ANNUAL REPORT  
2023  
Orphazyme A/S  
Company registration no.: 32266355  
Lyskær 8A, DK-2730  
Herlev, Denmark  
 
Contents  
Management Review ........................................................................................................... 3  
State of Business.............................................................................................................................. 4  
Key Figures & Ratios......................................................................................................................... 5  
2023 Financial Review....................................................................................................................... 6  
Outlook........................................................................................................................................... 6  
Shareholder Information ................................................................................................................... 7  
Corporate Governance ...................................................................................................................... 8  
Risk Management............................................................................................................................10  
Corporate Social Responsibility..........................................................................................................10  
Data Ethics.....................................................................................................................................12  
Diversity in Management Positions.....................................................................................................12  
Board of Directors ...........................................................................................................................13  
Corporate information......................................................................................................................14  
Financial Statements......................................................................................................... 15  
2023 Financial Statements................................................................................................................16  
Notes to Financial Statements...........................................................................................................20  
Statements by Board of Directors and Executive Management...............................................................44  
Independent Auditors’ Report............................................................................................................45  
2
 
Management Review  
3
 
State of Business  
In February 2022, Orphazyme announced that it had been notified by the Committee for Medicinal Products for  
Human Use (CHMP) of the European Medicines Agency (EMA) of a negative trend vote as part of the ongoing  
review of the Marketing Authorisation Application (MAA) for its investigational product candidate, arimoclomol,  
for the treatment of Niemann-Pick disease type C (NPC).  
In May 2022, the Company announced that it had signed an agreement to sell substantially all of the  
Company’s assets and business activities, including those relating to the development and approval of  
arimoclomol and the full claw back liability related to the French early access program, to Zevra Denmark A/S,  
a wholly owned subsidiary of Zevra Inc. now known as Zevra Therapeutics (Zevra) for a total of USD 12.8  
million in cash and assumed liabilities estimated to equal approximately USD 5.2 million (the Sale of Assets).  
Under the terms of the agreement, Zevra agreed to acquire substantially all of Orphazyme’s assets and  
business activities, including those relating to the development and approval of arimoclomol, retain a majority  
of Orphazyme’s remaining Danish employees, continue the early access programs with arimoclomol and pursue  
the potential approval of arimoclomol as a treatment option for NPC.  
Since the Sale of Assets, the focus has been to ensure the smooth transition of assets to Zevra. Further, the  
Company has also continued to assist its legal representatives with a putative class action lawsuit in the United  
States.  
In 2022, the business operations and activities that were part of the Sale of Assets agreement with Zevra were  
reclassified as discontinued operations, but in 2023 all activities and financials are considered related to  
continuing operations.  
At the Annual General Meeting in May 2023, the Board of Directors withdraw their positions and was replaced  
by Michael Hove (Chairman), Jakob Have and Jakob Bendtsen as board members. In May 2023, it was  
announced that the board of directors and CEO/CFO Anders Fink Vadsholt had entered into a severance  
agreement pursuant to which Mr. Vadsholt would step down as CEO/CFO of the Company end of September  
2023, at which point it was announced that the Company had signed an CEO agreement with Jakob Bendtsen  
as the new CEO of the company. Jakob Bendtsen is also continuing in his role as board member.  
On July 9, 2021, a putative class action lawsuit was filed against the Company and certain of its current and  
former directors and officers in the United States District Court for the Northern District of Illinois. The lawsuit  
alleged that certain representations about arimoclomol in the Company’s U.S. IPO offering documents and in  
subsequent public statements were false and misleading, in violations of U.S. securities. While the  
Management at the time did not believe the class action lawsuit claims had any merit, the Company decided,  
for cost control reasons and because of the risks inherent in any litigation, to engage in settlement discussions.  
In May 2023, Orphazyme agreed with the plaintiffs on the US Security class action case to settle the case by  
Orphazyme paying a settlement amount. A written settlement document was filed by the parties to the Court  
which was approved in October 2023. Following the approval there has been a general 100-day objection  
period, where the consortia behind the class action could object to the settlement amount. The court did not  
receive any objections and the case was finally closed early February 2024. The settlement amount was USD  
2,5 million of which USD 0,5 million was covered by insurance leaving a net amount of USD 2 million which  
was paid in November 2023.  
The Company is in process to liquidate its subsidiaries in US and Switzerland and expect that the liquidation  
will be finalized in H1 2024. Following these liquidations, the Company will only consist of the Danish parent  
company.  
The final parts of the remaining activities have been handed over to Zevra, however the Company believes it is  
entitled to receive additional compensation for expenses incurred in the level of DKK 4.5 million. The company  
is currently investigating options to recover the amount from Zevra.  
The Company has carried out a number of initiatives to strengthen the capital structure and liquidity of the  
Company which includes a reverse stock split and capital reductions during November 2023 until January 2024.  
4
 
