A 76-year-old woman with a 2-year history of Parkinson\'s disease presented with dropped head, which had developed rapidly after she had been prescribed donepezil hydrochloride (DNP) at 3 ‍mg/day. After one month of medication, the extent of the head drop reached 90°. Examination revealed hypertrophy of the left sternocleidomastoid muscle, but no weakness of the extensor muscles in the cervical region. Surface electromyography demonstrated co-‍contraction of the sternocleidomastoid and splenius capitus muscles during head flexion and extension. DNP was withdrawn, resulting in immediate amelioration of the head drop, and complete resolution was achieved after two months. Although head drop is often seen in patients with Parkinson\'s disease, few previous reports have documented DNP as a causative factor. If patients with Parkinson\'s disease develop head drop, it is important to investigate any history of DNP medication.

UNASSIGNED: Patients with Parkinson\'s disease and related disorders (PDRD) may exhibit dropped head syndrome (DHS), which does not yet have an effective treatment.
UNASSIGNED: To evaluate the effect of combining lidocaine injection into the bilateral scalene muscles and neck corset wearing on dropped head syndrome.
UNASSIGNED: We performed needle electromyography assessments of the scalene, sternocleidomastoid (SCM), levator scapulae, splenius capitis, and trapezius muscles. Patients received 2.5-5 ml injections of 1% lidocaine into both sides of the scalene muscles for 4/5 consecutive days and were instructed to wear a neck corset. We measured the neck flexion angle, which formed between the horizontal line and the straight line passing through the ear canal and orbital fossa, before (baseline) and after (Day 8 and Day 90) the intervention.
UNASSIGNED: Seven males and eight females (mean age, 68.9 years; range 56 to 85 years) who had PDRD with dropped head syndrome were enrolled in this study. Needle electromyography examination revealed abnormal discharge of the scalene muscles in all patients when the neck position was corrected; however, some patients did not show abnormal discharge of the SCM muscle. At Day 8, we observed an improvement of the neck flexion angle in 13 of the 15 patients, from an average of 27.7° ± 13.9° to 11.7 ± 14.6°. At Day 90, the average neck flexion angle was 15.3° ± 17.2°.
UNASSIGNED: Combining lidocaine injection into the scalene muscles and neck corset wearing is an effective treatment regimen for DHS in patients with PDRD.

A 64-year-old female developed Parkinson\'s disease at the age of 52 years. She experienced muscle weakness in the upper right extremities and dropped head at 62 and 63 years, respectively; both symptoms were considered to be associated with Parkinson\'s disease (PD). The dosage of L-DOPA was increased from 200 mg/day to 900 mg/day; however, her neurological symptoms did not improve. Eventually, she was diagnosed with amyotrophic lateral sclerosis (ALS) at 64 years. She was placed under palliative care, and died of respiratory failure and malnutrition. Neuropathologic findings were consistent with the coexistence of PD and ALS. In fact, there were α-synuclein immunoreactive Lewy bodies (Braak stage 4) as well as TDP-43 immunoreactive deposits in the motor nuclei at the level of brainstem and spinal cord. Therefore, coexisting pathologies must be taken into account in a patient showing multi-system symptoms.

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Head drop, or having a dropped head, is an uncommon condition in which patients present with a disabling inability to lift their head. It may arise in many neurological conditions that can be divided into those with neuromuscular weakness of neck extensors and those with increased tone of neck flexors. The most common neuromuscular causes include myasthenia gravis, motor neurone disease and myositis, while neck dystonia secondary to movement disorders can cause an increased tone. Investigations should include blood tests, imaging, muscle biopsy and neurophysiological studies. Here, we present a man with dropped head and discuss the investigation and management of this condition.

Head-drop is often encountered in myasthenia gravis (MG) patients, but its frequency and clinical course have not been studied systematically.
In a retrospective study of a cohort of MG patients seen over a period of 11 years in a tertiary medical center, we assessed the clinical characteristics of patients who had head-drop.
Of 146 generalized MG patients, 15 had head-drop during the course of their disease. Head-drop patients had older age of onset than those who did not have head-drop (mean age of onset 59.1 vs. 42.3 years) and were predominantly men. Head-drop was present in 23% of patients > 60 versus 6% of those < 60 years, and it improved in 9 of 11 patients with treatment directed to generalized MG.
Head-drop is a common, treatment-responsive manifestation of late-onset MG. Muscle Nerve 56: 441-444, 2017.

BACKGROUND: Dropped head syndrome is an easily recognizable clinical presentation of Lamin A/C-related congenital muscular dystrophy. Patients usually present in the first year of life with profound neck muscle weakness, dropped head, and elevated serum creatine kinase.
METHODS: Two patients exhibited head drop during infancy although they were able to sit independently. Later they developed progressive axial and limb-girdle weakness. Creatine kinase levels were elevated and muscle biopsies of both patients showed severe dystrophic changes. The distinctive clinical hallmark of the dropped head led us to the diagnosis of Lamin A/C-related congenital muscular dystrophy, with a pathogenic de novo mutation p.Glu31del in the head domain of the Lamin A/C gene in both patients. Remarkably, one patient also had a central involvement with white matter changes on brain magnetic resonance imaging.
CONCLUSIONS: Lamin A/C-related dropped-head syndrome is a rapidly progressive congenital muscular dystrophy and may lead to loss of ambulation, respiratory insufficiency, and cardiac complications. Thus, the genetic diagnosis of dropped-head syndrome as L-CMD and the implicated clinical care protocols are of vital importance for these patients. This disease may be underdiagnosed, as only a few genetically confirmed cases have been reported.

BACKGROUND: Congenital myasthenic syndromes are rare. Mutations in MUSK were first described in 2004. Thirteen patients have been reported to date, mostly with a relatively mild course. The molecular diagnosis has implications for choice of treatment and genetic counseling.
METHODS: Clinical course and electrophysiological, pathological, and genetic findings were assessed.
RESULTS: We describe the case of a boy with prenatal onset and severe respiratory symptoms with a persisting need for ventilation. The patient had severe bulbar symptoms, marked axial weakness causing a \"dropped head,\" and some facial and proximal weakness. Ophthalmoparesis developed during the first year of life. Salbutamol led to improvement, 3,4-diaminopyridine had a modest effect, but pyridostigmine produced deterioration. Two novel mutations in MUSK were found by whole exome sequencing.
CONCLUSIONS: We expand the phenotype of congenital myasthenic syndromes with MUSK mutations, describing a more severe clinical course with prenatal onset. Predominant bulbar and respiratory weakness with facial and axial weakness and ophthalmoparesis are diagnostic clues.

We report two patients with myasthenia gravis (MG) who showed dropped head as an early myasthenic manifestation. They had elevated anti-acetylcholine receptor antibody and showed improvement of the symptoms after intravenous injection of edorophonium chloride. One patient had thymoma and developed myasthenic crisis two weeks after thymectomy. The patient recovered from the crisis after a combination of immunoadsorption plasmapheresis (IAPP) and initiation of steroid and tacrolimus. The other patient without thymoma initiated treatment with steroid, tacrolimus and IAPP and showed complete recovery one month later. Dropped head in MG can recover well with immunosuppression therapy using steroid, and IAPP is helpful in getting a rapid improvement of dropped head as well as recovery from myasthenic crisis. When we consider treatment for MG with dropped head, we should take into account that MG of this type can develop myasthenic crisis and use the same treatment strategy as that for generalized MG.