The dropped head syndrome with chronic inflammatory demyelinating polyneuropathy

The dropped head syndrome occurs in a variety of neuromuscular disorders. We present a woman with chronic inflammatory demyelinating polyneuropathy who developed this syndrome, likely reflecting severe demyelination of nerves to cervical paraspinal muscles. © 1994 John Wiley & Sons, Inc.     

Primary amyloidosis presenting as "dropped head syndrome"

A 77-year-old man, with a history of lymphoma, presented with isolated neck extensor weakness and a 2-year history of bilateral carpal tunnel syndrome (CTS). Needle electromyography showed myopathic changes, and biopsy of cervical paraspinal muscles showed amyloid deposition in blood vessels. Amyloidosis should be considered in the differential diagnosis of dropped head syndrome.     

Late appearance of dropped head syndrome after radiotherapy for Hodgkin's disease

We present three cases of dropped head syndrome that occurred as a complication of mantle field (i.e., lymph nodes of the neck, axillae, and mediastinum) or whole-body radiation therapy for Hodgkin's disease. These cases are characterized by a late onset (2-27 years after radiation treatment), fibrosis, and contraction of the anterior cervical muscles, and atrophy of the posterior neck and shoulder girdle. This report adds to the increasing literature about the late neurological complications of radiation therapy and describes a previously unrecognized cause of dropped head syndrome.     

Response of the dropped head/bent spine syndrome to treatment with intravenous immunoglobulin

Weakness of neck extension causing a dropped head may result from many neuromuscular disorders. One etiology is isolated neck extensor myopathy. A similar focal myopathy of the lower axial muscles may cause the bent spine syndrome, which manifests as flexion of the trunk and inability to stand upright. Combination of both dropped head and bent spine myopathies is uncommon. Inflammation is usually not pronounced in these conditions and response to immunosuppressive treatment is rare. We present an 81-year-old man who developed progressive weakness of neck and trunk extension over several months, with a prominent inflammatory process in the thoracic paraspinal muscles, which responded dramatically to treatment with intravenous immunoglobulin (IVIg). This case, together with other rare reports, suggests that the presence of inflammation in the biopsy of an affected muscle may predict treatment response.     

Licorice-induced hypokalemia as a treatable cause of dropped head syndrome

Isolated weakness of the extensor muscles of the neck is a relatively rare condition, known as “dropped head syndrome” (DHS). This paper reports the case of a patient with DHS whose symptoms resolved rapidly on supplementation with potassium and discontinuation of licorice consumption. To the best of our knowledge, there are no reports in the literature of DHS due to licorice-induced hypokalemia.     

“Dropped head syndrome” caused by Lambert–Eaton myasthenic syndrome

A 67‐year‐old man was admitted with a 2‐year history of dropped head. Neurological examination revealed ptosis, dysarthria, neck weakness, hyporeflexia of all limbs, and autonomic failure. Electrophysiologic study showed a 400% increment response to high‐rate repetitive nerve stimulation. Serum anti‐P/Q‐voltage‐gated calcium channel antibody was positive, confirming the diagnosis of Lambert–Eaton myasthenic syndrome (LEMS). His symptoms and electrophysiological abnormalities improved with oral prednisolone following plasmapheresis. This is the first report of LEMS as a cause of dropped head syndrome. Muscle Nerve 40: 134–136, 2009     

Two children with "dropped head" syndrome due to lamin A/C mutations

LMNA-related congenital muscular dystrophy (L-CMD) is a recently described disorder characterized by infantile-onset myopathy due to mutations in the lamin A/C (LMNA) gene. We report the genetic and clinical characteristics of two unrelated L-CMD patients. Patient 1 harbored a novel, L35P mutation and patient 2 a previously reported R249W mutation. The striking phenotype associated with L-CMD is important to recognize, as molecular diagnostic testing can spare patients unnecessary procedures and prompt the physician to monitor for associated cardiac arrhythmias.     

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

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.     

The application of control limits analysis to the myometric assessment of motor performance in neuropathy patients.

