Texas Register

TITLE 25 HEALTH SERVICES
PART 1DEPARTMENT OF STATE HEALTH SERVICES
CHAPTER 1MISCELLANEOUS PROVISIONS
SUBCHAPTER DDESIGNATING INCURABLE NEURODEGENERATIVE DISEASES
RULE §1.61Incurable Neurodegenerative Diseases
ISSUE 11/29/2019
ACTION Final/Adopted
Preamble Texas Admin Code Rule

(a)An incurable neurodegenerative disease is a condition, injury, or illness:

  (1)that occurs when nerve cells in the brain or peripheral nervous system lose function over time; and

  (2)for which there is no known cure.

(b)A qualifying physician under Texas Occupations Code, Chapter 169, may prescribe low-THC cannabis to a patient with a documented diagnosis of one or more of the following incurable neurodegenerative diseases:

  (1)Incurable Neurodegenerative Diseases with Adult Onset:

    (A)Motor Neuron Disease:

      (i)Amyotrophic lateral sclerosis;

      (ii)Spinal-bulbar muscular atrophy; and

      (iii)Spinal Muscular Atrophy.

    (B)Muscular Dystrophies:

      (i)Duchenne Muscular Dystrophy;

      (ii)Central Core; and

      (iii)Facioscapulohumeral Muscular Dystrophy.

    (C)Freidreich's Ataxia.

    (D)Vascular dementia.

    (E)Charcot Marie Tooth and related hereditary neuropathies.

    (F)Spinocerebellar ataxia.

    (G)Familial Spastic Paraplegia.

    (H)Progressive dystonias DYT genes 1 through 20.

    (I)Progressive Choreas: Huntington's Disease.

    (J)Amyloidoses:

      (i)Alzheimer's Disease;

      (ii)Prion Diseases:

        (I)Creutzfeldt-Jakob Disease;

        (II)Gerstmann-Straussler-Scheinker Disease;

        (III)Familial or Sporadic Fatal Insomnia; and

        (IV)Kuru.

    (K)Tauopathies.

      (i)Chronic Traumatic Encephalopathy:

      (ii)Pick Disease;

      (iii)Globular Glial Tauopathy;

      (iv)Corticobasal Degeneration;

      (v)Progressive Supranuclear Palsy;

      (vi)Argyrophilic Grain Disease;

      (vii)Neurofibrillary Tangle dementia, also known as Primary Age-related Tauopathy; and

      (viii)Frontotemporal dementia and parkinsonism linked to chromosome 17 caused by mutations in MAPT gene.

    (L)Synucleinopathies:

      (i)Lewy Body Disorders:

        (I)Dementia with Lewy Bodies; and

        (II)Parkinson's Disease; and

      (ii)Multiple System Atrophy.

    (M)Transactive response DNA-binding protein-43 (TDP-43) Proteinopathies:

      (i)Frontotemporal Lobar Degeneration;

      (ii)Primary Lateral Sclerosis; and

      (iii)Progressive Muscular Atrophy.

  (2)Incurable Neurodegenerative Diseases with Pediatric Onset:

    (A)Mitochondrial Conditions:

      (i)Kearn Sayers Syndrome;

      (ii)Mitochondrial Encephalopathy Ragged Red Fiber;

      (iii)Mitochondrial Encephalopathy Lactic Acidosis Stroke;

      (iv)Neuropathy, Ataxia, and Retinitis Pigmentosa;

      (v)Mitochondrial neurogastrointestinal encephalopathy;

      (vi)Polymerase G Related Disorders:

        (I)Alpers-Huttenlocher syndrome;

        (II)Childhood Myocerebrohepatopathy spectrum;

        (III)Myoclonic epilepsy myopathy sensory ataxia; and

        (IV)Ataxia neuropathy spectrum;

      (vii)Subacute necrotizing encephalopathy, also known as Leigh syndrome;

      (viii)Respiratory chain disorders complex 1 through 4 defects: Co Q biosynthesis defects;

      (ix)Thymidine Kinase;

      (x)Mitochondrial Depletion syndromes types 1 through 14:

        (I)Deoxyguanisine kinase deficiency;

        (II)SUCLG1-related mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria; and

        (III)RRM2B-related mitochondrial disease.

    (B)Creatine Disorders:

      (i)Guanidinoacetate methytransferase deficiency;

      (ii)L-Arginine/glycine amidinotransferase deficiency; and

      (iii)Creatine Transporter Defect, also known as SLC 6A8.

    (C)Neurotransmitter defects:

      (i)Segawa Diease, also known as Dopamine Responsive Dystonia;

      (ii)Guanosine triphosphate cyclohydrolase deficiency;

      (iii)Aromatic L-amino acid decarboxylase deficiency;

      (iv)Monoamine oxidase deficiency;

      (v)Biopterin Defects:

        (I)Pyruvoyl-tetahydropterin synthase;

        (II)Sepiapterin reductase;

        (III)Dihydropteridine reductase; and

        (IV)Pterin-4-carbinolamine dehydratase.

