
  Ho Tae JEONG, Soo Na JEON, Sol HAN
  Korean Journal of Clinical Laboratory Science.2024; 56(3): 277.     CrossRef
• The Pathophysiology of Syncope and the Role of Transcranial Doppler in its Diagnostic Evaluation
  Bang-Hoon Cho
  Journal of Neurosonology and Neuroimaging.2024; 16(2): 51.     CrossRef
• Success Rate of Transcranial Doppler Scanning of Cerebral Arteries at Different Transtemporal Windows in Healthy Elderly Individuals
  Mandy Yuen-Man Chan, Yan To Ling, Xiang-Yan Chen, Suk-Tak Chan, Kenneth K. Kwong, Yong-Ping Zheng
  Ultrasound in Medicine & Biology.2023; 49(2): 588.     CrossRef

Background
Magnetic resonance (MR) images are useful for diagnosing myopathy. The purpose of this study was to determine the usefulness of lower-limb MR images in Korean patients with distal myopathy.
Methods
We reviewed medical records in the myopathy database from January 2002 to October 2016. We selected 21 patients from 91 unrelated families with distal myopathy: four with GNE myopathy, 11 with dysferlinopathy, and six with ADSSL1 myopathy.
Results
Ten (48%) of the 21 patients were men. The ages of the participants at symptom onset and imaging were 19.2 ± 9.5 and 30.4 ± 9.0 years (mean ± standard deviation), respectively. Their grade on the modified Gardner-Medwin and Walton grade was 3.3 ± 1.7. The strength grade of the knee extensors was not correlated with the Mercuri scale for the quadriceps (r = –0.247, p = 0.115). However, the Medical Research Council grades of the knee flexors, ankle dorsiflexors, and ankle plantar flexors were significantly correlated with the Mercuri scale ratings of the knee flexors (r = –0.497, p = 0.001), tibialis anterior (r = –0.727, p < 0.001), and ankle plantar flexors (r = –0.620, p < 0.001), respectively. T1-weighted MR images showed characteristic fatty replacement patterns that were consistent with the causative genes. Unsupervised hierarchical clustering of the Mercuri scale showed that the main factors contributing to the dichotomy were the causative gene and the clinical severity.
Conclusions
This study is the first to reveal the usefulness of lower-limb MR images in the differential diagnosis of distal myopathy in Korea.

Background
The standardized autonomic function test has become widely available. However, there are no reference data for this test for the Korean population. This study explored reference data for sudomotor and cardiovagal function tests for the Korean population.
Methods
The sweat volume by quantitative sudomotor axon reflex test, heart-rate response to deep breathing (HRdb), expiration:inspiration (E:I) ratio, and Valsalva ratio (VR) were measured in 297 healthy Korean volunteers aged from 20 to 69 years. Multivariate regression analysis was performed to evaluate the effects of age, sex, and body mass index on these variables. The 2.5th, 5th, 10th, 90th, 95th, and 97.5th percentile values were obtained for each investigation.
Results
The sweat volume was higher in males than in females. The HRdb and E:I ratio were negatively correlated with age, and were higher in males than in females. The VR was negatively correlated with age, but it was not correlated with sex.
Conclusions
This study has provided data on the reference ranges for sudomotor and cardiovagal function tests in healthy Korean adults.

Citations

• Abnormal quantitative sudomotor axon reflex test results in patients with tinnitus as a possible indicator of small fiber neuropathy
  Hye Lim Lee, Hyun Ji Lyou, Jae-Jun Song, Chi Kyung Kim
  Frontiers in Neurology.2024;[Epub]     CrossRef
• Generalized anhidrosis with preganglionic sudomotor dysfunction in Fabry disease: a case report
  Eun Bin Cho, Seung Joo Kim, Tae-Won Yang, Heejeong Jeong, Changhyo Yoon, Seunguk Jung, Ki-Jong Park
  Clinical Autonomic Research.2021; 31(4): 585.     CrossRef
• Interpretation of Autonomic Function Test
  Kee Hong Park, Eun Hee Sohn
  Journal of the Korean Neurological Association.2021; 39(2): 61.     CrossRef

