Dropped head syndrome in amyotrophic lateral sclerosis

Uemura, Masahiro; Kosaka, Takayuki; Shimohata, Takayoshi; Ishikawa, Masanori; Nishihira, Yasushi; Toyoshima, Yasuko; Yanagawa, Kaori; Kawachi, Izumi; Takahashi, Hitoshi; Nishizawa, Masatoyo

doi:10.3109/17482968.2012.727424

Cited by 15 publications

(12 citation statements)

References 11 publications

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“…Neck extensor muscle weakness with head drop as an early symptom has been reported in a few patients with ALS 42 43. However, Katz et al 44 wrote that neck flexor weakness is typically observed.…”

Section: Discussionmentioning

confidence: 99%

Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients

Nakamura

Atsuta

Watanabe

et al. 2013

J Neurol Neurosurg Psychiatry

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Section: Discussionmentioning

confidence: 99%

Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients

Nakamura

Atsuta

Watanabe

et al. 2013

J Neurol Neurosurg Psychiatry

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“…In spite of detailed examination, the direct cause of death was not verified, but the medial group of the cervical cord contains neurons of respiratory function and a few references of respiratory failure in patients with DHS have been described, and this condition is rare accounting for 1–3%, so further examination was required for this possibility.…”

Section: Discussionmentioning

confidence: 99%

“…In ALS, neck and trunk muscle weakness is observed as the first symptom in 2% of patients, and neck flexion is typically affected . The neck extensor muscle weakness with head drop has been reported in a few patients, and there are only two case series in India and Niigata . Furthermore, the neuropathological findings of this condition have been rarely described.…”

Section: Introductionmentioning

confidence: 99%

Autopsy findings in the early stage of amyotrophic lateral sclerosis with “dropped head” syndrome

et al. 2019

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Dropped head syndrome (DHS) has been rarely observed in amyotrophic lateral sclerosis (ALS), and the neuropathological findings of this condition have almost never been described. The identification of transactivation response DNA‐binding protein 43 kDa (TDP‐43), which binds to RNA/DNA has provided a new method for studying ALS and frontotemporal lobar degeneration (FTLD). Post‐mortem examination of an adult sudden death case of a 71‐year‐old patient who complained of DHS exhibited severe loss of anterior motor neurons in the cervical cord (C4‐6). Loss of nerve fibers of the anterior roots was striking compared with posterior roots, together with marked neurogenic atrophy of posterior muscles semispinalis cervicis. Bunina bodies were found in large neurons of Betz giant cells, but not in the motor neurons of spinal cords, or neurons of bulbar regions. Phosphorylated TDP‐43 (p‐TDP‐43)‐positive structures were detected in the residual neurons of the cervical, thoracic and lumber cords, hypoglossal nucleus, cerebellar dentate nucleus and parahippocampal cortex, together with ubiquitin‐positive inclusions. Phosphorylated Tau positive structures in neuronal cytoplasm were found in the amygdala, entorhinal cortex and parahippocampal cortex, some of which co‐expressed p‐TDP‐43. The medial zone of cervical cords may be the first onset site, and that is the cause of head drop in the early stage of ALS. In spite of detailed examination, the direct cause of sudden death was not verified. This autopsy report revealed the relation of DHS which is a rare clinical manifestation of ALS, and neuropathological findings.

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“…Dropped head is a rare manifestation of ALS occurring around 1-3% of patients. 34 Dropped head is usually an early feature of ALS but it is usually not the presenting symptom. Neck flexor muscles weakness is usually worse than neck extensor muscles in ALS patients.…”

Section: Dhs In Amyotrophic Lateral Sclerosismentioning

confidence: 99%

“…DHS in ALS patients has been reported in few case reports. 2,3,34 A single-hospital-based retrospective study 34 from Japan including 105 sporadic ALS patients showed the incidence rate of DHS was 2.9% (3/105). The ages were 56, 63, and 53 years.…”

Section: Dhs In Amyotrophic Lateral Sclerosismentioning

confidence: 99%

Dropped head syndrome due to neuromuscular disease: clinical manifestation and evaluation

2019

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In this article, we discuss the clinical approach to patients with dropped head syndrome and identify the various neuromuscular causes of dropped head syndrome, including muscle, neuromuscular junction, peripheral nerve, and motor neuron etiologies. We aim to increase awareness and recognition of the entity of dropped head syndrome and factors that may predict responses to immunomodulating therapy.

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Dropped head syndrome in amyotrophic lateral sclerosis

Cited by 15 publications

References 11 publications

Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients

Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients

Autopsy findings in the early stage of amyotrophic lateral sclerosis with “dropped head” syndrome

Dropped head syndrome due to neuromuscular disease: clinical manifestation and evaluation

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