    (D)Congenital Disorders of Glycosylation.

    (E)Lysosomal Storage Diseases:

      (i)Mucopolysaccaridosis:

        (I)Mucopolysaccharidosis Type I, also known as Hurler Syndrome or Scheie Syndrome;

        (II)Mucopolysaccharidosis Type II, also known as Hunter Syndrome;

        (III)Mucopolysaccharidosis Type III, also known as Sanfilippo A and B;

        (IV)Mucopolysaccharidosis Type IV, also known as Maroteaux-Lamy; and

        (V)Mucopolysaccharidosis Type VII, also known as Sly.

      (ii)Oligosaccharidoses:

        (I)Mannosidosis;

        (II)Alpha-fucosidosis;

        (III)Galactosialidosis;

        (IV)Asparylglucosaminuria;

        (V)Schindler; and

        (VI)Sialidosis;

      (iii)Mucolipidoses:

        (I)Mucolipidoses Type II, also known as Inclusion Cell disease; and

        (II)Mucolipidoses Type III, also known as pseudo-Hurler polydystrophy;

      (iv)Sphingolipidoses:

        (I)Gaucher Type 2 and Type 3;

        (II)Neimann Pick Type A and B;

        (III)Neimann Pick Type C;

        (IV)Krabbe;

        (V)GM1 gangliosidosis;

        (VI)GM2 gangliosidosis also known as Tay-sachs and Sandhoff Disease;

        (VII)Metachromatic leukodystrophy;

        (VIII)Neuronal ceroid lipofuscinosis types 1-10 including Batten Disease; and

        (IX)Farber Disease; and

      (v)Glycogen Storage-Lysosomal: Pompe Disease.

    (F)Peroxisomal Disorders:

      (i)X-linked adrenoleukodystrophy;

      (ii)Peroxisomal biosynthesis defects:

        (I)Zellweger syndrome:

        (II)Neonatal Adrenoleukodystrophy; and

      (iii)D Bidirectional enzyme deficiency.

    (G)Leukodystrophy:

      (i)Canavan disease;

      (ii)Pelizaeus-Merzbacher disease;

      (iii)Alexander disease;

      (iv)Multiple Sulfatase deficiency;

      (v)Polyol disorders;

      (vi)Glycine encephalopathy, also known as non-ketotic hyperglycinemia;

      (vii)Maple Syrup Urine Disease;

      (viii)Homocysteine re-methylation defects;

      (ix)Methylenetetrahydrofolate reductase deficiency severe variant;

      (x)L-2-hydroxyglutaric aciduria;

      (xi)Glutaric acidemia type 1;

      (xii)3-hydroxy-3-methylglutaryl-CoA lyase deficiency;

      (xiii)Galactosemia;

      (xiv)Manosidosis alpha and beta;

      (xv)Salidosis;

      (xvi)Peripheral neuropathy types 1 through 4;

      (xvii)Pyruvate Dehydrogenase Deficiency;

      (xviii)Pyruvate Carboxylase Deficiency;

      (xix)Refsum Disease; and

      (xx)Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy.

    (H)Fatty Acid Oxidation:

      (i)Trifunctional protein deficiency; and

      (ii)Long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency.

    (I)Metal Metabolism:

      (i)Wilson Disease;

      (ii)Pantothenate Kinase Associated Neurodegeneration; and

      (iii)Neurodegeneration with brain iron accumulation.

    (J)Purine and Pyrimidine Defects:

      (i)Adenylosuccinate synthase Deficiency;

      (ii)5-aminoimidazole-4-carboxamide ribonucleotide transformylase deficiency;

      (iii)Hypoxanthine-guanine phosophoribosyltransferase Deficiency also known as Lesch-Nyhan disease;

      (iv)Dihydropyrimidine dehydrogenase Deficiency; and

      (v)Dihydropirimidinase Deficiency.

(c)A treating physician of a patient suffering from an incurable neurodegenerative disease not listed in subsection (b) of this section may submit a request to the department to have a disease added.

(d)A request under subsection (c) of this section shall be submitted to the department on a form prescribed by the department, which can be found on the department's website at https://www.dshs.texas.gov/chronic/default.shtm.

(e)After review of the submitted documentation, the department may request additional information or make a determination.

The agency certifies that legal counsel has reviewed the adoption and found it to be a valid exercise of the agency's legal authority.

Filed with the Office of the Secretary of State on November 15, 2019

TRD-201904262

Barbara L. Klein

General Counsel

Department of State Health Services

Effective date: December 5, 2019

Proposal publication date: September 27, 2019

For further information, please call: (512) 776-3829



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