Dejerine Sottas syndrome

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Dejerine Sottas syndrome Classification and external resources
ICD-10	G60.0
ICD-9	356.0
OMIM	145900
DiseasesDB	5821
MeSH	D015417

Dejerine-Sottas Syndrome/Neuropathy (hereditary motor and sensory polyneuropathy type III; sometimes also described as a subtype III of Charcot-Marie-Tooth disease) is an autosomal dominant or autosomal recessive neuropathy.

Symptoms

It is usually characterized by infantile onset of moderate to severe lower and upper extremity weakness and loss of sensation.

Symptoms are usually more severe and rapidly progressive than in the other more common Charcot-Marie-Tooth diseases, and some carriers may never walk and be wheelchair-bound by the end of their first decade, while others may need only a cane (walking stick) or similar support through life.

Symptoms range from very common (muscle weakness, reduced sensation in the extremities, pain in the extremities, clawed hands and deformed feet, ataxia, peripheral areflexia) to rare (hearing loss, nystagmus, anisocoria).

Diagnosis

On medical imaging, the nerves of the extremities (and cranial nerves in some cases) appear enlarged due to hypertrophy of the connective interstitial tissue, giving the nerves a distinct 'onion-bulb' appearance. Peripheral (and possibly cranial) nerve excitability and conduction speed are reduced.

External Links

v • d • e Nervous system pathology, primarily PNS (G50-G99, 350-359)
Nerve, nerve root and plexus disorders (neuropathy, radiculopathy, plexopathy)	cranial nerve: V (Trigeminal neuralgia) - VII (Facial nerve paralysis, Bell's palsy, Melkersson-Rosenthal syndrome, Central seven) - XI (Accessory nerve disorder) nerve root and plexus: Brachial plexus lesion - Thoracic outlet syndrome - Phantom limb mononeuropathy: upper limb (Carpal tunnel syndrome, Ulnar nerve entrapment, Radial neuropathy) - Causalgia - lower limb (Meralgia paraesthetica, Tarsal tunnel syndrome, Morton's neuroma) - Mononeuritis multiplex
Polyneuropathies and other disorders of the PNS	hereditary and idiopathic (Charcot-Marie-Tooth disease, Dejerine Sottas syndrome, Refsum's disease, Morvan's syndrome) Guillain-Barré syndrome - Alcoholic polyneuropathy
Diseases of myoneural junction	autoimmune: Myasthenia gravis - Lambert-Eaton myasthenic syndrome
Diseases of muscle (myopathy)	Muscular dystrophy myotonic (Myotonic dystrophy, Myotonia congenita, Thomsen disease, Neuromyotonia, Paramyotonia congenita) congenital myopathy (Centronuclear myopathy, Nemaline myopathy, Central core disease) Mitochondrial myopathy periodic paralysis: Hypokalemic - Hyperkalemic
Autonomic	Familial dysautonomia - Horner's syndrome - Multiple system atrophy (Shy-Drager syndrome, Olivopontocerebellar atrophy)

Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .