(Q06.2) Diastematomyelia

More details coming soon

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51 463 in individuals diagnosis diastematomyelia confirmed
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1 311 deaths with diagnosis diastematomyelia
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3% mortality rate associated with the disease diastematomyelia

Diagnosis diastematomyelia is diagnosed Women are 0.62% more likely than Men

25 573

Men receive the diagnosis diastematomyelia

0 (less than 0.1%)

Died from this diagnosis.

100
95
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80
75
70
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60
55
50
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15
10
5
0
25 890

Women receive the diagnosis diastematomyelia

1 311 (5.1 %)

Died from this diagnosis.

Risk Group for the Disease diastematomyelia - Men and Women aged 0-5

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In Men diagnosis is most often set at age 0-69, 75-79
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Less common in men the disease occurs at Age 70-74, 80-95+Less common in women the disease occurs at Age 75-95+
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In Women diagnosis is most often set at age 0-74

Disease Features diastematomyelia

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Non-contagious
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Moderate individual risk, low public risk
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Diastematomyelia - what does this mean

Diastematomyelia is a rare congenital disorder where the spinal cord is split into two parts, caused by a bony or fibrous septum that runs down the center of the spinal canal. it is usually associated with a tethered cord and can cause nerve damage, weakness, and pain.

What happens during the disease - diastematomyelia

Дефект считают результатом неправильного развития эмбриональной хорды и нервной трубки с 15 по 18 дни беременности, когда происходит закладка основных зародышевых структур на этапе нейруляции. Аномальное сообщение между зачатком спинного мозга и примитивным нейрокишечным каналом или амниотическим мешком формируется путем создания эндомезенхимального тракта. Последний образуется из мезодермальных элементов, заполняющих патологический свищевой канал.

 

Разделение каудального мозга в продольном направлении сопровождается образованием костной, хрящевой либо фиброзной срединной перегородки или шпоры. Она исходит из тела или дужки позвонка, может распространяться на всю ширину спинального канала, прикрепляясь к вентральной части твердой мозговой оболочки. Спинной мозг на протяжении нескольких сегментов расщепляется на два обычно неравных рукава, снабженных собственным центральным каналом, что является основной патоморфологической особенностью диастематомиелии.

 

Общие эмбриогенетические механизмы обуславливают частую связь патологии с другими миелодиспластическими состояниями. Заболевание может сочетаться с миеломенингоцеле, спинальными липомами и кистами, дермальным синусом. Выявляют ассоциацию с врожденными дефектами позвоночника – гемивертеброй, бабочковидными позвонками, сколиозом. При множественных признаках дизрафии встречаются висцеральные пороки с поражением почек, прямой кишки и половых органов.

Clinical Pattern

More details coming soon

How does a doctor diagnose

  • Physical examination
  • X-ray imaging
  • Magnetic resonance imaging (MRI)
  • Computed tomography (CT) scan
  • Myelogram
  • Electromyography (EMG)
  • Nerve conduction velocity (NCV) test
  • Blood tests

Treatment and Medical Assistance

Main Goal: Treat Diastematomyelia
  • Perform MRI scan to identify the extent of the condition
  • Prescribe pain medications to manage the symptoms
  • Provide physical therapy to strengthen the muscles and improve mobility
  • Perform surgery to separate the spinal cord and repair the vertebrae
  • Administer corticosteroids to reduce inflammation
  • Provide bracing to stabilize the spine
  • Recommend lifestyle changes to reduce stress on the spine
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13 Days of Hospitalization Required
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Average Time for Outpatient Care Not Established

Diastematomyelia - Prevention

The best way to prevent diastematomyelia is to maintain a healthy lifestyle with a balanced diet, regular exercise, and adequate rest. it is also important to avoid activities that can cause trauma to the spine, such as contact sports or extreme physical activity. additionally, regular check-ups with a doctor can help to identify any potential signs of the condition.

Main symptoms of the disease diastematomyelia

Muscle weakness

(Aching muscles, Lack of strength, Muscular fatigue, Decreased muscle power, Reduced muscle tone, Muscle exhaustion, Muscle debility, Lack of muscle control, Lack of muscular coordination, Loss of muscle strength)

Muscle weakness is a decrease in the strength of the muscles that can lead to difficulty in performing everyday tasks. It can manifest in a variety of ways, including fatigue, difficulty walking, and reduced range of motion. It can develop gradually or suddenly, and can be caused by a variety of med...