Measurement of muscle strength by myometry is used to monitor the natural course and treatment response of motor system diseases, both in individual patients and clinical trials. However, the practical usefulness of myometric data is reliant upon a statistical method for analysing serial strength measurements which distinguishes disease-related changes from random fluctuations in patient performance and operator/device dependent measurement errors. In this study we have applied control limits analysis to this problem. Using hand-held dynamometry, sets of baseline strength measurements were collected on two separate occasions during a period of clinical stability from up to four muscle groups in 22 patients with peripheral neuropathies. From these sets of data, 76 control limits were calculated and then used to describe the inter-measurement variation in muscle strength in individual muscles. The range of control limits was wide, varying from <10% of baseline (in 36% of muscles tested) to >50% (in 4% of muscles tested), with 88% of muscles falling within 30% of baseline. Follow-up data were also collected from all patients, including those undergoing treatment. Control limits analysis is a powerful and simple method for assessing the significance of motor performance changes in individual muscles in patients undergoing serial monitoring and can be easily applied to both single patients and clinical trials.     

Establishment of a novel rehabilitation program for patients with dropped head syndrome: Short and intensive rehabilitation (SHAiR) program

The pathophysiology of dropped head syndrome (DHS) remains unknown, and its treatment algorithm and indication are not standardized. Here, we established a novel rehabilitation program, short and intensive rehabilitation program for DHS (SHAiR program), consisting of cervical paraspinal muscles exercise, range of motion exercise, cervical and thoracic mobilization, deep cervical flexor muscle exercise, hip lift exercise, anterior pelvic tilt exercise, and walking exercise. The aim of this study was to evaluate the clinical effectiveness of this program. We reviewed clinical outcomes for five consecutive patients with DHS who underwent the SHAiR program (SHAiR group). The outcomes were compared with those of other five patients with DHS who received exercise instruction (control group). Demographic data, the duration from onset of DHS, the apex of sagittal kyphosis on the lateral radiographs, and clinical outcomes including the ability to maintain normal horizontal gaze, chin brow vertical angle, and numerical rating scale (NRS) were evaluated at the initial visit and final follow-up at 7.5 months. There was no significant difference between the two groups in terms of demographic and radiographic data. The ability of horizontal gaze and NRS of cervical pain improved rapidly for all five patients in the SHAiR group as compared to no improvement for all patients in the control group. Rehabilitation for DHS was considered effective not only for localized rehabilitation such as exercise for training cervical extensor muscle function but also exercises for thoracolumbar posture improvement and the psoas muscle.     

Effect of the short and intensive rehabilitation (SHAiR) program on dynamic alignment in patients with dropped head syndrome during level walking

The purpose of this study was to assess the change of dynamic alignment after the short and intensive rehabilitation (SHAiR) program in patients with dropped head syndrome (DHS). Eighteen patients with DHS patients who complained of their inability to maintain horizontal gaze and underwent the SHAiR program. Patients performed level walking at a self-selected speed. Spatiotemporal, kinematic, and kinetic data were recorded using a three-dimensional motion analysis system. Statistical analysis was performed to compare these data before and after the SHAiR program. Those who underwent the SHAiR program showed a significant increase in the head angle and stride length compared to pre-treatment measurements (p < 0.05). The SHAiR program modifies the malalignment of the head and neck and spatiotemporal parameters in DHS patients during level walking.     

The role of corticosteroids in the management of chronic subdural hematoma: a systematic review

The role of corticosteroids in the management of chronic subdural hematoma (CSDH) remains a matter of debate. Standard surgical treatment has recurrence rates reported between 4 and 26%. We reviewed the safety and effectiveness of corticosteroids both as a monotherapy and as an adjunct to surgery in patients with CSDH. PubMed‐MEDLINE, EMBASE and Cochrane databases were searched in July 2011 for randomized controlled trials and for prospective and retrospective cohort studies, reporting on 10 or more adult patients with CSDH. Quality was assessed according to the STROBE checklist. Corticosteroid monotherapy and surgery with corticosteroids as an adjunct were compared with no treatment or surgery only, with regard to lethality, neurological outcome, secondary intervention and complications. Five observational studies were included in this review. There was no randomized allocation of treatment in any study. Secondary intervention rates ranged from 3 to 28%, lethality rates ranged from 0 to 13%, and good outcome was seen in 83–100%. Hyperglycemia occurred more often in patients treated with corticosteroids. In only two studies, one case of gastrointestinal bleeding was observed. Five observational studies suggest that corticosteroids might be beneficial in the treatment of CSDH; however, there is a lack of well‐designed trials that support or refute the use of corticosteroids in CSDH. These results encourage further randomized clinical trials to establish the role of corticosteroids in CSDH.     

