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Dropped Head Syndrome Due to Neuromuscular Disorders: Clinical Neurology International 2019; volume 11:8198 Dropped head syndrome due inflammatory polyneuropathy (CIDP),11 to neuromuscular disorders: neuromuscular causes include myasthenia Correspondence: Ahmet Z. Burakgazi, gravis (MG),12-14 Lambert-Eaton myasthe- Neuroscience Section, Department of Clinical manifestation and nia syndrome (LEMS),15 muscular causes Medicine, Virginia Tech Carilion School of evaluation includes primary inflammatory such as Medicine, 3 Riverside Circle, Roanoke, VA polymyositis,16 scleromyositis,17,18 isolated 24016, USA. inflammatory axial myopathy,19 primary Tel.: +1.540-521-4592. Ahmet Z. Burakgazi, Perry K. E-mail: [email protected] Richardson, Mohammad Abu-Rub non-inflammatory such as nemaline myopa- 20-22 thy, mitochondrial myopathy, congeni- Key words: Dropped head syndrome, neuro- Virginia Tech Carilion School of 23 24 tal myopathy, FSHD, and isolated neck muscular disease. Medicine, Roanoke, VA, USA extensor myopathy (INEM).19 Contributions: the authors contributed equally. Conflict of interest: the authors declare no Abstract General approach: clinical mani- potential conflict of interest. festation and evaluation In this article, we discuss the clinical Funding: none. approach to patients with dropped head syn- DHS occurs as a result of weakness of drome and identify the various neuromus- posterior neck muscles. It usually disap- Received for publication: 11 June 2019. cular causes of dropped head syndrome pears with supine position. The common Accepted for publication: 18 June 2019. including muscle, neuromuscular junction, chief complaints are “chin on the chest” and This work is licensed under a Creative peripheral nerve and motor neuron etiolo- “difficulty maintaining a forward gaze”. It gies. We aim to increase awareness of Commons Attribution NonCommercial 4.0 may contribute to dysphagia and has cos- License (CC BY-NC 4.0). recognition the entity of dropped head syn- metic and social implications.1,12,19,25 drome and factors that may predict response Semispinalis cervicis and semispinalis capi- ©Copyright: the Author(s), 2019 to immunomodulating therapy in dropped tis are primarily responsible for neck exten- Licenseeonly PAGEPress, Italy head syndrome. sion. DHS is thought to be directly related Neurology International 2019; 11:8198 to weakness or laxity of the semispinalis doi:10.4081/ni.2019.8198 cervicis muscles.1,25 During the evaluation of dropped head,use Introduction providers first should rule out structural dis- tive weakness pattern (as in IBM), and The dropped head syndrome (DHS), eases of the cervical spine. To differentiate pelvic girdle weakness (as in most general- also known as the floppy head syndrome, NM causes from non-NM causes is very ized myopathies) should be assesses care- was first described as in 1986.1 It was ini- important. In NM causes, a significant fully.1,2,25,27 tially recognized in 12 patients with neuro- weakness of neck extensors is usually Following physical examination, a muscular (NM) disorders. This syndrome is observed. In non-NM causes, imbalance of provider should decide if further imaging defined by weakness of neck extensor mus- neck muscle tone leads to dystonic antero- tests are warranted. X-rays can be ordered cles against gravity with or without weak- collis. It is paramount important to identify to rule out kyphosis, fractures, and arthritis. 1,2,12,19,25 ness of neck flexor muscles. It can be an potential treatable causes. Magnetic resonance imaging (MRI) is supe- isolated clinic entity presenting with diffi- A deliberate and detailed history taking rior to Computed tomography (CT) to culty raising the head or it can be part of the is a key factor for an accurate diagnosis and imagine soft tissue includes tendons, liga- generalized muscular disease manifestation. important clues can be gathered from the ments, fascia, fibrous tissues, fat, synovial In the first report, definite diagnosis was history. Firstly, a provider should identify membranes, muscles, and nerves.28,29 established in 9 patients; myasthenia gravis pattern of involvement and characteristics Contrast enhancement is sensitive to detect in 4; motor neuron disease in 3; polymyosi- of weakness. These questions should be inflammation. MRI imaging demonstrates tis (PM), sclerodermatomyositis Non-commercial(SDM), asked if a patient has isolated neck extensor fatty infiltration, edema, and/or atrophy of and facioscapulohumeral dystrophy weakness or diffuse weakness; if a patient the neck musculature.28 Radiological imag- (FSHD) in 1.1 Then in 1992, Suarez and has fatigable weakness or diurnal variation; ing particularly MRI is useful for determin- Kelly described a non-inflammatory if there is any cranial and/or bulbar muscle ing biopsy sites in selective cases.28,29 myopathy affecting predominantly neck involvement; if there is any respiratory Electrodiagnostic (EDx) studies pro- extensors