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.

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.

Atrofia muscular distal bilateral dos membros superiores (Síndrome de O'Sullivan-McLeod): relato de caso / Bilateral distal muscular atrophy of upper limbs(O’Sullivan-McLeod syndrome): Case report

As amiotrofias espinhais progressivas (AEP) são um grupo de desordens geneticamente determinadas marcadas pela depleção dos neurônios da ponta anterior da medula espinhal e, frequentemente, núcleos de nervos cranianos (bulbares). A forma mais comum de AEP usualmente compromete os músculos proximais dos membros. Entretanto, existe uma forma incomum, usualmente esporádica, que envolve somente a região distal braquial. A proposta do presente relato é apresentar os achados clínicos e eletrofisiológicos de um paciente com AEP crônica e com comprometimento dos músculos do terço distal dos membros superiores. A eletroneumiografia revelou anormalidades neurogênicas e potenciais de desnervação com velocidade de condução sensitiva e motora normais. Descrevemos algumas teorias acerca da fisiopatologia. O reconhecimento dessa forma infrequente é muito importante para uma ótima abordagem terapêutica nesses pacientes.

The spinal muscular atrophies (SMA) are a group of genetically determined disorders in which the primary defect is a loss of the anterior horn neurons of the spinal cord and, commonly, of nuclei of cranial nerves (medulla). A more common chronic form of SMA usually affects proximal limb muscles. However, there is an uncommon form, frequently sporadic, involving only the distal part of upper limbs. The purpose of the present report is to describe the clinical and electrophysiological features of a patient with chronic SMA affecting the muscles of the distal third of the upper limbs. Electroneuromyography revealed neurogenic anomalies and denervation potentials with normal motor and sensory nerve conduction velocities. We describe some theories concerning its pathophysiology. The recognition of this infrequent form is very important for an optimized therapeutical approach of this kind of patients.

Upper limb proximal form of benign monomelic amyotrophy: on purpose of 2 cases / Amiotrofia monomélica benigna forma proximal de membro superior: a propósito de 2 casos

Benign monomelic amyotrophy (BMA) is a rare form of motor neuron disease of unknown cause and it?s characterized by weakness and muscular atrophy restricted to an upper or lower limb, usually in the distal part, absence of upper motor neuron signs and self limited course. In this report we describe the clinical and electromyographic features of two young men with BMA in the proximal portion of the right upper limb, a location of BMA rarely described in the literature.

Amiotrofia Monomélica Benigna (AMB) é uma entidade rara do neurônio motor, de causa desconhecida, caracterizada por fraqueza muscular e amiotrofia restritas a 1 membro superior ou inferior, frequentemente em terço distal, sem comprometimento da via piramidal e cursoautolimitado. Descrevemos os achados clínicos e eletroneuromiográficos de 2 pacientes jovens com AMB na porção proximal do membro superior, localização raramente descrita na literatura.

Spinal anaesthesia in a patient with Kennedy's disease: A case report / 대한마취과학회지

Kennedy's disease is an adult onset form of motor neuron disease characterized by progressive proximal and bulbar muscle weakness. The authors report the anaesthetic management of a 43-year-old man with Kennedy's disease who underwent elective orthopaedic surgery under spinal anaesthesia. The anaesthetic implications of this X-linked lower motor neuron disorder are discussed, and guidelines for safe anaesthetic management are suggested.

General anesthesia for laparoscopic cholecystectomy in a patient with Kennedy's disease: A case report / 대한마취과학회지

Kennedy's disease is a rare lower motor neuron disease affecting the limbs and bulbar musculature. Regional anesthesia is generally the recommended anesthetic technique in patients with Kennedy's disease because of bulbar involvement and airway clearing disturbance. We administered general anesthesia in a patient with Kennedy's disease who was undergoing a laparoscopic cholecystectomy. We closely monitored the degree of neuromuscular blockade throughout the operation and injected atracurium on demand. There was no exacerbation of neurologic signs or symptoms postoperatively. Therefore, we report the successful administration of general anesthesia for laparoscopic cholecystectomy in a patient with Kennedy's disease.

A Case of Lower Motor Neuron Syndrome Which May be Paraneoplastic

We present a 55-year-old man who has a six-month history of progressive weakness of all limbs. Findings from neurologic examination were notable for a diffuse muscular weakness and atrophy in all limbs. Laboratory findings for collagen vascular disease, monoclonal gammopathy, and infections were normal. Electrophysiologic studies supported lower motor neuron (LMN) syndrome. CT scan and needle biopsy disclosed small cell lung cancer. This case suggests that in some elderly patients with LMN syndrome a careful search for an underlying cancer is warranted.

