Deformities of the vertebral column such as scoliosis may also be observed.While there is no universally accepted grading scale, the OMENS scale (standing for Orbital, Mandible, Ear, Nerves and Soft tissue) was developed to help describe the heterogeneous phenotype that makes up this sequence or syndrome.Intellectual disability is not typically seen in people with HFM. Hemifacial microsomia sometimes results in temporomandibular joint disorders. Cause
The condition develops in the fetus at approximately 4 weeks gestational age, when some form of vascular problem such as blood clotting leads to insufficient blood supply to the face. This can be caused by physical trauma, though there is some evidence of it being hereditary [2]. This restricts the developmental ability of that area of the face. Currently there are no definitive reasons for the development of the condition. Diagnosis
Classification
Figueroa and Pruzanksky classified HFM patients into three different types:
Type I : Mild hypoplasia of the ramus, and the body of the mandible is slightly affected. Type II : The condyle and ramus are small, the head of the condyle is flattened, the glenoid fossa is absent, the condyle is hinged on a flat, often convex, infratemporal surface, the coronoid may be absent. Type III: The ramus is reduced to a thin lamina of bone or is completely absent. There is no evidence of a TMJ. Treatment
Depending upon the treatment required, it is sometimes most appropriate to wait until later in life for a surgical remedy – the childhood growth of the face may highlight or increase the symptoms.
For diagnosis a person with documented responsiveness to medication has to increase medication intake beyond dosage needed to relieve their parkinsonian symptoms in a pathological addiction-like pattern. A current mood disorder (depression, anxiety, hypomanic state or euphoria), behavioral disorder (excessive gambling, shopping or sexual tendency, aggression, or social isolation) or an altered perception about the effect of medication also have to be present. A questionnaire on the typical symptoms of DDS has also been developed and can help in the diagnosis process. Prevention
The main prevention measure proposed is the prescription of the lowest possible dose of dopamine replacement therapy to individuals at risk. The minimization of the use of dopamine agonists, and of short duration formulations of L-Dopa can also decrease risk of the syndrome. Management
First choice management measure consists in the enforcement of a dopaminergic drug dosage reduction. If this decrease is maintained, dysregulation syndrome features soon decrease. Cessation of dopamine agonists therapy may also be of use. Some behavioral characteristics may respond to psychotherapy; and social support is important to control risk factors. In some cases antipsychotic drugs may also be of use in the presence of psychosis, aggression, gambling or hypersexuality.Based upon five case reports, valproic acid may have efficacy in controlling the symptoms of levodopa-induced DDS that arise from the use of levodopa for the treatment of Parkinsons disease. Epidemiology
DDS is not common among PD patients. Prevalence may be around 4%. Its prevalence is higher among males with an early onset of the disease.