Dropped head in systemic sclerosis: a case based review (2023)

Abstract

Dropped head syndrome is a rare disease entity characterized by severe weakness of the cervical para-spinal muscles, resulting in a chin-on-chest deformity. Systemic sclerosis is one of the causes of dropped head syndrome, but its characteristics and prognosis remain unclear due to the extreme rarity of this condition. We present a case of dropped head which occurred in systemic sclerosis. He presented with severe dropped head and relatively mild weakness of the proximal limb muscles. Serum level of creatine kinase was elevated, myopathic change was observed in electromyography, and gadolinium enhancement was found in magnetic resonance imaging of his posterior neck muscles. Anti-topoisomerase I antibody was positive, while other autoantibodies such as anti-PM/Scl and anti-Ku antibodies were negative. Since his dropped head acutely progressed, high dose of glucocorticoid therapy was initiated, which successfully improved dropped head, serum level of creatine kinase, and gadolinium enhancement in magnetic resonance imaging. Our present case and literature review suggest that dropped head occurring in systemic sclerosis can be treatable with immunosuppressive therapy. It is important to recognize this rare but treatable involvement of systemic sclerosis because early diagnosis and treatment initiation are crucial to prevent the irreversible organ damage and the significant decrease of daily activities.

Original languageEnglish
Pages (from-to)1483-1489
Number of pages7
JournalRheumatology International
Volume42
Issue number8
DOIs
Publication statusPublished - 2022 Aug

Keywords

  • Anti-topoisomerase I antibody
  • Dropped head syndrome
  • Inflammatory myopathy
  • Myositis
  • Systemic sclerosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology

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Shimada, T., Higashida-Konishi, M., Akiyama, M., Hama, S., Takei, H., Izumi, K., Oshima, H., & Okano, Y. (2022). Dropped head in systemic sclerosis: a case based review. Rheumatology International, 42(8), 1483-1489. https://doi.org/10.1007/s00296-021-04942-z

Dropped head in systemic sclerosis: a case based review. / Shimada, Tatsuya; Higashida-Konishi, Misako; Akiyama, Mitsuhiro et al.
In: Rheumatology International, Vol. 42, No. 8, 08.2022, p. 1483-1489.

Research output: Contribution to journalReview articlepeer-review

Shimada, T, Higashida-Konishi, M, Akiyama, M, Hama, S, Takei, H, Izumi, K, Oshima, H & Okano, Y 2022, 'Dropped head in systemic sclerosis: a case based review', Rheumatology International, vol. 42, no. 8, pp. 1483-1489. https://doi.org/10.1007/s00296-021-04942-z

Shimada T, Higashida-Konishi M, Akiyama M, Hama S, Takei H, Izumi K et al. Dropped head in systemic sclerosis: a case based review. Rheumatology International. 2022 Aug;42(8):1483-1489. doi: 10.1007/s00296-021-04942-z

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Shimada, Tatsuya ; Higashida-Konishi, Misako ; Akiyama, Mitsuhiro et al. / Dropped head in systemic sclerosis : a case based review. In: Rheumatology International. 2022 ; Vol. 42, No. 8. pp. 1483-1489.

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FAQs

Is dropped head syndrome serious? ›

The dropped head syndrome occurs among 1-3% of individuals diagnosed with Amyotrophic Lateral Sclerosis (ALS) or sometimes referred to Motor Neurone Disease. Even though this is quite a rare manifestation of ALS but bears a poor prognosis.

What can be done for dropped head syndrome? ›

Neck collars are one of the most useful treatments for Dropped Head Syndrome. Wearing a neck collar when you are up will likely improve your activities of daily living. The collar can partially correct the chin-on-chest deformity. This improves forward gaze and activities of daily living.

What is the differential diagnosis of head drop? ›

What causes this condition? Most of the time, Dropped Head Syndrome is caused by a specific generalized neuromuscular diagnosis. These include amyotrophic lateral sclerosis (ALS) also known as Lou Gehrig's disease, Parkinson's disease, myasthenia gravis, polymyositis, and genetic myopathies.

Is there a cure for dropped head syndrome? ›

(while) surgical treatment can dramatically correct the cervical kyphosis of dropped head syndrome patients. After surgery, however, patients' neck mobility is severely restricted. This is a great disadvantage for patients. Thus, surgical indications for dropped head syndrome should be considered carefully.

What is the progression of dropped head syndrome? ›

With progression, it becomes hard to hold the head up for a long time, and then the head drops all the time so that the patient has to hold his or her chin up to see. Excessive strain on the neck extensors leads to posterior cervical pain, which is a common early manifestation.

How rare is dropped head syndrome? ›

Dropped head is a rare manifestation of ALS occurring around 1-3% of patients. 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.

Is dropped head syndrome a disability? ›

Patients with dropped head syndrome are unable to lift their chin off the chest wall and find difficulty in looking forward while walking, talking, or eating, using their hands to support their chin. The head drop not only causes significant disability but is also a source of social embarrassment.