Effect of Duration of Lower Motor Neuron Injury on Mivacurium-induced Muscluar Relaxation in Rabbits / 대한마취과학회지

BACKGROUND: The purpose of this study was to investigate whether the effects of mivacurium on muscular relaxation were similar by the duration of more than 2 weeks after the injury of lower motor neurons in rabbits. METHODS: The animals were divided into five groups. The control group was without lower motor neuron injury. In the experimental groups, the lower motor neuron injury was made by denervating with 75 - 80% lesion on the common peroneal nerve to the right anterior tibialis muscle. The experimental groups were subdivided as 1, 2, 3 and 4 week groups (referred to ad the 1 wk, 2, 3 and 4 wks group) according to the durations of the denervation of common peroneal nerve, respectively. The dose-response relationship of mivacurium on the muscle twitches induced by TOF (train of four) stimulation (supramaximal stimulus of 0.2 ms duration, square-wave pulses, 2 Hz rate, repeated every 10 seconds) was studied by calculating ED50 and ED95 in the anterior tibialis muscles and compared between all groups. After recording the muscle twitches, microscopic findings were observed. RESULTS: The effective dose for 95% twitch depression (ED95) of mivacurium at 1week after denervation was significantly higher than that of the control group (P <0.05), but the ED95 of 2, 3 and 4wks groups were not significantly different from that of the control group. However, the ED95 of 3 and 4wks group were inclined to be lower than that of the control and significantly lower than 1wk group (P < 0.05). There was no significant difference in the effective dose for 50% twitch depression (ED50) of mivacurium in all groups. The size of the anterior tibialis muscle was significantly decreased at 4weeks after the lower motor neuron injury (P <0.05), but the number of its sarcoplasmic nuclei was increased, according to the duration after the denervation. CONCLUSIONS: Our results therefore suggest that neuromuscular response of denervated anterior tibial muscle was resistant to intravenous mivacurium in early periods of 1 or 2 weeks but sensitive 4 weeks after the lower motor neuron injury.

The Effect of Mivacurium on Onset and Recovery According to the Durations of Lower Motor Neuron Injury / 대한마취과학회지

BACKGROUND: The purpose of this study was to investigate whether the effects of mivacurium on onset and recovery were affected by the duration of more than 2 weeks after injury of the lower motor neuron in rabbits. METHODS: The animals were divided into five groups. The control group was without lower motor neuron injury. In the experimental groups, the lower motor neuron injury was made by denervating with a 75 - 80% lesion on the common peroneal nerve to the right anterior tibialis muscle. The experimental groups were subdivided as 1, 2, 3 and 4 week groups (named group 1 wk, 2, 3 and 4 wks) according to the duration of the denervation of the common peroneal nerve. The response relationship of mivacurium on the muscle twitches induced by TOF (train of four) stimulation (supramaximal stimulus of 0.2 ms duration, square-wave pulses, 2 Hz rate and 10 mA, repeated every 10 seconds) was studied in the anterior tibialis muscles and compared between all groups. Neuromuscular responses (onset, recovery time to T1(1), T1(25), T1(75), T1(95) and recovery index) of muscle twitches to intravenous mivacurium (0.18 mg/kg) were studied. After recording the muscle twitches, macroscopic findings were observed. RESULTS: The recovery time, T1(1) of group 4 wks was significantly longer than those of group 1, 2 and 3 wks (P < 0.05), but not different from the control group. The recovery time, T1(25), T1(75) and T1(95) of group 4 wks was significantly longer than those of all other groups (P < 0.05), but the onset times of all groups were not significantly different. The recovery index of group 4 wks was significantly higher than that of the control group (P < 0.05), but those of groups 1, 2 and 3 wks were not significantly different from that of the control group. The mass of the anterior tibialis muscle was significantly decreased at 4 weeks after the lower motor neuron injury (P < 0.05). CONCLUSIONS: Our results therefore suggest that the neuromuscular response to intravenous mivacurium on recovery in rabbits becomes prolonged according to the durations of the denervation and represents sensitivity at 4 weeks after the lower motor neuron injury.

The Urodynamic Findings of Neuropathic Bladder with Lower Motor Neuron Lesion / 대한비뇨기과학회지

No abstract available.