Please see Company Announcement 2/2024 for more information. The Company is still considering further  
initiatives to strengthen the capital structure including raising new capital.  
The Board has evaluated the Company's position, cash holdings and purpose and, as an extension of this, has  
decided that the primary strategy going forward for the company will be to successively raise capital to become  
a strategic investor in one or more partnerships and/or enter into a collaboration with either unlisted or listed  
biotech companies, where the company in partnership with the management will use its skills to develop the  
values in the companies.  
Throughout 2023, the board held meetings with various companies with a view to carrying out investments in  
biotech activities, including potential to entering into partnerships or collaboration with other companies where  
the Company could become a strategic co-owner. In February 2024 the Company announced the first  
investment in the Swedish listed company CombiGene AB via the purchase of 10 % of the shares in the  
company.  
The strategy will continuously require capital, and it may also be possible to issue new shares in Orphazyme as  
part of the payment that must be invested to become an active co-owner/partner in the selected companies.  
It is the board's preliminary assessment that this will best enable us to create value for the company's  
shareholders.  
Key Figures & Ratios  
TDKK  
2023  
2022  
2021  
2020  
2019  
Operating Loss  
(27,041)  
(41,241)  
(83,472)  
(608,534)  
(335,954)  
Net Financial income/expense  
Net Loss from continuing operations  
Net result from discontinued operations  
Net result for the period  
Total comprehensive profit/ loss  
Total profit/loss per share, basic (DKK)  
Total non-current assets  
Cash  
993  
(26,048)  
-
193  
(38,312)  
64,382  
26,070  
25,165  
713  
37  
(78,495)  
(548,044)  
(626,539)  
(626,841)  
(17,934)  
14,285  
(26,627)  
-
(7,043)  
-
-
-
(26,048)  
(26,048)  
(738)  
(633,246)  
(632,641)  
(22,187)  
38,829  
726,929  
56,735  
822,493  
34,698  
620,525  
166,627  
(337,497)  
(337,430)  
(16,884)  
32,529  
123,588  
19,137  
180,754  
19,984  
52,969  
65,988  
-
-
11,269  
8,043  
42,464  
15,658  
58,122  
35,312  
41,667  
16,357  
102,255  
56,689  
Other current assets  
Total assets  
19,312  
12,283  
14,242  
5,070  
173,229  
34,952  
Share capital  
Total equity  
9,339  
Total current liabilities  
129,092  
Cash flow from operating activities  
Cash flow from investing activities  
Cash flow from financing activities  
Net cash flow from discontinued operations  
(23,870)  
(6,944)  
(685)  
-
(117,945)  
90,347  
(602,571)  
46  
(539,076)  
(5,101)  
1,159,422  
-
(326,818)  
(3,285)  
58,939  
-
(35,078)  
32,862  
(30,344)  
(549,447)  
Share Price (DKK)1  
1,330  
35,312  
47.0  
74%  
403  
888  
35,312  
31.1  
68%  
1,180  
21  
17,160  
34,952  
600  
67,100  
28,514  
1,913  
75%  
72,400  
19,985  
1,447  
29%  
2,650  
74  
Total outstanding shares1  
Market capitalization (MDKK)2  
Equity ratio3  
5%  
Equity per share (DKK)4  
Average number of employees  
Number employees at year end  
267  
21,762  
117  
1
130  
1
1
62  
141  
86  
(1) Comparison figures for total outstanding shares and share price for 2019-2022 is adjusted for the reverse stock split in November 2023. (2)  
Market cap is calculated as the share price multiplied with the total outstanding shares as of the balance sheet date; (3) Equity ratio is calculated as  
the equity divided by the total assets as of the balance sheet date; (4) Equity per share is calculated as the total equity divided by the total  
outstanding shares as of the balance sheet date.  
Key figures & ratios for 2022 and 2021 are presented with a split of continuing and discontinued operations for P&L and cash flow to reflect  
restructuring and the Zevra transaction. 2020 and 2019 figures have not been restated since they represent a significantly different stage of the  
business and such presentation is not meaningful to an understanding of Orphazyme’s current business.  
5
 
2023 Financial Review  
Income statement  
The net result for the full year ended December 31, 2023 was an operating loss of DKK 26.0 million compared  
to a net operating loss of DKK 38.3 million for the same period in 2022 from continuing operations.  
The operational loss for 2023 was realized at DKK 27.0 million which is marginally better than our previously  
communicated outlook of a loss of DKK 30 – 35 million for the period.  
General and administrative (G&A) expenses totaled DKK 27.0 million in 2023, a reduction of approx. DKK 14.2  
million compared to the prior year (DKK 41.2 million in 2022). G&A expenses include costs associated with  
employees and Board of Directors, service providers and external assistance, legal and technology expenses.  
The difference primarily relates to lower costs in 2023 following the Sale of Assets to Zevra. G&A expenses  
from discontinued operations were DKK 59.5 million for the year ended December 31, 2022.  
Net financial income for the financial period ended December 31, 2023, was DKK 690 thousand compared to  
DKK 185 thousand for the same period in 2022. Net financial items from discontinued operations were an  
expense of DKK 5.0 million in 2022.  
Income tax was DKK 0 million compared to DKK 2.8 million in the same period in 2022 which included income  
tax benefits related to tax credits for R&D expenses at the applicable tax rate under the Danish Corporate  
Income Tax Act, which is no longer relevant for the Company due to the limited activities.  
Statement of financial position  
As of December 31, 2023, Orphazyme held cash of DKK 11.3 million as compared to DKK 38.9 million as of  
December 31, 2022. The cash position is marginally better than our previously communicated outlook for cash  
of DKK 6 – 10 million.  
As of December 31, 2023, total equity amounted to DKK 14.2 million compared to DKK 41.0 million as of  
December 31, 2022.  
Cash flows  
Net cash flow from operating activities amounted to an outflow of DKK 23.9 million for the full year ended  
December 31, 2023, compared to an outflow of DKK 102.6 million for the same period in 2022. Net cash flow  
from discontinued operating activities was an outflow of DKK 43.0 million in 2022.  
Net cash flow from investing activities amounted to an outflow of DKK 6.9 million in 2023 (cash inflow of DKK  
90.3 million in 2022).  
Net cash flow from financing activities amounted to an outflow of DKK 0.7 million in 2023 compared to an  
outflow of DKK 35.1 million in 2022.  
Outlook  
For the full-year 2024 we anticipate an operating loss in the range of DKK 3 – 4 million. There are inherent  
risks and uncertainties in our Outlook for 2024 including the limited nature of our business activities, potential  
investments and our future prospects.  
6
 
