Neurology & Pain Management Coding Alert

Coding symptoms can yield proper reimbursement.

Where can you find the ICD-9 code for the rare neurologic disorder Holmes-Gang Syndrome? In this case -- like many rare conditions -- your only choice is to code symptoms and/or the associated conditions.

In 1984, two physicians reported in the American Journal of Medical Genetics on an infant who died at six months. The baby had manifestations which included microcephaly, a large anterior fontanel, short nose with anteverted nostrils, club foot deformity, retarded psychomotor development, and, in the index case only, kidney hypoplasia and dysplasia, according to one of the authors, Lewis B. Holmes, MD, unit chief of genetics and director of genetic counseling and screening services at Vincent Obstetrics and Gynecology in Boston. This Xlinked mental retardation (XLMR) syndrome was named after the physicians: Holmes-Gang Syndrome.

Learn the Details of Rare Diseases and Disorders

Holmes-Gang syndrome is a rare inherited disorder characterized by moderate to severe mental retardation, microcephaly (small head), club feet, and facial and skull abnormalities. The condition is inherited in an X-linked manner, so only males present with the full severity of the symptoms. Female carriers may be mildly symptomatic or have no symptoms at all.

As is frequently the case with many of the eponym syndromes such as Holmes-Gang Syndrome, there currently are no specific ICD-9 codes representing the specific syndrome. What providers will need to report are diagnosis codes that describe the symptoms or conditions associated with the syndrome.

Some degree of genital abnormality is common, reported R. Curtis Rogers, MD, at the 2009 American College of Medical Genetics conference. The prevalence is about 80 percent, he says. Additionally, according to Rogers, who is director of Greenwood Genetic Centers Greenville, S.C. office:

" 90 percent of affected individuals will have hematological signs of alpha thalassemia (282.49)

" 90 percent will have skeletal abnormalities (754.xx)

" 75 percent will have microcephaly (742.1)

" 30 percent will experience seizures (779.0, 780.39)

" 20 percent will suffer cardiac defects (745.xx)

" 15 percent will have renal/urinary abnormalities (753.xx).

The infant mentioned by Holmes and his co-author died of renal complications, so having the complete picture is crucial.

Code Symptoms and Signs to Give Accurate Report

Once you know the details of how the disorder manifests, and the symptoms your neurologist is treating, your coding process should be much simpler. For instance, potential ICD-9 codes for the Holmes-Gang Syndrome include:

" 759.89 -- Other congenital malformation syndromes affecting multiple systems, not elsewhere classified

" 317-319 -- Mental retardation

" 742.1 -- Microcephalus

" 754.51 -- Talipes equinovaruss

" 754.0 -- Certain congenital musculoskeletal deformities of skull, face, and jaw.

Be Aware of Synonomous Conditions

X-linked alpha thalassaemia/mental retardation syndrome, also called ATR-X syndrome, is one of many genetic conditions that can lead to learning difficulties.

The gene involved in the ATR-X syndrome (XNP/ATR-X) has been found to be responsible for several other syndromes, including Holmes-Gang, as well as Juberg-Marsidi, Carpenter-Waziri, and Smith-Fineman-Myers syndromes, as well as one form of XLMR with spastic paraplegia (334.1). All these syndromes, now designated as the XLMR-hypotonic facies syndromes, have characteristic facial dysmorphology and severe mental retardation. Codes like 759.89 may come into play again, but each syndrome will have its own particular set of symptoms or associated conditions, so keep your eyes peeled for your neurologists documentation specifics.