Treatment resistance of depression after head injury: a preliminary study of amitriptyline response.

Thirteen patients with depression following minor head injury were matched with 13 functional depressives. Both groups were indistinguishable psychopathologically. Five of the functional group had a family history of affective disorder and none of the head-injury group had such a history. Both groups were treated with amitriptyline, beginning with 100 mg daily, increasing to a maximum of 250 mg/d. There were significant differences in response between the 2 groups, with only 4 of the head-injury patients improving. Our preliminary results suggest that this condition is relatively resistant to tricyclic therapy.     

Light microscopic response of neuronal somata,dendrites and axons to post-mortem concussive head injury

Summary Forty anesthetized rats were cooled below 3°C by 30-min transcardial perfusion of chilled physiological saline before a concussive head injury. The animals were then perfusion-fixed with a buffered formaldehyde-glutaraldehyde solution. Another forty rats were fixed by 30-min transcardial perfusion of the same fixative before a similar concussive head injury. In brain sections of both groups of animals a new silver method stained, in a Golgi-like fashion, a number of neurons and long axonal segments scattered among unstained ones. The similarity between these findings and those obtained following in vivo concussive head injuries described in accompanying papers suggests that the formation of traumatically induced argyrophilic neuronal damage is independent of metabolic processes, i.e., it may be a primary morphopathological process.     

Triphasic behavioral response of motor units to submaximal fatiguing exercise

We have measured the firing rate and amplitude of 4551 motor unit action potentials (MUAPs) recorded with concentric needle electrodes from the brachial biceps muscles of 10 healthy young adults before, during, and after 45 minutes of intermittent isometric exercise at 20% of maximum voluntary contraction (MVC), using an automatic method for decomposition of electromyographic activity (ADEMG). During and after exercise, MUAPs derived from contractions of 30% MVC showed progressive increase in mean firing rate (P less than or equal to .01) and amplitude (P less than or equal to .05). The firing rate increase preceded the rise in mean amplitude, and was evident prior to the development of fatigue, defined as reduction of MVC. Analysis of individual potentials revealed that the increase in firing rate and in amplitude reflected different MUAP subpopulations. A short-term (less than 1 minute) reduction in MUAP firing rates (P less than or equal to .05) was also observed at the onset of each test contraction. These findings suggest that motor units exhibit a triphasic behavioral response to prolonged submaximal exercise: (1) short-term decline and stabilization of onset firing rates, followed by (2) gradual and progressive increase in firing rates and firing variability, and then by (3) recruitment of additional (larger) motor units. The (2) and (3) components presumably compensate for loss of force-generating capacity in the exercising muscle, and give rise jointly to the well-known increase in total surface EMG which accompanies muscle fatigue.     

Effect of lead trajectory on the response of essential head tremor to deep brain stimulation

BackgroundEssential tremor (ET) is one of the most common movement disorders. Normally ET affects the distal upper extremities, but it can also be accompanied by midline symptoms. Ventralis intermedius (VIM) thalamic deep brain stimulation (DBS) has been shown to be effective in reducing hand tremor, but its effects on head tremor have been inconsistent.MethodsTwenty-nine DBS patients with a diagnosis of ET met inclusion criteria. All implantations targeted VIM. The factors examined included age, gender, disease duration, presence or absence of head tremor, handedness, and the Fahn-Tolosa-Marin rating scale (TRS). This analysis specifically focused on TRS head tremor sub-scores at baseline, 6 months and 12 months post-DBS. Additionally, DBS lead entry angles were examined.ResultsTwenty-three ET patients underwent unilateral DBS and six underwent staged bilateral DBS. At both 6 and 12 months following DBS, stimulation resulted in diminished head tremor (ON vs OFF; p < 0.0001). The most important predictor of head tremor suppression was the entry angle of the DBS lead in the sagittal projection relative to the AC–PC axial plane (AC–PC angle). Head tremor reduction was greater among more vertical AC–PC angles.ConclusionA more vertical AC–PC angle of the DBS lead trajectory was associated with improved head tremor suppression. Further studies will be necessary to confirm this potentially important finding.     