Shareholder Information  
Orphazyme’s shares are listed on Nasdaq Copenhagen (since November 16, 2017) under the ticker symbol  
ORPHA.  
We conduct our communications in accordance with the applicable rules and regulations required under Danish,  
and EU laws, including as set forth by the Danish Financial Supervisory Authority.  
As of December 31, 2023, Orphazyme has a total share capital of nominally DKK 12,283,333 divided into  
35,312 shares and representing a total of 35,312 voting rights.  
At the company’s extraordinary general assembly on November 2, 2023, it was decided to implement a reverse  
share split at a consolidation ratio of 1.000:1. The reverse stock split reduced the number of shares in the  
company so 1.000 current shares at a nominal value of 1 DKK was consolidated to 1 new share at a nominal  
value of 1.000 DKK.  
At an extraordinary general meeting on 30 November, 2023, it was adopted to lower the nominal value per  
share from DKK 1,000 to DKK 150. A nominal value per share below the share price after the reverse stock  
split takes effect will enable the board of directors with various options to optimize the capital structure of the  
company under its current authorizations.  
At the same extraordinary general it was also adopted to reduce the share capital with a total nominal value of  
DKK 30,015,200, which included a reduction of nominal DKK 23,028,667 for the purpose of covering losses and  
a reduction of nominal DKK 6,986,533 for the purpose of transfers to a special reserve.  
The reduction of DKK 6,986,533 did not take effect before January 9, 2024, after a four week proclamation  
period.  
As of December 31, 2023, the Company had approximately 1,984 registered shareholders. The Company has  
limited ongoing operational business activities and, as of the date of this annual report, does not expect to  
make dividend payments within the foreseeable future.  
As of the publication of this Annual Report the Company has the below major shareholders:  
-
-
-
-
MH Investment ApS, Dyrehavevej 47, 2930 Klampenborg owns 5,482 shares representing 15.52% of  
total share capital.  
Nordic Compound Invest A/S, Annexstræde 6, 2500 Valby owns 5,482 shares representing 15.52% of  
total share capital.  
Færch B Holding ApS, Stengårds Alle 243, 2860 Søborg owns 3,012 shares representing 8.53% of  
total share capital.  
LSP V Cooperatieve U.A., Johannes Vermeer, Plein 9, 1071 DV Amsterdam, Netherlands owns 2,426  
shares representing 6.87% of total share capital.  
7
 
Corporate Governance  
Orphazyme is committed to ensuring transparent and good corporate governance. As a Danish company listed  
on Nasdaq Copenhagen, Orphazyme is subject to the Danish Recommendations on Corporate Governance. The  
Recommendations on Corporate Governance are best practice guidelines for the management of companies  
admitted to trading on a regulated market.  
Orphazyme complies with the Recommendations on Corporate Governance where deemed relevant given  
Orphazyme’s current situation and focus.  
Orphazyme’s corporate governance statement includes Orphazyme’s position on the Recommendation on  
Corporate Governance as well as a complete list of the Company’s comments to recommendations that the  
Company opted to deviate from.The corporate governance statement is available under “Corporate  
Governance” in the Investors & Media section of our website: investors.orphazyme.com/corporate-governance.  
Board of Directors  
The Board of Directors is responsible for the overall management and strategic direction of Orphazyme’s  
business and operations and it supervises the Company’s activities, management, and organization. The Board  
of Directors appoints and dismisses the members of the Executive Management, who are responsible for the  
day-to-day management of the Company.  
The Board has chosen that the functions of the Audit Committee are exercised by the entire board of directors  
with Jakob Have as Chairman of the Audit Committee.  
Meetings  
The Board of Directors normally holds at least five regular meetings annually, including a strategy review, plus  
ad-hoc meetings as required. Extraordinary board meetings are convened by the Chairman when necessary or  
when requested by a member of the Board of Directors, a member of the Executive Management, or by the  
Company‘s auditor. There was a high frequency of meetings in 2023 due to the change in management,  
initiatives to strengthen the capital structure of the Company and US lawsuit. The Board of Directors forms a  
quorum when more than half of its members are represented, including the Chairman. Resolutions of the Board  
of Directors are passed by a simple majority of the votes present at the meeting. In the event of equal votes,  
the Chairman shall have the casting vote.  
The members of the Board of Directors elected by the general meeting are elected for a term of one year.  
Members of the Board of Directors may be re-elected.  
Orphazyme Board of Directors  
(1)  
Name  
Position  
Independent  
Year of first  
appointment  
2023  
Expiration of  
term  
Michael Hove  
Jakob Have  
Jakob Bendtsen  
Chairman  
Member  
Member  
Independent  
Independent  
2024  
2024  
2024  
2023  
2023  
Not Independent  
(1) According to the Danish Recommendations on Corporate Governance at least half of the members of the  
Board of Directors should be independent.  
8
 