Age: 0 to 99Accociated with 47 diseases

Dissociated sensory loss

(Dissociated sensory deficit, Partial sensory loss, Differential sensory loss, Discontinuous sensory loss, Segmental sensory loss, Loss of sensation, Asymbolia, Partial anesthesia, Sensory disconnection)

Dissociated sensory loss is a neurological symptom characterized by the loss of sensation in a specific area of the body, while other areas remain unaffected. It is often caused by damage to the nerve pathways that transmit sensory information from the affected area. Symptoms typically include a lac...

Age: any ageAccociated with 11 diseases

Urinary incontinence

(Bladder control problem, Urinary leakage, Involuntary urination, Urge incontinence, Overactive bladder, Stress incontinence, Functional incontinence, Mixed incontinence)

Urinary incontinence is the loss of bladder control, resulting in the involuntary leakage of urine. It can range from mild to severe, and can manifest in different ways. Stress incontinence is the most common type, where physical activities such as coughing, sneezing, or laughing can cause leakage. ...

Age: 1 to 100Accociated with 22 diseases

Skin depigmentation

(Discoloration, Lightening, Whitening, Bleaching, Fading, Loss of pigmentation, Depigmenting, Pallor, Chalkiness)

Skin depigmentation is a condition in which patches of skin lose their normal color, becoming lighter or completely white. It can appear in different forms and can develop gradually or suddenly. It is often accompanied by itching, burning, or a tingling sensation. Common types of skin depigmentation...

Age: any ageAccociated with 9 diseases

Excess hair growth

(Hypertrichosis, Hirsutism, Excessive hair growth, Unwanted hair growth, Facial hair growth, Excessive body hair, Abnormal hair growth, Unusual hair growth, Excess body hair, Hypertrichiasis)

Excess hair growth is a common symptom of hormonal imbalances. It can appear as increased facial and body hair, in both men and women. It usually starts with a slight increase in the amount of hair, and then progresses to a more noticeable amount of hair. It can manifest as thick, coarse hairs on th...

Age: 1 to 100Accociated with 11 diseases

Incontinence of feces

(Bowel incontinence, Fecal incontinence, Loss of bowel control, Loss of fecal control, Accidental bowel leakage, Accidental fecal leakage)

Incontinence of feces is an inability to control the bowels, resulting in the involuntary passage of feces. This symptom may present as either fecal incontinence (involuntary leakage of solid stool) or diarrhea (involuntary leakage of loose, watery stool). It can develop due to a variety of causes, ...

Age: any ageAccociated with 14 diseases

Kyphosis and lordosis

(Humpback, Roundback, Hunchback, Hunching, Swayback, Swaybacked, Concave back, Convex back, Hyperlordosis, Hypolordosis)

Kyphosis and lordosis are spinal conditions that involve an excessive curvature of the spine. Kyphosis is an outward curvature of the spine, resulting in a hump-like appearance in the upper back. Lordosis is an inward curvature of the spine, resulting in a swayback appearance in the lower back. Both...

Age: 1 to 100Accociated with 4 diseases

Scoliosis

(Spinal curvature, Curvature of the spine, Lateral curvature of the spine, Abnormal curvature of the spine, Side-to-side curvature of the spine, Lateral spinal deviation, Asymmetrical spinal curvature, Abnormal lateral curvature of the spine)

Scoliosis is a condition where the spine curves sideways in an "S" or "C" shape instead of its normal straight line. It can develop in childhood or adolescence and is characterized by uneven shoulders, a tilted pelvis, and a raised or lowered hip. It can also cause pain, fatigue, and respiratory pro...

Age: 1 to 100Accociated with 10 diseases

Bone deformity

(Skeletal abnormality, Bone malformation, Skeletal malformation, Bone abnormality, Skeletal disorder, Bone disorder, Bone deformity, Skeletal deformity, Bone misalignment, Skeletal misalignment)

Bone deformity is a condition in which the shape of the bone is altered due to disease or injury. It can be caused by a number of factors, including genetic abnormalities, trauma, infection, or tumors. Symptoms of bone deformity can include pain, swelling, limited range of motion, and deformity of t...

Age: any ageAccociated with 25 diseases