Prevalence of family history in vasovagal syncope and haemodynamic response to head up tilt in first degree relatives

Vasovagal syncope is an exaggerated form of the common faint affecting all age groups. Aetiology is unknown but the tendency for the disease to run in families has previously been noted. Aim: To determine the true prevalence of family history in subjects with a definitive diagnosis of vasovagal syncope made by positive head up tilt with symptom reproduction. To determine the strength of the genetic effect in vasovagal syncope by calculation of sibling (λs) and offspring (λo) relative risk. Haemodynamic responses to head up tilt were also examined in a sample of first-degree relatives of those with vasovagal syncope. Results: All subjects identified from the Cardiovascular Investigation Unit database with a definitive diagnosis of vasovagal syncope (n = 603) between 1993–2001 were asked to complete a questionnaire. 19 % had positive family history for blackouts or faints. From these pedigrees and using a crude estimate of population prevalence, sibling and offspring relative risk was calculated: λs = 1080, λo = 1356. Eleven first-degree relatives from 6 families attended for head up tilt testing with glyceryl trinitrate (GTN) provocation (4 unaffected, 7 affected). All subjects had symptoms in response to tilt in association with a range of haemodynamic responses. Conclusions: Vasovagal syncope has a strong genetic component. Elucidating underlying genetic mechanisms may lead to more effective, specific treatments. Received: 6 November 2001, Accepted: 31 October 2002 Correspondence to Julia L. Newton, MD, PhD     

Current incidence and management of children with traumatic head injuries: The Stockholm experience

Purpose: A traumatic head injury is one of the most common causes of morbidity and mortality among children, however few population-based studies in this area have been reported. Therefore, the aim of this study was to evaluate the incidence and management of traumatic head injuries in children at a level-one trauma centre in Stockholm, Sweden.

Participants: All children (n?=?3168) who visited the emergency department with a history of head injury during 1 year were included.

Method: The required information was collected retrospectively and the children's medical records were reviewed.

Results: The overall incidences of head injury were 865 per 100 000 children with the highest incidence (2379/10⁵ children) occurring among children younger than 18 months of age. Twelve per cent (n?=?396) were admitted to a hospital ward and CT scans were performed in 13% (n?=?412) of all children. During this year, 0.3% required a neurosurgical intervention and only 1% of all children had documentation of a planned follow-up appointment.

Conclusion: The findings indicate that clinical documentation as a part of the early management in children with a head injury is inconsistent and suffers from lack of valid criteria. Implementation of clinical guidelines during emergency care would help improve subsequent hospital care, as well as the planning of health care services for these children.     

An immediate light microscopic response of neuronal somata,dendrites and axons to contusing concussive head injury in the rat

Summary Thirty-four rats were killed by transcardial perfusion fixation 1 min after a contusing concussive head injury, and 17 rats 1 day later. From the results obtained with a new silver method demonstrating traumatically damaged neuronal somata, dendrites and axons the following conclusions were drawn: (1) outside the contused territories all features of traumatically induced neuronal argyrophilia are similar to those found in non-contusing concussive head injury, as reported in an accompanying paper; (2) within contused territories the neuronal argyrophilia is abolished by some substance released either from damaged blood vessels or from damaged parenchymal cells, while the neuronal damage otherwise underlying the induction of argyrophilia is present; (3) different phenotypes of neurons are vulnerable to different values of the parameters of the intracranial pressure wave generated by the trauma; (4) some of the neurons may recover from the traumatically induced argyrophilic damage; (5) traumatically induced inundation of neurons with extracellular tracers, as reported by other authors, and somato-dendritic argyrophilia may be different manifestations of one and the same phenomenon; and (6) diffuse primary traumatic axonal injury in human neuropathology may be closely correlated to axonal argyrophilia.