Internal controls and financial reporting procedures  
The Board of Directors and the Executive Management are responsible for risk management and internal  
controls over its financial reporting and approve general policies in that regard. The Board of Directors is  
overseeing the reporting process and the most important risks involved in this respect. The Executive  
Management is responsible for the effectiveness of the internal controls and risk management and for the  
implementation of such controls aimed at mitigating the risk associated with the financial reporting.  
The Board of Directors and Executive Management assess risks on an on-going basis, including risks related to  
financial reporting, and assess measures to manage, reduce, or eliminate identified risks.  
Orphazyme has adopted and defined an internal control framework that identifies key processes, inherent  
risks, and control procedures in order to secure appropriate accounting processes. The control procedures  
include a variety of processes in order to prevent any misrepresentation, significant errors, omissions, or  
fraudulent behavior.  
Orphazyme’s independent auditors are appointed for a term of one year by the shareholders at the Company’s  
annual general meeting. The Board of Directors assesses the independence and competencies and other  
matters pertaining to the auditors. The framework for the auditors’ compensation and duties, including audit  
and non-audit tasks, is agreed annually between the Board of Directors and the auditors.  
At the company’s extraordinary general assembly on November 2, 2023, KPMG P/S was elected as new  
auditors replacing EY Godkendt Revisionspartnerselskab.  
9
 
Risk Management  
Our financial situation and risks are assessed on an ongoing basis and reported to the Board of Directors. The  
Company has currently only identified currency risks as relevant for the Company due to the limited ongoing  
business operations of Orphazyme.  
Financial risks may arise from changes in exchange rates. The Company are mostly exposed to foreign  
exchange movements relating to SEK, USD and EUR.  
Corporate Social Responsibility  
This section constitutes the Company’s statutory reporting according to Section 99a of the Danish Financial  
Statements Act.  
In May 2022, substantially all Orphazyme’s assets and business activities were sold to Zevra and we now have  
limited ongoing operational business activities and employees. Our headquarter is in Copenhagen, and we have  
non-operating subsidiaries in the U.S. and Switzerland which are expected to be liquidated in 2024.  
In light of such limited business operations, Orphazyme’s Corporate and Social Responsibility (CSR) risks are  
considered very limited. Our CSR activities for 2023 were commensurate with the size and limited operations of  
the Company, though we continued to strive to uphold our values and responsibilities towards society,  
employees, and our stakeholders. For 2024 we will continue, where possible, to fulfill our CSR obligations as  
we execute our strategy.  
CSR reporting areas  
Human Rights  
Risk: Very Limited: Orphazyme has limited operations, employees and suppliers.  
Actions: Continued to respect internationally declared human rights and did not employ child labor.  
Policies in place: Orphazyme acknowledges and supports the maintenance of internationally declared human  
rights and bases its work on the UN Universal Declaration of Human Rights and the interpretation that it is the  
responsibility of the State to protect, and the companies’ responsibility to respect, these rights. Orphazyme  
interprets human rights to comprise respect for diversity.  
•
Diversity policy.  
•
Whistleblower policy.  
Results:  
•
•
No diversity related incidents or human rights violations reported in 2023.  
Employee composition: Not meaningful given company structure (One employee as of December 31,  
2023; male).  
•
•
Leadership: Not meaningful given company structure (One employee as of December 31, 2023; male).  
The Company has set a target of increasing the representation of women on the Board of Directors to  
40% by 2026 in accordance with the guidelines from the Danish Business Authority. As at year end  
2023 the Board of Directors was comprised of three members, none of which were women.  
FuturePlans:  
•
Continue to support and respect internationally declared human rights and will not employ child labor.  
Aim to improve Board diversity in the future, subject to expansion of current business operations.  
10  
 
Anti-Corruption & Bribery  
Risk: Very Limited: We do not tolerate the use of bribery or corruption to achieve business objectives. Given  
that we have limited operations, employees and suppliers our anti-corruption and bribery risk is very limited.  
Actions: The Company is committed to maintaining the highest standards of conduct and will not tolerate the  
use of bribery or corruption to achieve its business objectives.  
•
•
Anti-corruption and bribery training conducted when employees start.  
Legal & Compliance training refreshers, including anti-corruption and bribery  
Policies in place: Our policies on bribery and corruption are clearly set out in our anti-corruption policy and  
our employee handbook.  
Results: No bribery and corruption violations identified or reported in 2023.  
Future Plans: Continue to maintain the highest standards of conduct and not tolerate the use of bribery or  
corruption to achieve business objectives.  
Environment & Climate  
Risk: Very limited: We have a very limited number of employees, minimal physical office presence and use  
external suppliers for certain activities such as administration, finance and legal activities which we believe  
have a low potential risk for impact on the environment & climate.  
Actions: Followed established procedures both during use and at disposal of hazardous substances.  
Policies in place: Considering the business of the Company, and its limited operations and employees,  
Orphazyme’s general potential impact on the environment and climate and the impact of the climate on  
Orphazyme’s business is viewed as minimal. Applicable rules and procedures were followed regarding the use  
of hazardous substances (no longer relevant due to closure of our laboratory space early in 2022) and we  
continue to endeavor to protect the environment and climate through mindful business practices such as, e.g.  
careful use of office materials and energy consumption.  
Results:  
•
•
•
Continued to keep records of all accidents in 2023.  
Recorded no records of spill of hazardous substances  
Continued to focus on efficient energy use and management of office materials in 2023.  
Future Plans: Orphazyme is no longer active in research and development activities and further with only  
limited operating activities, employees and suppliers focusing on administration, finance and legal activities,  
the general potential impact on the environment and climate and the impact of the environment and climate on  
Orphazyme’s business is viewed as minimal. We will continue to endeavor to protect the environment and  
climate through mindful business practices such as, e.g. careful use of office materials and energy  
consumption.  
Social / Employees  
Risk: Very Limited: As of December 31, 2023, Orphazyme had only one employee. The company continues to  
value diversity in gender, age, ethnicity, nationality, religion, education, sexual orientation, work history,  
perspectives, opinions, and skills at all levels of our business however given its limited employees it currently  
does not have a diverse workforce. Further, the Company’s limited operations, such as office space and  
support network, could impact the working conditions of remaining employees.  
Actions: The health and safety of our employees is of utmost importance and Orphazyme continually works to  
ensure that all systems and processes meet strict international standards. Continued to foster an open,  
trusting and inclusive workplace committed to freedom from discrimination, harassment, and bullying.  
Policies in place: Diversity Policy as well as Health and Safety Policies.  
11  
 
