Classical Pantothenate Kinase-Associated Neurodegeneration (PKAN)

Welcome to the PKAN Road Map

Pantothenate Kinase-Associated Neurodegeneration (PKAN), a form of Neurodegeneration. Neurodegeneration with Brain Iron Accumulation (NBIA) develops when iron accumulates in the brain resulting in nerve damage and other debilitating symptoms. PKAN is the most common form of NBIA and 30 to 50% of MBIA patients are diagnosed with PKAN.

Also referred to as:

  • Hallervorden-Spatz Syndrome
  • HARP
  • HSS
  • NBIA1
  • neurodegeneration with brain iron accumulation type 1
  • Pigmentary Degeneration of Globus Pallidus, Substantia Nigra, Red Nucleus


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Symptoms include dystonia, dysarthria, rigidity, spasticity, hyperreflexia and extensor toe signs


Symptoms typically begin in childhood and are progressive, often resulting in death by early adulthood. Symptoms of PKAN begin before middle childhood, and most often are noticed before ten years of age. Symptoms include:

  • dystonia (repetitive uncontrollable muscle contractions that may cause jerking or twisting of certain muscle groups)
  • dysphagia & dysarthria due to muscle groups involved in speech being involved
  • rigidity/stiffness of limbs
  • tremor
  • writhing movements
  • dementia
  • spasticity
  • weakness
  • seizures (rare)
  • toe walking
  • retinitis pigmentosa, another degenerative disease that affects the individual’s retina, often causing alteration of retinal color and progressive deterioration of the retina at first causing night blindness and later resulting in a complete loss of vision
Affected Population:

PKAN affects one in 1 million people and is caused by genetic defects.



PKAN is inherited and is called an autosomal recessive disorder.


Prenatal Testing:

If the disease-causing mutations have been identified in the family, prenatal diagnosis for pregnancies can be done by analyzing DNA extracted from fetal cells in amniocentesis or chorionic villus sampling.

PKAN is suspected when magnetic resonance imaging (MRI) changes are seen in an individual with symptoms typically seen in this disease.

All individuals with PKAN have high levels of brain iron, mainly in the globus pallidus. PKAN has a unique characteristic seen on an MRI. Iron accumulation generally makes the brain look dark on certain (T2-weighted) MRI views. In PKAN, this dark area has a very bright spot in the center, called the ‘eye of the tiger sign. It is rarely seen in other forms of NBIA.

The sign sometimes is absent in the early stages of disease. In the Dominican Republic, where 21 affected individuals have been diagnosed with PKAN and have the same PANK2 mutation, it has been reported that six individuals lacked the ‘eye of the tiger sign’ despite their similarities to others in this group.

Some cases with a purported ‘eye of the tiger’ sign will be found to have Mitochondrial-membrane Protein-Associated Neurodegeneration or MPAN, a different form of NBIA that can look similar to PKAN when comparing scans.

Another hallmark feature is the presence of a movement disorder, including one or more of the following: dystonia, rigidity or choreoanthetosis (twisting and writhing). Other common features include corticospinal tract involvement which is responsible for conducting impulses from the brain to the spinal cord, extensor toe signs that indicate damage to the central nervous system and spasticity, along with retinal degeneration or optic atrophy. Seizures are rare.

MRI scans may reveal the eye of the tiger sign, an abnormal low T2 signal (due to abnormal accumulation of iron) in the globus pallidus.

Symptomatic Treatment:

  • Benztropine for rigidity and tremor
  • Dopamine
  • Baclofen for spasm relief and reduced dystonia
  • Chelating agents for excess iron removal


  • Speech therapy


  • Gastrostomy feeding tube placement for advanced dysphagia

PKAN Treatment Updates


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Contact these non-profit organizations to learn more about PKAN and NBIA disorders:

Dana Jones Foundation



Spoonbill Foundation


View all

New publication BRAINCURE for PKAN by Prof. Sánchez Alcázar … Precision medicine in PKAN …

