Sunday, January 26, 2020

Ricky Gervais - Its a Sneezing Tic


Dear 60 Minutes Australia, Mr. Woolley and Mr. Gervais, 

Its an instructive video for people suffering, and though unintended,  please don't needlessly add to stigma. #Tourette syndrome is not funny!

Most Respectfully, 

Dr Josh

#tourettes
#tics



Yes. Mr Gervais, neurologic problems are not funny. 

Sneezing Tics are uncommon

https://youtu.be/gXJwHwZ3oi0

Asperger was a Nazi who killed children.

How about we stop honoring this Nazi collaborator and murderer? The Holocaust could not have happened without the collaboration of physicians who believed in  lebensunwertes Leben or “ life unworthy of life,”

Asperger
University of Vienna’s Children’s Hospital
where Asperger worked 
 ”...protected children he deemed intelligent. But he also referred several children to Vienna’s Am Spiegelgrund clinic, which he undoubtedly knew was a centre of ‘child euthanasia’, part of what was later called Aktion T4. “


https://www.nature.com/articles/d41586-018-05112-1

Saturday, December 28, 2019

CBD without THC and Children? What can a parent do?

How does one pick a CBD product?

There are many people or families who want to try CBD without THC.

Keep in mind that these products are as regulated as your coffee.

Here is a reasonable pop article on the topic Link here

FDA tests CBD products and sends warning letters.

http://www.fda.gov/news-events/public-health-focus/warning-letters-and-test-results-cannabidiol-related-products

I have listed some criteria that seem important:
  • Compliance WITH All Laws 
  • Transparency in labeling (Can I calculate how many mg of CBD per ml)
  • Testing by a third party (Can I verify dosing)
  • Neutral taste or at least tolerable taste?
  • Economy (how many cents per mg CBD?)
CBD WITHOUT THC? Here is one example of a CBD that is:
1) Open about test results
2) Neutral Taste
3) Economical. #veterandiscount

One CBD Example

“It is important to note that CBD products are not approved by FDA for the diagnosis, cure, mitigation, treatment, or prevention of any disease. Consumers should beware purchasing and using any such products.” ... From FDA

Monday, December 09, 2019

Vayarin is Found! Its...Called Zoom

OK. Zoom is vayarin. Zoom is distributed in Israel by Teva. 
I ordered some from Israel. Indeed,  it works (and it smells) like good old vayarin. I read the ingredients and the box and it appears to be the same (except in Hebrew). 

So I called the pharmacy below and met Jeff (he’s Canadian).

Zoom is Vayarin. Free shipping details on my blog.
Jeff will send Vayarin to you if you send your prescription to the contacts below (a medical food, remember).

Use the discount code DrJosh” - they will ship a 3 month order for free.

(Btw ... I get no special payment from anyone ... except the thrill of having a discount code with my name). by fax and by email. The prescription must be faxed (toll-free Fax 
  1. 1-866-544-8993) or emailed (rx@israelpharm.com) to us.

Tuesday, December 03, 2019

Adult Neurology Disorders and Low THC CBD in Texas

Adult Neurology Disorders Qualifying for THC Cannabis - Altzheimer Parkinsons CTE

The rule is published and effective 5 Dec 2019 


§1.61. Incurable Neurodegenerative Diseases.
(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) Freidrich’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-Strausller-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.

Pediatric Neurology Disorders Qualifying for Low THC Cannabis in Texas

Texans... here is the published pediatric  list....  

Low 'THC Cannabis for Pediatric Neurology Disorders






The rule is published and effective 5 Dec 2019 


§1.61. Incurable Neurodegenerative Diseases.
(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:


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-Huttenlodcher 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, encephalmyopathic 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;
                                      (I) 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; and
                                      (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.




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

Wednesday, September 11, 2019

An open letter to DSHS regarding tcup program.


Dear Governor Abbot and Dr. Hellerstedt,

I am a pediatric neurologist and epilepsy specialist practicing in Houston. I have experience with the TCUP for over 1 year and manage over 80 patients on cannabanoids (including Epidiolex). I am also a parent of a child with a neurogenetic disorder.

Today the DSHS will take public comment on a puzzling list of selected conditions where medical cannabis may be allowed in Texas.

While well-intentioned, the "list" appears to be far from ready to deploy. 
  •  Many illnesses are inexplicably omitted. One glaring omission in my 1 minute review is  ataxias. What is the evidentiary basis for inclusion the list?
  • Pediatric illnesses such as SMA and muscular dystrophies are listed under "Incurable Neurodegenerative Diseases with Adult Onset". Does the adult-pediatric division on the list imply that people with SMA (a pediatric illness) cannot get CBD until they are adults?  The distinction makes no biologic sense.
  • The specificity of this list is problematic. The era of genetic sequencing has opened our eyes to thousands of low denominator disorders that are progressive and incurable.  New conditions are genetically defined rapidly with daily changes. How often will DSHS update this list to keep pace? 
  • Many progressive degenerative disorders are still NOT diagnosed at a level of specificity demanded here.  For instance, people can have a leukodystrophy without a specific diagnosis.   There is no test for these conditions that, in sum, are not rare. If doctors cannot come to a genetic diagnosis, are patients not allowed to seek relief ? Do you really want to commit staff to review charts or trust the doctors?
  • The decision to treat should be one between a doctor and patient.  Conceptually, one wonders why DSHS is deciding that a person with Refsum disease is meritorious but not one with Rett Syndrome? 
Ultimately,  from a policy perspective the list will usher a blizzard of “what about me” requests and an understandable outpouring of accusations of arbitrary insensitivity.

Further, the list is an assault on the practice of medicine. Physicians can prescribe narcotics, chemotherapy and many other toxic agent. But, it seems odd that government will decide who has an incurable neurodegenerative disorder?

Perhaps the list should include a general statement allowing a physician to decide e.g. “All Other neurologic disorders of  genetic, inflammatory, oncologic, metabolic, vascular and/ or idiopathic etiology”? 

Dr. Rotenberg