Results: Established a resilient culture centered on trust and collaboration.  
Future Plans: As of the date of this annual report, the Company has limited ongoing business activities and  
only one employee. Our future social / employee activities will be commensurate with the size and limited  
operations of the Company. We will continue to strive to uphold our values and responsibilities and promote a  
healthy, diverse and inclusive workplace, as we execute our strategy.  
Data Ethics  
We currently do not have a data ethics policy. Given the Company has limited operational business activities, it  
is no longer an integrated part of the Company’s business strategy or activities to process data or use  
algorithms for data analysis in connection with clinical trials, etc. However, our practices will be evaluated on  
an ongoing basis in order to ensure they align with the statutory requirements set forth in Section 99d of the  
Danish Financial Statements Act.  
Diversity in Management Positions  
Given the size of our workforce with only one employee, it is not meaningful to set out diversity figures nor is it  
required in accordance with the Danish Companies Act. This is also the case for the information requirements  
in section 107 d in the Danish Companies Act.  
Due to the evolution of the business, there were only three Board members at the end of 2023, all of which  
were men. The company remains committed to promoting a diverse and inclusive workplace and has therefore  
set a target of increasing the representation of women on the Board of Directors to 40% by 2026 in accordance  
with the guidelines from the Danish Business Authority.  
2023  
Board of directors  
Total number of members  
3
0%  
Underrepresented gender in %  
Target figure in % Net result from discontinued operations  
Year of target fulfilment  
40%  
2026  
Other Management levels  
Total number of members (all male)  
1
12  
 
Board of Directors  
Michael Hove, Chairman of the Board  
•
•
•
Member since:  
Born in:  
Nationality:  
2023  
1971  
Danish  
Special competencies: Strategy, Capital structure, MA-competences and turn-around of companies.  
Michael Hove holds a bachelor at CBS from 1995 and several degrees in management.  
Current positions: CEO MH Investment ApS, CEO Scandinavian Investment Company A/S, Chairman Antique  
89 Invest A/S. Large investor/advisor in a number of listed companies.  
Jakob Have  
•
•
•
•
Member since:  
Born in:  
Nationality:  
Committees:  
2023  
1981  
Danish  
Audit (Chair)  
Special competencies: Capital markets, accounting and financial management, tax and M&A.  
Jakob Have has previous experience from positions at various listed companies and holds a cand.merc.aud  
from 2007.  
Current positions: Jakob Have is currently member of the executive management of Nordic Compound Invest  
A/S, Nordic Compound Management A/S and Nordic Compound A/S and a board member of Scandinavian  
Medical Solutions A/S, Scandinavian Investment Company A/S, Nordic Compound A/S and Nordic Compound  
Management A/S.  
Jakob Bendtsen  
•
•
•
Member since:  
Born in:  
Nationality:  
2023  
1978  
Danish  
Special competencies: Compliance, accounting, financial management, tax and M&A.  
Jakob Bendtsen has previous experience from positions at various listed companies and as external consultant  
and advisor and holds a cand.merc.aud from 2004.  
Current positions: Jakob Bendtsen is currently member of the executive management of Færch Family Invest  
and Consulting ApS, Cewijo ApS, Færch B Holding ApS, Færch Ejendomsservice ApS and Accounti ApS.  
Further, Jakob Færch Bendtsen is a member of the board of directors of Nordic Compound Invest A/S.  
13  
 
Executive Management  
Jakob Bendtsen, Chief Executive Officer  
Jakob Bendtsen took the position of Chief Executive Officer, in addition to his role as Board Member, October 1,  
2023.  
Corporate information  
Annual Report  
This annual report will be available on www.orphazyme.com and printed copies are available upon request.  
Annual General Meeting  
Information about our Annual General Meeting can be found in the section for Investors & Media at  
www.orphazyme.com under Investors and General Meetings.  
14  
 
Financial Statements  
15  
 
2023 Financial Statements  
STATEMENTS OF PROFIT OR LOSS AND OTHER COMPREHENSIVE INCOME  
For the years ended December 31  
DKK 000, except per share and share data  
General and administrative expenses  
Note  
2.3  
2023  
(27,041)  
2022  
(41,241)  
Operating loss  
(27,041)  
993  
(41,241)  
185  
Financial income  
2.6  
2.6  
Financial expenses  
—
—
Loss before tax  
(26,048)  
—
(41,056)  
2,750  
Income tax benefit  
2.7  
1.7  
Loss from continuing operations  
Net result from discontinued operations  
(26,048)  
(38,306)  
—
73,378  
Net result for the year  
(26,048)  
35,072  
Items that will be reclassified subsequently to the Statement of  
Profit or Loss:  
Exchange difference from translation of foreign operations  
-
-
(141)  
(141)  
Other comprehensive profit/loss  
Total comprehensive profit/loss  
(26,048)  
34,931  
Weighted-average shares outstanding  
Weighted-average shares outstanding, dilutive  
35,312  
35,518  
35,269  
35,269  
Profit/ loss per share from continuing operations, (DKK)  
Profit/ loss per share from discontinuing operations, (DKK)  
Profit/ loss per share, basic (DKK)  
(738)  
—
(1,086)  
2,081  
995  
4.3  
4.3  
(738)  
(733)  
Profit/ loss per share, diluted (DKK)  
995  
16  
 