in four patients, aged 63 to 81 muscle weakness; and if there are any asso- vide valuable information in the diagno- years and they named the DHS as a new ciated symptoms such as autonomic symp- sis.30,31 The needle study should include clinical disorder, characterized by severe toms. Detailed family and developmental cervical and thoracic paraspinal, sternoclei- weakness of neck extensors with milder history should be taken as well as history of domastoid muscle, and trapezius muscles. weakness of shoulder girdle and proximal onabotulinumtoxin A injections.1,2,16,19,25,26 Facial and tongue muscles should be tested arm muscles. After detailed history, cautious and if any weakness is detected. DHS is relatively rare condition that complete physical examination should be Electromyographer should be careful not to may occur as a result of several neurologi- performed to collect more clues for an accu- overestimate paraspinal muscles finding cal, neuromuscular, muscular, and other rate diagnosis. In the physical exam, upper during the needle testing since normal causes. Neurological causes includes amy- and/or lower motor signs, muscle paraspinal motor unit action potentials have otrophic lateral sclerosis (ALS),2,3, wasting/atrophy (as in motor neuron dis- smaller amplitudes and a shorter duration, Parkinson disease4, multiple system atro- ease) , skin findings (as in dermatomyosi- but the recruitment is normal as opposed to phy,4,5 cervical dystonia,6 postpolio syn- tis), ocular and bulbar weakness (as in MG), reduced or early recruitment.30-32 drome,7 cervical myelopathy,8-10 chronic facial weakness (as in FSHD), highly selec- Muscle biopsy can be considered in [page 52] [Neurology International 2019; 11:8198] Review selected cases. Most of the biopsies are Another retrospective study,3 including The pathogenesis of DHS in ALS may taken from shoulder girdle muscles includ- 683 patients with ALS from India showed 9 be preferential involvement of paraspinal ing deltoid and trapezius muscles.30,33 of the patients (1.3%) had severe weakness anterior horn cells. Electromyography Biopsy from cervical paraspinal muscles of neck extensor muscles observed as an (EMG) test shows denervation with no are more valuable, but more challenging early feature, or developing during the later myopathic features. Muscle biopsy is usual- because small and larger muscles are inter- stages of the disease. The mean of affected ly consistent with neurogenic atrophy.2,12 mingled and it is difficult to know exactly patients was 53.3 (±10.3) (range 33 to 65). DHS may cause neck pain and discom- which muscles should be biopsied.30,32 Six of them developed head drop as an early fort in ALS patients. DHS can cause severe Muscle biopsy may provide valuable infor- feature (mean interval from onset of illness impairments in activities of daily living mation particularly in diagnosis of inflam- was 11.6 months) and three has late onset (ADL) such as eating, dressing so ALS matory muscle diseases such as size vari- (between three and eight years after onset).3 patients with DHS are more dependent in ability with fibrosis (Figure 1) and inflam- In their series, none of patients developed ADL.12,34 We advise ALS patients with mation with some necrotic fibers (Figure 2). respiratory difficulties but all had swallow- DHS to wear a neck collar, which improve Ultrasound guided biopsy may be helpful, ing difficulties and social embracement.3 their posture, swallowing, and neck pain. but not is not widely used. DHS in amyotrophic lateral sclerosis 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 only usually worse than neck extensor muscles in ALS patients. DHS in ALS patients has been reported in few case reports.2,3,34 A single-hospital-based retrospective study34 use 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. The initial symptoms of the patients were bulbar palsy in two patients and weakness of the upper limb in one patient.34 The onset to DHS was 14, 4 and 26 months after the onset of illness. Patients with prominent legs weakness did not Figure 1. The picture demonstrates size variability with fibrosis. develop DHS in their study.34 Non-commercial Figure 2. The pictures demonstrate inflammation with some necrotic fibers. [Neurology International 2019; 11:8198] [page 53] Review and mycophenolate mofetil. Only one had DHS in neuromuscular junction full remission and two showed some DHS in peripheral neuropathy disorders improvements. The pathological relationship between Chronic inflammatory demyelinat- Myasthenia gravis MG and axial myopathy is unclear. Most of ing polyneuropathy Drooped head is a well-recognized fea- the reported cases are in their late 70s or DHS is very rare presentation of CIDP. ture of MG even in the absence of ocular or early 80s. Mechanical stretch injury, loss of Two cases have been reported on DHS with bulbar signs. It can be associated with or tissue elasticity with aging, chronic use of CIDP.
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