Assessment of the upper motor neuron degeneration by detailed motor homunculus cortex thickness in amyotrophic lateral sclerosis / 西安交通大学学报(医学版)

【Objective】 The involvement of upper motor neuron (UMN) degeneration is crucial to the diagnosis of amyotrophic lateral sclerosis (ALS). This study aimed to determine objective and sensitive UMN degeneration markers for an accurate and early diagnosis. 【Methods】 A total of 108 ALS patients and 90 age- and gender-matched control subjects were recruited from ALS Clinic of The First Affiliated Hospital of Xi’an Jiaotong University. The motor homunculus cortex thickness data in MRI were collected from all the participants. The clinical characteristics and UMN clinical examination of bulbar, cervical, thoracic and lumbosacral regions were collected from the ALS patients. 【Results】 Cortical thickness was significantly thinner in the ALS group than in the control group in bilateral head-face-bulbar and upper-limb areas (P<0.05). The cortical thickness of the global UMN positive group was significantly thinner than that of control groups in bilateral head-face-bulbar and upper-limb areas (P<0.05). The cortical thickness of the UMN positive group in the corresponding region was significantly thinner than that of control groups in bilateral head-face-bulbar and upper-limb areas (P<0.05). 【Conclusion】 The thinning of the motor homunculus cortex can be used as an objective marker of UMN involvement in ALS patients in clinical practice.

Neuroimaging advances in patients with amyotrophic lateral sclerosis / 西安交通大学学报(医学版)

Amyotrophic lateral sclerosis (ALS) is a multi-system neurodegenerative disease characterized with degeneration of both motor and non-motor areas. Complicated clinical manifestations and lack of objective biomarkers for upper motor neuron deficits challenged the early diagnosis of ALS. Meanwhile, heterogeneous non-motor symptoms and conflicted treatment effects exacerbated the management and therapy of the disease. The multiparametric functional MRI has the potential to address all the needs for diagnosis, management, and disease modified therapy in ALS. The present paper summarizes the research progress in both motor and non-motor impairment in ALS, as well as their potential value in visualizing disease stages and drug effect evaluation. Focusing on the heterogeneity of the disease and combining with brain and spinal cord imaging may provide direct evidence for disease diagnosis and treatment and be the priority in the future for ALS.

Mills hemiparetic or hemiplegic variant of amyotrophic lateral sclerosis

Mills hemiplegic variant of Amyotrophic lateral sclerosis (ALS) is a gradually progressive, spastic ascending or descending hemiparesis or hemiplegia without any sensory involvement. Authors presented a 47 years old female with history of gradually progressive left sided wasting of muscles including the tongue, left hemiparesis along with dysarthria and fasciculation’s of tongue and left sided muscles with left sided cortico-spinal tract signs of 2 years duration. There were no sensory as well as bowel bladder involvement. Her cognition was intact. Relevant blood and CSF examinations were within normal limit. MRI Brain and whole spine were unremarkable. Nerve conduction study was essentially normal. Electromyography(EMG) showed chronic denervation potentials which is in accordance to Revised El Escorial criteria, 2015 for the diagnosis of this extremely uncommon entity- Mills hemiplegic variant of ALS. The major challenge in diagnosis of this disease entity is to exclude other diseases/disorders that may mimic its symptomatology.

Differential diagnosis of motor neuron disease / 中华神经科杂志

Motor neuron disease (MND) is a type of chronic progressive neurodegenerative disease involving upper and/or lower motor neurons, including amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, and spinal muscular atrophy. Different types of motor neuron diseases have distinct characteristics. Therefore, the diagnosis mainly depends on clinical manifestations, physical examination and electrophysiological examination, and is made by exclusion of other diseases. Atypical cases are sometimes difficult for clinical physicians to distinguish from other diseases involving upper and/or lower motor neurons. This article is going to introduce various types of MND and their differential diagnosis according to site of lesion of motor neuron disease.

Differential diagnosis of motor neuron disease / 中华神经科杂志

Motor neuron disease (MND) is a type of chronic progressive neurodegenerative disease involving upper and/or lower motor neurons, including amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, and spinal muscular atrophy. Different types of motor neuron diseases have distinct characteristics. Therefore, the diagnosis mainly depends on clinical manifestations, physical examination and electrophysiological examination, and is made by exclusion of other diseases. Atypical cases are sometimes difficult for clinical physicians to distinguish from other diseases involving upper and/or lower motor neurons. This article is going to introduce various types of MND and their differential diagnosis according to site of lesion of motor neuron disease.