STATEMENTS OF FINANCIAL POSITION  
As of December 31,  
DKK 000  
ASSETS  
Current assets  
Note  
2023  
2022  
5,500  
Corporation tax receivable  
Trade receivables  
2.8  
3.5  
3.4  
3.7  
3.7  
—
—
4,103  
8,305  
—
Prepayments and other receivables  
Securities  
1,099  
6,944  
Cash  
11,269  
19,312  
38,918  
56,826  
Total current assets  
Total assets  
19,312  
56,826  
EQUITY AND LIABILITIES  
Equity  
Note  
2023  
2022  
Share capital  
Share premium  
Other reserves  
Retained earnings  
4.2  
3.6  
12,283  
—
—
1,959  
14,242  
35,312  
2,087,437  
(303)  
(2,081,472)  
40,974  
Total equity  
Non-current liabilities  
Other non-current liabilities  
Total non-current liabilities  
Current liabilities  
Trade payables and accruals  
Discount and rebate liabilities  
Other liabilities  
—
—
98  
98  
3.6  
3.6  
3.6  
5,070  
—
9,206  
4,457  
2,090  
—
Total current liabilities  
Total equity and liabilities  
5,070  
19,312  
15,753  
56,826  
17  
 
STATEMENTS OF CHANGES IN SHAREHOLDERS’ EQUITY  
DKK 000  
Share-based  
compensation  
– acquisition  
of intangible  
assets  
Foreign  
currency  
transla.  
reserve  
Share  
capital  
Accumulated  
Deficit  
Share premium  
2,082,487  
Total  
(1,121)  
Balance as of December 31, 2021  
34,952  
(167)  
2,486  
(2,120,884)  
Net loss for the year  
—
—
—
—
—
—
—
(141)  
(141)  
—
—
—
35,072  
—
35,072  
(141)  
Other comprehensive income  
Total other comprehensive income (loss)  
Transactions with owners:  
Capital increase  
35,072  
34,931  
360  
5,091  
—
—
—
—
—
5,451  
(141)  
Transaction cost related to capital increase  
Reserve for bonus shares reversed  
Share-based compensation expense  
Total transactions with owners  
Balance as of December 31, 2022  
Total other comprehensive income (loss)  
Reclassification  
(141)  
—
—
—
—
—
(2,486)  
2,486  
—
—
4,950  
2,087,437  
—
—
—
1,853  
1,853  
7,163  
40,974  
(26,048)  
—
360  
35,312  
—
—
(2,486)  
4,339  
(303)  
—
—
—
—
(2,081,472)  
(26,048)  
(303)  
—
—
303  
Transactions with owners:  
Purchase and sale of shares  
Capital reduction  
—
(23,029)  
—
—
—
—
—
—
—
—
—
—
—
—
—
(684)  
23,029  
(684)  
—
Reclassification  
(2,087,437)  
(2,087,437)  
—
2,087,437  
2,109,782  
1,959  
—
Total transactions with owners  
Balance as of December 31, 2023  
(23,029)  
12,283  
(684)  
14,242  
18  
 
STATEMENTS OF CASH FLOWS  
For the years ended December 31,  
DKK 000  
Note  
2023  
(27,041)  
—
2022  
(41,241)  
Operating result from continuing operations  
Operating result from discontinuing operations  
(67,278)  
Adjustments to reconcile operating result to cash  
flows from operating activities:  
Equity-settled share-based compensation expense  
Depreciation and amortization  
2.5  
—
—
1,773  
782  
2.2,2.3  
3.4, 3.5  
3.6  
Change in prepayments, deposits, and other receivables  
Change in trade payables, accruals, and other liabilities  
Change in intercompany receivables  
7,125  
(10,124)  
41,393  
(37,889)  
4,183  
(657)  
1,117  
(7,794)  
5,500  
Change in intercompany payables  
Corporation taxes received  
Interest received  
5,500  
993  
185  
Interest paid  
—
(1,064)  
(117,945)  
Cash flow from operating activities  
(20,021)  
Investing activities  
Proceeds from sale of property, plant and equipment  
—
—
1,460  
Proceeds from sale of activity  
Purchase of securities  
1.7  
3.2  
88,887  
(6,944)  
—
Cash flow from investing activities  
(6,944)  
90,347  
Financing activities  
—
Repayment of borrowings  
(39,155)  
(1,233)  
5,451  
—
Repayment of lease obligations  
Proceeds from issuance of shares  
Purchase of shares in connection with reverse stock split  
Sale of shares  
—
—
(3,020)  
2,335  
—
—
Transaction cost related to issuance of shares  
(141)  
Cash flow from financing activities  
(685)  
(35,078)  
Net change in cash and cash equivalents  
Effects of changes in exchange rates  
Cash at the beginning of the year  
Cash at the end of the year  
(27,650)  
1
(47,298)  
523  
38,918  
11,269  
85,692  
38,918  
19  
 