Can medications cause dropped head syndrome? ›

The 'dropped head sign' is considered a rare symptom of unknown etiology in parkinsonian disorders, though a disproportionate antecollis is frequently observed in multiple system atrophy. It has also been described as a side effect of dopamine agonist medication with cabergoline and pramipexole.

What is the medical term for a dropped head? ›

Dropped head syndrome (DHS) is characterized by severe kyphotic deformity of the cervico-thoracic spine. It is a relatively rare condition with a broad differential diagnosis. 1. The conditions linked with DHS can be categorized into neurological, neuromuscular, muscular, and other causes (Table 1).

Can a stroke cause dropped head syndrome? ›

We report a rare case of transient "dropped head syndrome" (DHS) after acute ischemic stroke. A 64-year-old man noticed a sudden onset of mild weakness in his left hand and also difficulty in preventing his head from dropping onto his chest without weakness of the neck extensor muscles.

What is the ICD 10 code for dropped head syndrome? ›

ICD-10 Code for Cervicocranial syndrome- M53. 0- Codify by AAPC.

What muscle holds your head up? ›

Posterior neck muscles include: Splenius capitis and splenius cervicis: Strap-like muscles in the back of your neck that help you extend and rotate your head. Suboccipital muscles: Four muscles just below the occipital bone at the base of your skull. They help extend your head in different directions.

What causes inability to lift head? ›

The dropped head syndrome (DHS) is a disabling condition caused by severe weakness of the neck extensor muscles causing progressive reducible kyphosis of the cervical spine and the inability to hold the head up. Weakness can occur in isolation or in association with a generalized neuromuscular disorder.

How does ALS affect the neck? ›

Neck muscle weakness and head drop are well recognised in patients with Amyotrophic lateral sclerosis (ALS), but an objective characterisation of the consequent head movement impairment is lacking.

How do you strengthen weak neck muscles? ›

Tilting and holding your head on each side is one repetition.
  1. With your head facing forward, slowly tilt your head towards one shoulder as far as is comfortable – you should feel a stretch on the opposite side of your neck.
  2. Hold for 2 seconds then return to where you started.
  3. Repeat on the other side.

What neurological disorders qualify for disability? ›

What types of neurological disorders does the SSA consider disabling?
  • Epilepsy.
  • Benign brain tumors.
  • Parkinsonian syndrome.
  • Cerebral palsy.
  • Spinal cord disorders.
  • Multiple sclerosis.
  • Amyotrophic lateral sclerosis (ALS)
  • Muscular dystrophy.

Why does it feel like my neck can't support my head? ›

Muscle strain

Any injury that causes strain or pain in the muscles of the head and neck can make your head feel heavy and more difficult to hold up. Sports injuries, car accidents, or overexertion of the neck caused by lifting heavy items, can put strain on the neck muscles and lead to a heavy head feeling.

What does dropped my head mean? ›

: to look down. When the teacher became angry at her, she dropped her eyes/head.

What is the medical term for drop? ›

gtt.: Abbreviation meaning drops (from the Latin "guttae", drops). One of a number of hallowed abbreviations of Latin terms that have traditionally been used in prescriptions.

What does a stroke droop look like? ›

Face drooping is one of the most common signs of a stroke. One side of the face may become numb or weak. This symptom may be more noticeable when the patient smiles. A lopsided grin could indicate that the muscles on one side of the face have been affected.

What muscles hold your head up in your neck? ›

The two trapezius muscles together form a kite shape. The trapezius muscle can be involved in extending the head upward or neck backward, rotating/turning the head, or lifting the shoulder blade.

Can neck position cause a stroke? ›

This type of rare injury leading to stroke isn't just limited to the hair salon. “Extreme head and neck motions pose a risk of wounding the cerebral arteries and thus, producing clots, swelling and constriction, which can cause serious damage in the brain,” Dr. Zand said.

What is the ICD-10 for dropped something? ›

ICD-10 code W20 for Struck by thrown, projected or falling object is a medical classification as listed by WHO under the range - Other external causes of accidental injury .

What is the ICD for abnormal head movements? ›

ICD-10 code R25. 0 for Abnormal head movements is a medical classification as listed by WHO under the range - Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified .

What is ICD 9 head fall? ›

WISH Injury-Related Traumatic Brain Injury ICD-9-CM Codes
ICD-9-CM CodeDescription
850.0-850.9Concussion
851.00-854.19Intracranial injury, including contusion, laceration, and hemorrhage
950.1-950.3Injury to the optic chiasm, optic pathways, or visual cortex
959.01Head injury, unspecified
3 more rows
Jul 5, 2020

How far should you be able to turn your head to the side? ›

Between 160 to 180 degrees; i.e. you should be able to rotate your neck to the right and to the left, so that your nose is in line with your shoulder (or near enough).

Which muscles restore the head to its natural position? ›

The longus capitis and rectus capitis anterior are the direct antagonists of the muscles at the back of the neck, serving to restore the head to its natural position after it has been drawn backward. These muscles also flex the head, and from their obliquity, rotate it, so as to turn the face to one or the other side.