The patients with myotonic dystrophy (MD) show ocular motor abnormalities including strabismus, vergence deficits, and inaccurate or slow saccades. Two theories have been proposed to explain the oculomotor deficits in MD. The central theory attributes the defects of eye movements of MD to the involvement of the central nervous system while the muscular theory attributes to dystrophic changes of the extraocular muscles. A 58-year-old woman with MD showed selective slowing of horizontal saccades and reduced peak velocities for both horizontal canals in head impulse tests, while smooth-pursuit eye movements and vertical head impulse responses were normal. This case suggests that the extraocular muscles-as a final common pathway of the voluntary saccade and reflexive vestibular eye movements-may better explain the defective rapid eye movements observed in MD.

A 64-year-old man presented with facial diplegia occurring 2 weeks after scrub typhus diagnosis. The serum scrub typhus antibody titer was elevated to 1:5120. Brain magnetic resonance imaging revealed contrast-enhancement of the signal for both facial nerves. He was administered prednisolone. After two weeks, the symptoms improved, and after one month, he completely recovered from facial diplegia. This is the first case in the literature in which the patient exhibited facial diplegia, a delayed complication, in scrub typhus. Facial diplegia should be considered a type of cranial nerve palsy that may occur as a delayed complication of scrub typhus.

Transient global amnesia is a syndrome of temporary loss of short-term memory and is not accompanied by any other neurological deficit. Diffusion-weighted imaging is useful to improve the diagnostic accuracy of transient global amnesia. We report a 68-year-old woman with multiple lesions on diffusion-weighted imaging in the right corpus callosum and left hippocampus. To the best of our knowledge, this is the first case of a diffusion-weighted imaging lesion in the body portion of the corpus callosum.

Acute ophthalmoparesis that includes the oculomotor, trochlear, or abducens nerve may occur as an initial presentation of Miller Fisher syndrome (MFS). The symptoms of MFS or variant forms of Guillain-Barre syndrome are pathogenically related to anti-GQ1b antibodies. We report a case of a 36-year-old man with unilateral isolated abducens nerve palsy associated with anti-GM1 antibody. To the best of our knowledge, this is the first report of unilateral isolated abducens nerve palsy with positivity for anti-GM1 immunoglobulin M antibody.

Citations

• Ganglioside (GM1) Immunoglobulin G Antibody Chronic Bilateral Ophthalmoplegia
  Jesse Cheung, Saif Aldeen Alryalat, Osama Al Deyabat, Andrew G. Lee
  Journal of Neuro-Ophthalmology.2025; 45(1): e31.     CrossRef
• Isolated facial diplegia variant of Guillain–Barré syndrome with anti-GM1 IgG antibody
  Jin Ho Jung, Sukyoon Lee, Jung Hwa Seo, Jong Seok Bae, Kyong Jin Shin, Jong Kuk Kim, Byeol-A Yoon, Seong-il Oh
  Annals of Clinical Neurophysiology.2022; 24(1): 17.     CrossRef

Non-Hodgkin’s lymphoma (NHL) may initially present with atypical neurological manifestations, including paraneoplastic neurological syndromes. Herein, we report the case showing an initial manifestation of systemic NHL with paraneoplastic demyelination in the brain that initially mimicked the symptoms of stroke, seizure, and brain tumor. A high index of suspicion and timely diagnostic workup is required to prevent diagnostic delay and commence proper management of the condition. In this situation, a whole-body FDG PET/CT could be useful to screen for occult malignancy.

Myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) are distinct disorders. ALS affects motor neurons that control muscle movement, while MG controls communication between neurons and muscles, which occurs at neuromuscular junctions. However, on rare occasions, ALS develops after MG and vice versa. The coexistence of the two diseases represents a diagnostic challenge and requires thoughtful interpretation of clinical features. We present the case of a 53-year-old Korean male who developed ALS after MG, confirmed by clinical and electrophysiological follow-up.

Citations

• Two Cases of Sporadic Amyotrophic Lateral Sclerosis With Contrasting Clinical Phenotypes: Genetic Insights
  Andrey Frolov, Miguel A Guzman, Ghazala Hayat, John R Martin
  Cureus.2024;[Epub]     CrossRef