Notes to Financial Statements  
SECTION 1  
Basis of preparation and significant accounting policies  
1.1 CORPORATE INFORMATION  
Orphazyme A/S (the “Company”) is headquartered in Copenhagen, Denmark and is publicly traded on Nasdaq  
Copenhagen.  
In April 2018, a wholly-owned subsidiary, Orphazyme U.S., Inc., was incorporated in Delaware, USA and in  
March 2020, a wholly-owned subsidiary, Orphazyme Schweiz GmbH, was incorporated in Zug, Switzerland  
(together with Orphazyme A/S, “Orphazyme” or “the Company”). These companies are currently in the process  
of being dissolved by solvent liquidations.  
Orphazyme was previously involved in the research and development of novel therapeutics for the treatment of  
neurodegenerative rare diseases. In May 2022, Orphazyme sold substantially all of its assets and business  
activities to Zevra who retained all of Orphazyme’s remaining Danish employees. The disposed assets and  
business activities were presented as discontinued operations in 2022.  
As of the date of publication of this annual report, Orphazyme has defined a new strategy and has currently  
entered into a strategic partnership with the Swedish listed company CombiGene AB.  
These financial statements were approved and authorized for issuance by the Board of Directors on March 27,  
2024.  
1.2  
BASIS OF PREPARATION  
The financial statements have been prepared in accordance with International Financial Reporting Standards  
(IFRS) as adopted by the EU and additional requirements of the Danish Financial Statements Act.  
The financial statements have been prepared on a going concern basis and are presented in Danish Kroner, or  
DKK, which is both the functional and presentation currency of the Company. Where indicated, amounts are  
rounded to the nearest thousand.  
The financial statements are prepared based on the concept of materiality, which considers both quantitative  
and qualitative factors. Items that are considered individually significant or are required under the minimum  
presentation requirements of IFRS are presented separately. If items are individually immaterial, they are  
aggregated with other items of similar nature in the financial statements or in the notes.  
1.3  
SIGNIFICANT ACCOUNTING POLICIES  
A detailed description of accounting policies and significant accounting estimates and judgements related to  
specific financial statement line items is presented in each note to the relevant line item. The financial  
statements have been prepared on a historical cost basis except for share-based compensation and securities,  
which is measured at fair value.  
Principles of consolidation  
The financial statements include the financial statements of the parent company, Orphazyme A/S (the “Parent  
Company”), Orphazyme US, Inc. and Orphazyme Schweiz GmbH, fully-owned subsidiaries over which the  
Parent Company has control.  
Pursuant to the exemption clause for the Group in section 110 of the Danish Financial Statements Act, the  
annual report of Orphazyme A/S does not comprise consolidated financial statements.  
Management determined that the Group falls into the reporting Class B in the financial year 2023 and 2022  
applying the size limits per the Danish Financial Statements Act.  
Translation of foreign currencies  
On initial recognition, transactions denominated in foreign currencies are recorded using the foreign exchange  
spot rate at the transaction date. For monetary assets and liabilities, differences arising between the foreign  
exchange spot rates at the transaction date and the date of settlement or period-end exchange rates are  
recognized in the Statement of Profit or Loss as financial income or financial expenses.  
Statement of cash flows  
The statement of cash flows is presented using the indirect method and shows cash flows resulting from  
operating activities, investing activities, financing activities, and the cash at the beginning and end of the year,  
including any effects of exchange rate changes.  
20  
 
Cash flows used in operating activities converts items in the Statement of Profit or Loss from the accrual basis  
of accounting to the cash basis of accounting. Non-cash items such as foreign exchange gains and losses,  
depreciation, amortization, and changes in working capital are reversed from the net result for the year and  
actual cash receipts and payments are included.  
Cash flows from investing activities shows payments related primarily to the purchase of licenses and property,  
plant, and equipment and sale of activity.  
Cash flows from financing activities shows proceeds from share issuance, borrowings, net of transaction costs,  
repayment of debt, and lease payments.  
Discontinued operations  
Discontinued operations in 2022 are excluded from the results of continuing operations and are presented as a  
single amount as profit or loss after tax from discontinued operations in the statement of profit or loss.  
Additional disclosures are provided in Note 1.7. All other notes to the financial statements include amounts for  
continuing operations, unless indicated otherwise.  
Segment information  
The Company is managed and operated as one business unit that is reflected in the internal reporting. No  
separate lines of business or separate business entities have been identified with respect to any product  
candidate or geographical market and no segment information is currently disclosed in the internal reporting.  
1.4 SIGNIFICANT ACCOUNTING ESTIMATES AND JUDGEMENTS  
The use of reasonable estimates and judgements is an essential part of the preparation of the financial  
statements. Given the uncertainties inherent in the Company’s business activities, Management must make  
certain significant accounting estimates and judgements, which affect the application of accounting policies and  
therefore the reported amounts of assets, liabilities, expenses, and disclosures in the consolidated financial  
statements and parent company financial statements. The significant accounting estimates and judgements  
identified are those that have a significant risk of resulting in a material adjustment to the financial statements.  
Management bases its estimates on historical experience, assumptions, and information currently available and  
deemed to be reasonable at the time the financial statements are prepared. However, actual amounts may  
differ from the estimated amounts as more detailed information becomes available. Estimates and assumptions  
are reviewed on an ongoing basis and, if necessary, changes are recognized in the period in which the estimate  
is revised. Management has made significant accounting estimates and judgements in the following areas,  
which are further presented in each note to the relevant financial statement line items:  
•
Estimate of inputs and assumptions used in share-based compensation valuation models (Note  
2.5)  
•
Judgement regarding the recognition of deferred tax assets related to taxable losses to be carried  
forward (Note 2.7)  
Please refer to the specific referenced notes for further information on the significant accounting estimates and  
judgements as well as assumptions applied.  
1.5 NEW IFRS STANDARDS APPLICABLE TO THE COMPANY  
The Company has implemented the standards and amendments that are effective for the financial year 2023.  
The new standards and amendments have not affected the Company’s recognition or measurement for 2023,  
nor are they expected to have significant future impact.  
The IASB has issued a number of new standards and updated some existing standards, which are effective for  
accounting periods beginning January 1, 2024 or later. Therefore, they are not incorporated in these financial  
statements. There are no standards presently known that are not yet effective and that would be expected to  
have a material impact on our current or future reporting periods.  
1.6 SIGNIFICANT EVENTS AFTER THE REPORTING PERIOD  
In May 2023, Orphazyme agreed with the plaintiffs on the US Security class action case to settle the case by  
Orphazyme paying a settlement amount. A written settlement document was filed by the parties to the Court  
which was approved in October 2023. Following the approval there has been a general 100-day objection  
period, where the consortia behind the class action could object to the settlement amount. The court did not  
receive any objections and the case was finally closed early February 2024. The settlement amount was USD  
2,5 million of which USD 0,5 million was covered by insurance leaving a net amount of USD 2 million which  
was already paid in November 2023.  
No other significant events after the reporting period to disclose.  
21  
 