Assessment of the function of cervical spinal upper motor neuron in patients with frail arm syndrome / 中华神经科杂志

Objective To investigate cervical spinal upper motor neuron (UMN) dysfunction in patients with frail arm syndrome (FAS) by physical examination,triple stimulation technique (TST) and pectoralis tendon reflex tests.Methods Sixty-seven FAS patients,coming from Peking University Third Hospital from June 2013 to June 2016,underwent physical examination and routine electrophysiological tests.The pyramid signs,the results of pectoralis tendon reflex and TST were collected to estimate the function of cervical spinal UMN.Results On the time of diagnosis,weakness of bilateral proximal upper limbs was found in 61 patients,while weakness of unilateral proximal upper limb was found in six patients.There were 25 patients with tendon hyperreflexia,20 patients with tendon hyporeflexia and 22 patients with tendon areflexia.All the patients were pectoral muscle tendon hyperreflexia except one.UMN score of cervical region was 2.0 ± 0.5.Lower motor neuron score of cervical region was 2.0 ± O.2.The amplitude ratio of TSTtest/TSTcontrol was 78.31％ ± 6.52％.The latency and amplitude of quantitative detection of pectoralis tendon reflex was (7.80 ± 1.22) ms and (1.23 ± 0.14) mV,respectively.In the follow-up study,the tendon reflexes and the UMN score declined,the amplitude ratio of TSTtest/TSTcontrol decreased,while the lower motor neuron score increased and the latency of quantitative detection of pectoralis tendon reflex remained almost unchanged.Conclusion The results showed that there was cervical spinal UMN dysfunction in patients with FAS,and the pyramid signs were often concealed by muscle atrophy with progression of the disease.

Definition of Spasticity / The Japanese Journal of Rehabilitation Medicine

<p>Although the term “spasticity” refers to one of the most important concepts in physical medicine and rehabilitation, it is inconsistently defined, particularly in the Japanese literature.<br/>Here, I have presented several definitions widely used throughout Japan and discussed differences among those proposed by Lance, Shimamura, and Tanaka. The use of the phrase “phasic and tonic stretch reflexes” and the meaning of “rigidity” were indicated to cause confusion regarding the definition.<br/>Exaggerated tendon jerks without a reflex response during a continuous muscle stretch should not be included under spasticity. However, if tendon jerks are exaggerated, the increased stretch reflexes without muscle (or joint) movement should be termed “spasticity” rather than “rigidity.” Although some researchers use the term “rigidospasticity” to express severe spasticity, it differs from the original meaning.<br/>According to the discussion, I agree with the definition proposed by Lance：“Spasticity is a motor disorder characterized by a velocity-dependent increase in tonic stretch reflexes (muscle tone) with exaggerated tendon jerks, resulting from hyperexcitability of the stretch reflex, as one component of the upper motor neuron syndrome.” However, it is a mysterious phenomenon that depends not only on velocity but also on muscle length. Further review of its definition is required.</p>

Disease onset regions and spreading patterns in sporadic amyotrophic lateral sclerosis and related influencing factors / 西安交通大学学报(医学版)

Objective To evaluate the disease onset regions and spreading patterns in sporadic amyotrophic lateral sclerosis (ALS)patients and related influencing factors.Methods We performed a prospective analysis of 1 58 ALS patients.The disease-onset was confirmed according to the patients’self-reports,neurological examination results and electromyogram study.We followed up 1 5 1 patients with the second or other affected body regions during the disease progression.Data were analyzed according to the different groups of onset regions.Results 1.In 94.3% (149/1 58)of the patients,the early motor manifestations were focally in the bulbar,upper or lower limbs.2.The region of onset was associated with signs of lower motor neuron (LMN)and upper motor neuron (UMN)involvement (P = 0.000 ).The LMN involvement was more distinctive in patients with bulbar onset (65.4%,1 7/26 )group.Patients with cervical onset more frequently showed pure LMN (47.9%,45/94 )or concomitant UMN (52.1%,49/94)signs in the affected limbs.The highest proportion of UMN and LMN signs in the affected lower limb was found in the lumbar onset (83.8%,31/37 )group.3.Spreading patterns:Rostral to caudal spreading pattern was more frequent in bulbar onset patients.For patients with limb onset,there were significant differences between spreading patterns and disease-onset regions (P =0.04).Circular (31.5%,28/89),horizontal (30.3%,31/89)and vertical (21.3%,1 9/89)spreading patterns were more frequent in cervical onset patients whereas circular (47.2%,1 7/36)spreading patterns were more frequent in lumbar onset patients.4.There was a strong association between the rate of progression and age of disease onset (P =0.01 1).Patients aged over 60 had a faster progression.Conclusion ALS is a focal process at motor axis along the spinal cord and cerebral cortex.Different disease-onset can cause different distribution of UMN and LMN signs.Therefore,special attention should be paid to the signs of disease-onset clinically.ALS does start focally and spreads to adjacent regions.Elder patients have a faster disease progression.

Cerebral Infarction Presenting with Unilateral Isolated Foot Drop

Weakness of the dorsiflexor muscles of the ankle or toe, referred to as foot drop, is a relatively common presentation. In most cases, foot drop is caused by a lower motor neuron disease such as peroneal peripheral neuropathy, L4-5 radiculopathic sciatic neuropathy, or polyneuropathy. Although upper motor neuron lesions can present as foot drop, the incidence is very rare. Here, we report an extremely rare case in which foot drop was the only presenting symptom of cerebral infarction.

Two Cases of Autoaugmentation Cystoplasty in Spastic Neurogenic Bladder with Vesicoureteral Reflux / 대한비뇨기과학회지

Urinary incontinence is an annoying complaint in patients with imbalanced upper motor neuron lesion, and prevention of vesicoureteral reflux and urinary tract infection is important for the preservation of renal function. We present two cases of autoaugmentation cystoplasty and extravesical ureteral reimplantation (Lich-Gregoir Technique) in paraplegics who have upper motor neuron lesion with vesicoureteral reflux.