What is the pain of dropped head syndrome? ›

Dropped Head Syndrome Results In Neck Pain, Trouble Eating & Interferes With Vision. Dropped head syndrome causes a recognizable deformity with varying degrees of “chin on chest” posture. There is usually weakness of the neck muscles that results in the head dropping forward, sometimes called ptosis of the head.

Why can I barely lift my arm over my head? ›

You can't move your arm or lift it over your head

A “frozen shoulder” is often a sign of a rotator cuff tear or another injury that's become inflamed and swollen. The rotator cuff is a set of four muscles and their corresponding tendons that hold your shoulder in its joint and allow you a great range of motion.

What does it mean if I can't lift my arm above my head? ›

Rotator cuff pain is the number one reason you have pain on the outside or top of your arm. It is also the most common reason you may not be able to lift or move your arm. Shoulder pain could also be brought on by injury. Believe it or not, traumatic injuries are a less frequent cause of shoulder pain.

What are signs that ALS is progressing? ›

As the disease progresses, muscle weakness and atrophy spread to other parts of your body. You may develop problems with: People with ALS eventually will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. Chewing food and swallowing (dysphagia)

What are 3 things that can lead to ALS? ›

Environmental factors, such as the following, have been associated with an increased risk of ALS .
  • Smoking. Evidence supports that smoking is an environmental risk factor for ALS . ...
  • Environmental toxin exposure. ...
  • Military service.

Will an MRI of the neck show ALS? ›

MRI revealed marked abnormalities at the time of ALS diagnosis. Early atrophy of the cervical spinal cord may predict the progression of respiratory disorders, and so may be of value in patient care and as a primary endpoint in pilot neuroprotection studies.

What happens if flat head syndrome is left untreated? ›

If congenital plagiocephaly, which is caused by craniosynostosis, is left untreated, it can lead to serious complications, including: Head deformities, possibly severe and permanent. Increased pressure inside the head. Seizures.

How long do you need to worry about flat head syndrome? ›

When does flat head syndrome go away? Flat head syndrome is most common between the ages of 6 weeks and 2 months old, and almost always resolve completely by age 2, particularly if parents and caregivers regularly work on varying baby's positions when he's awake.

How long does flat head syndrome last? ›

Your baby's head may not return to a completely perfect shape, but by the time they're 1 or 2 years old any flattening will be barely noticeable. More severe cases will also get better over time, although some flattening will usually remain.

Can medications cause Dropped Head Syndrome? ›

The 'dropped head sign' is considered a rare symptom of unknown etiology in parkinsonian disorders, though a disproportionate antecollis is frequently observed in multiple system atrophy. It has also been described as a side effect of dopamine agonist medication with cabergoline and pramipexole.

What is a severe case of flat head syndrome? ›

Signs and Symptoms of Flat Head Syndrome

The ear on the side that has flattened may be bent forward. If your little one has a severe case, the forehead may bulge out on the opposite side. Your baby's neck, jaw, and face may look uneven if the flat head syndrome is caused by a stiff neck muscle.

What is considered severe flat head? ›

An asymmetry of over 12mm is considered moderate, whilst a difference of over 18mm is regarded as a severe flat head. We often see asymmetries of over 25mm as well as head shapes than are wider than they are long, at significantly over 100%.

Does flat head affect brain? ›

In most cases, a flattened head does not affect brain growth or mental development. The condition typically improves — though may not resolve completely — once your child spends more time sitting and standing.

How do I stop my flat head from getting worse? ›

Try these tips:
  1. Practice tummy time. Provide plenty of supervised time for your baby to lie on the stomach while awake during the day. ...
  2. Vary positions in the crib. Consider how you lay your baby down in the crib. ...
  3. Hold your baby more often. ...
  4. Change the head position while your baby sleeps.

What are the disadvantages of flat head syndrome? ›

While it can temporarily cause a misshapen head and possible misalignment of ears and eyes, the effects are generally mild and tend to resolve as a baby ages and becomes more mobile. Positional plagiocephaly doesn't affect brain development and, in many cases, it needs no medical intervention and resolves on its own.

Can flat head syndrome cause headaches? ›

In addition, skull deformities may contribute to headaches, migraines, painful teething, jaw and dental problems. It is therefore recommended that treatment takes place as early as possible, in order to restore normal head shape.

How do you fix flat head syndrome in adults? ›

Although it is not possible to undertake major skull reshaping surgery in adults, the situation can often be improved by reshaping the outer layers of the skull (burring) or by inserting implants to improve skull shape. Small irregularities can be treated with fat transfer.

Do cranial helmets work? ›

Do not recommend helmet therapy for positional skull deformity in infants and children. Wearing a helmet causes adverse effects but does not alter the natural course of head growth.

What is corrective for flat head syndrome? ›

A corrective helmet can help treat your baby's flat head syndrome. The helmet, also called a cranial remolding orthosis (CRO), is designed to gently correct the shape of your child's skull over time. Your child will likely need to wear the helmet for three to five months.

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