ENACH Announcement Video

NBIA Disorder Isabel Valle

PKAN Updates

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  • PureK Virtually Opens The Market 12 Feb 2021 12:58 TORONTO, Feb. 11, 2021 (Canada NewsWire via COMTEX) -- Kathy Casey, Chief Executive Officer, PureK Holdings Corp. (TSXV: PKAN) (or the "Company"), and her team joined Arne Gulstene, Head, Company Services, TMX Group, to celebrate the Company' …
  • The Week In Cannabis: Stocks Up By Double-Digits On Jazz Pharma's GWPH Acquisition, Potential Federal Moves 06 Feb 2021 03:04 Benzinga Cannabis stocks had one of their strongest weeks in history. Jazz Pharmaceuticals PLC (NASDAQ:JAZZ) acquired GW Pharmaceuticals PLC- ADR (NASDAQ:GWPH) for $7.2 billion, or $6.7 billion excluding GW Pharma's cash. The price of $220 per ADS in cash and …
  • CBD Consumer Products Company PureK To Acquire Skincare Brand No B.S. Life 01 Feb 2021 20:41 Benzinga PureK Holdings Corp‎. (TSX:PKAN) is taking over skincare and beauty company No B.S. Life LLC. Under the deal, the Vancouver-based company agreed to pay $500,000 in cash, plus a refundable deposit in the amount of $1.5 million. PureK will pay an additional …
  • The Week In Cannabis: Stocks Spike By Double Digits On Earnings, Strong Sales, Financings 16 Jan 2021 15:07 Benzinga Cannabis stocks widely outperformed the markets after a slew of financing deals and strong earnings reports. “The cannabis industry is off to a great start this year with New York state pushing hard for legalization," Green Market Report editor-in- …
  • Retrophin Inc Shares Up 35.9% Since SmarTrend's Buy Recommendation (RTRX) 12 Jan 2021 07:00 Jan 12, 2021 (SmarTrend(R) Spotlight via COMTEX) -- SmarTrend identified an Uptrend for Retrophin Inc (NASDAQ:RTRX) on October 6th, 2020 at $20.36. In approximately 3 months, Retrophin Inc has returned 35.89% as of today's recent price of $27.66. …
  • PureK To Acquire BudaPets All-Natural Pet Wellness Brand 11 Jan 2021 17:50 Benzinga PureK Holdings Corp‎. (TSXV:PKAN) plans to acquire the BudaPets brand. The Vancouver company will purchase Nirvana Group LLC, a Florida-based producer and distributor of all-natural pet wellness products.  Under the deal, PureK agreed to issue $1.5 million …
  • Jeff Yauck Announces Filing of Early Warning Report Related to Disposition of Common Shares of PureK Holdings Corp. 31 Dec 2020 23:29 VANCOUVER, British Columbia, Dec. 31, 2020 (GLOBE NEWSWIRE) -- On December 29, 2020, Jeff Yauck (the "Acquiror"), with an address care of PureK Holdings Corp. located at ‎6710 N Scottsdale Road‎, Paradise Valley, Arizona, 85253‎ United States, …
  • PureK Holdings Corp. Announces Completion of Qualifying Transaction 08 Dec 2020 22:18 EIN Presswire PHOENIX, Dec. 08, 2020 (GLOBE NEWSWIRE) -- PureK Holdings Corp‎. (the "Company") (TSX Venture: PKAN), formerly AF1 Capital Corp., is pleased to announce that it has completed its previously announced qualifying transaction (the “Qualifying …
  • Uptrend Call Working As Retrophin Inc Stock Rises 17.7% (RTRX) 23 Nov 2020 19:22 Nov 23, 2020 (SmarTrend(R) Spotlight via COMTEX) -- SmarTrend identified an Uptrend for Retrophin Inc (NASDAQ:RTRX) on October 8th, 2020 at $20.95. In approximately 2 months, Retrophin Inc has returned 17.71% as of today's recent price of $24.66. Over …
  • Mitochondria control cells using their own complete fatty acid synthesis machine 31 Aug 2020 11:49 It shouldn't be any secret that mitochondria can make their own fatty acids. The enzymes mitochondria use to do it were discovered decades ago. Unfortunately, only a few individuals among the biologically literate masses have come to appreciate this …
  • 45.2% Return Seen to Date on SmarTrend Retrophin Inc Call (RTRX) 17 Jul 2020 20:07 Jul 17, 2020 (SmarTrend(R) Spotlight via COMTEX) -- SmarTrend identified an Uptrend for Retrophin Inc (NASDAQ:RTRX) on March 26th, 2020 at $14.13. In approximately 4 months, Retrophin Inc has returned 45.20% as of today's recent price of $20.51. In the …
  • Life-saving medicine finally arrives for Morgan County girl 08 Feb 2020 01:10 WTHR MORGAN COUNTY, Ind. (WTHR) - Nick Cragen and his little girl Amelia are waiting for a small package to arrive that promises to have a huge impact on their lives. “Heaven in a box,” Nick called it, the promised cure for a rare disease that is killing his …
  • God-powered fight against PKAN 06 Feb 2020 02:58 McClusky Gazette - North Dakota By ALLAN TINKER The Kulsrud boys and their parents, grandparents and friends have been following the positive battle the family has made against the neurological condition that has challenged the family. Laura Kulsrud offered their story for us this week. …
  • 5 Beaten-Down Biotech Stocks Set To Rebound In 2020 18 Dec 2019 14:46 The Zacks Medical-Biomedical and Genetics industry is up 8.6% this year so far compared with the S&P 500’s increase of 26.1%. The industry includes large as well as small biotech companies, which are much riskier investment bets than drug companies …
  • Retrophin Reports Third Quarter 2019 Financial Results 30 Oct 2019 20:01 EIN Presswire Enrollment on-track in pivotal Phase 3 clinical trials to support registration of sparsentan for patients with FSGS and IgAN Third quarter revenues rose nine percent to $44 million SAN DIEGO, Oct. 30, 2019 (GLOBE NEWSWIRE) -- Retrophin, Inc. (NASDAQ: RTRX) …
  • Retrophin: Thesis Falls Back To Sparsentan 15 Oct 2019 04:06 Seeking Alpha 2019 was not kind to Retrophin (RTRX). The company made little progress on the commercial front, though it got the new version of Thiola approved by the FDA, and that’s about it on the good news front. On the negative side, the company decided to pass on …
  • Daron Jessop 12 Oct 2019 10:56 St. George News - Utah March 9, 2008 – October 9, 2019 Our beautiful son, Daron Jessop, age 11, passed away in his mother’s arms on October 9, 2019 after a long and courageous battle with PKAN. Daron was born on March 9, 2008 in St. George, Utah, to Noreen Zitting Jessop and …
  • Yayasan Sabah conducts furniture making course 09 Oct 2019 01:44 Borneo Post Loretto with participants of the furniture making course. KENINGAU: Yayasan Sabah Group through Pusat Kraftangan Sabah (PKS) collaborated with Wood Industry Skills Development Centre (WISDEC) under the Malaysian Timber Industry Board of the Ministry of …

Where You Purchase Your Christmas Tree Could Save a Life 

PKAN Treatment Case Studies

Dominican Republic Patients on Compassionate Use Treatment

Cyprus Patient on Compassionate Use

Learn more about the Fort Study here.

Related Terms:

Eye of the Tiger


Iron accumulation

NBIA Disorders


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