Differential Diagnosis of Amyotrophic Lateral Sclerosis

There are several diseases that have symptoms and signs such as Amyotrophic Lateral Sclerosis. Here are some diseases that become differential diagnosis for, Amyotrophicl Lateral Sclerosis (ALS):

1. Another Motor Neuron Disease (MND)

a. Primary lateral sclerosis (UMN signs only)
b. Progressive muscular atrophy (LMN signs only)
c. Progressive bulbar palsy
2. Anatomical abnormalities / compression syndrome :
a. Tumors of the spine
        Spinal cord tumors have clinical manifestations such as limb weakness, anesthesia, and the UMN lesion signs

b. Syringomyelia
        Sirinomyelia is a developmental disorder that is characterized by the presence of an abnormal cavity due to dilatation of the central canal of the spinal cord. This cavity is derived from the region but can midservikal extends upward to the medulla  produces syringobulbia) or down to the thoracic and lumbar regions. Cavity enlarges slowly over several years. Clinical syndrome characterized mixed between sensory and motor disturbances. Damage in the ventral part of the central gray leads on LMN signs, weakness, atrophy, fasciculations of intrinsic hand muscles, loss of arm reflkes always the case. UMN sign on the lower extremities occur with prolonged cavity to the corticospinal tracts . Syringobulbia can cause vocal cord paralysis, dysarthria, nystagmus, weakness of the tongue and Horner's syndrome.

c. Cervical spondylosis
          Can be found a combination of UMN and LMN lesions on the superior limb muscles. Usually accompanied by sensory disturbances. Although cervical spondylosis myelopathy severe, sometimes it can makes confusion with MND, especially if there is spasticity in the lower limbs and hyperrefexia in conjunction with muscle atrophy and fasciculations in the upper limbs, is not likely to cause widespread fasciculations, and weakness. Progressive limb weakness, asymmetrical, combined UMN signs and LMN in the arm , spastic paraparesis , sometimes fasciculations in arms.

3. Infection:
a. Lyme disease
          Neurologic manifestations of Lyme disease include meningitis and polyradiculoneuropathy. The second and third stages of Lyme disease associated with neurological changes that can cause neuropathy, a motor axonal low. Lyme disease is caused by a spirochete (Borrelia burgdorfere). Abnormalities in nerve roots occurs at the beginning or end stage of the disease. Symptoms include weakness, sensory disturbance and hyporeflexia on any part of the affected nerve root.

b. HIV myelopathy
         Myelopathy associated with HIV infection usually seen in the later stages of the disease. It is characterized by impaired walking (gait) with impaired sensory, sphincter disturbances and fast reflexes. In HIV myelopathy also a sign of UMN and LMN. Peripheral neuropathy (damage to axons) is a clinical sign of HIV.

4. Neuro Musculer Junction
a.Myasthenia gravis
        It is an acquired autoimmune disease and interfere with neuromuscular transmission at the neuromuscular junction due to shortage / damage ACh receptors. Typical complaints are muscle weakness after / momentary use, and improved after the break. Initiation Symptoms  are: (focal, bulbar muscles, limb muscles, eye muscles disturbance such as (diplopia, ptosis). Myasthenia gravis can cause weakness in the respiration muscles. There’s no Fasciculations and  sign of weakness UMN.

5. Endocrine :
a. Hyperthyroidism
        Clinical neurological manifestations of hyperthyroidism is varied including altered mental status, seizures , movement abnormalities such as tremors and khorea, eye disorders, weakness, atrophy, fasciculations . in addition, patients with hyperthyroidism in general have a deep tendon reflexes and fast , in some patients choose a damage of corticospinal tracts and Babinski sign. Patients with hyperthyroidism may develop weakness and signs combine with UMN , which resembles ALS.
       Of course, most patients with hyperthyroidism have evidence of toxic goiter, anxiety, and insomnia that can be distinguished with ALS . It is important to be declared, however, in older patients with hyperthyroidism may present with apathy and depression are called apathetic hyperthyroidism .

b. Hyperparathyroidism
       Neurological manifestations in patients with hyperparathyroidism commonly associated with hypercalcemia, hypophosphatemia, and elevated levels of parathyroid hormone da consists of such lethargi altered mental status, confusion, and eventually coma. when hyperkalemia or acute but not severe weakness and fatigue may appear as symptoms in primary hyperparathyroidism. Rarely, the patient develops symptoms of myopathy. Rarely, hyperparathyroidism and ALS coexist in patients, the likelihood increases if the increase in parathyroid hormone levels contribute to the development of motor neuron syndrome. Hypercalcemia and increased parathyroid hormone levels but may help distinguish between these endocrine disease with ALS .

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