This creates structural problems throughout the skeletal muscle and in the Z line of the sarcomere, creating the weakness in the muscle. Myopathies with fiber size variation
Myopathies with varying fiber size, such as congenital fiber type disproportion,
occurs when type 1 fibers, the slow twitch fibers involved in sustaining activity, are smaller than type 2 fibers, the fast twitch fibers involved in quick activity. Since smaller type 1 fibers is not associated with nemaline myopathy, the most common type of congenital myopathy, it has not been studied in as great detail as many of the others. However, the smaller type 1 fibers explains why patients typically can participate in activities for shorter periods of time, but struggle with extended activity. Cause
Diagnosis
There are rarely any specific tests for the congenital myopathies except for muscle biopsy. Tests can be run to check creatine kinase in the blood, which is often normal or mildly elevated in congenital myopathies. Electromyography can be run to check the electrical activity of the muscle. Diagnosis heavily relies on muscle pathology, where a muscle biopsy is visualised on the cellular level. Diagnosis usually relies on this method, as creatine kinase levels and electromyography can be unreliable and non-specific. Since congenital myopathies are genetic, there have been advancements in prenatal screenings. Types
The conditions included under the term "congenital myopathy" can vary. One source includes nemaline myopathy, myotubular myopathy, central core myopathy, congenital fiber type disproportion, and multicore myopathy. The term can also be used more broadly, to describe conditions present from birth.
Palatal myoclonus is a rare condition in which there are rhythmic jerky movements or a rapid spasm of the palatal (roof of the mouth) muscles. Chronic clonus is often due to lesions of the central tegmental tract (which connects the red nucleus to the ipsilateral inferior olivary nucleus).When associated with eye movements, it is known as oculopalatal myoclonus. Signs and symptoms
Signs and symptoms of Palatal Myoclonus include:
- A rhythmic clicking sound in the ear due to the opening and closing of the Eustachian tube.- Rhythmic, jerky movements in the face, eyeballs, tongue, jaw, vocal cord or extremities (mostly hands). Diagnosis
Classifications
physiologic, essential, epileptic, and symptomatic

Treatment
Drugs
Drugs used to treat palatal myoclonus include clonazepam, carbamazepine, baclofen, anticholinergics, tetrabenazine, valproic acid, phenytoin, lamotrigine, sumatriptan, and PIR. A rare case of palatal myoclonus that associated with orofacial buccal dystonia has been treated with Botulinum toxin A (Dysport) injection and counseling. Notes
http://www.tchain.com/otoneurology/disorders/central/opm.html
https://www.hindawi.com/journals/criot/2013/231505/
"NINDS Tremor Information Page". National Institute of Neurological Disorders and Stroke. July 20, 2007. Archived from the original on October 6, 2007. Retrieved 2007-10-08. References


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