1.7 DISCONTINUED OPERATIONS AND DISPOSALS COMPANY HELD FOR SALE  
In May 2022, Orphazyme announced that it had signed an agreement to sell substantially all of the Company’s  
assets and business activities, including those relating to the development and approval of arimoclomol and the  
full claw back liability related to the French early access program, to Zevra.  
The Sale of Assets agreement for a total of USD 12.8 million in cash and assumed liabilities estimated to equal  
approximately USD 5.2 million (the Sale of Assets). Under the terms of the agreement, Zevra agreed to also  
retain a majority of Orphazyme’s employees.  
The business operations and activities that were part of the Sale of Assets agreement with Zevra have been  
reclassified as discontinued operations in 2022 -, which is presented below-. The net result related to the  
above-mentioned operations held for sale are therefore presented separately in the income statement and the  
statement of cash flows.  
Judgement regarding management’s accounting for discontinued operations  
Significant judgment is required to determine the presentation of discontinued operations in profit and loss and  
cash flow, for both the current and prior year. Management has done the judgement based on the Purchase  
agreement from the sale of assets to Zevra.  
Substantially all of Orphazyme’s assets and business activities was sold to Zevra (the “Sale of Assets”) on an  
“as-is” basis and there have not been given any substantial representations or warranties in favor of Zevra.  
While the management are not aware of any outstanding matters that would reasonably form a basis for a  
claim related to the Sale of Assets, if the Company become subject to liability based upon our contractual  
obligations to Zevra or otherwise, it could have a material adverse effect on our financial position.  
Net result and net cash flow from operations held for sale and assets and liabilities in disposal Company held  
for sale are specified below:  
For the years ended December 31,  
DKK 000  
Net revenue  
Note  
2.1  
2023  
2022  
17,867  
-
-
-
-
-
-
-
-
Research and development expenses  
General and administrative expenses  
Operating loss  
2.2  
2.3  
(36,660)  
(48,485)  
(67,278)  
4,106  
Financial income  
2.6  
2.6  
Financial expenses  
(9,116)  
(72,288)  
—
Loss before tax  
Income tax benefit  
2.7  
Net result for the period  
-
-
-
(72,288)  
145,666  
73,378  
Gain from disposal of assets and liabilities held for sale  
Net result from discontinued operations  
DKK 000  
Note  
2023  
2022  
Cash flow from operating activities  
Cash flow from investing activities  
Cash flow from financing activities  
Net cash flow from operations held for sale  
-
-
-
-
(6,973)  
90,347  
(40,388)  
42,986  
22  
 
Gain on disposal/carrying amount of disposed assets  
DKK 000  
Carrying values of intangible assets  
Note  
2023  
2022  
1,603  
-
-
-
-
-
-
-
Carrying values of property, plan and equipment  
Carrying values of liabilities  
134  
(58,370)  
(56,633)  
145,520  
145,520  
88,887  
Carrying values of assets and liabilities  
Gain on disposal – net  
Gain on disposal  
Net cash inflow from disposal of business  
2.1  
NET REVENUE  
§ ACCOUNTING POLICIES  
Orphazyme recognizes revenue when fulfilling its performance obligation by transferring control of promised  
goods or services to its customer, in an amount that reflects the consideration that the entity expects to  
receive in exchange for those goods or services.  
Due to the sale of activities to Zevra in 2022, the Company did not have any revenue in 2023. Net revenue in  
2022 comprises revenue from the sale of arimoclomol for the treatment of NPC under the remunerated early  
access compassionate use program (“nATU”) in France. An early access compassionate use program is a  
program giving specific patients access to a drug, which is not yet approved for commercial sale. Only drugs  
targeting serious or rare indications and for which there is currently no appropriate treatment are considered  
for early access compassionate use programs. Further, to be considered for the early access compassionate  
use program, the drug must have proven efficacy and safety and must either be undergoing price negotiations  
or seeking marketing approval.  
As of June 2022, the remunerated early access compassionate use program (“nATU”) in France, was  
transferred to Zevra as part of the sale of substantially all of the Company’s assets and business activities. All  
revenue is therefore presented as discontinued operations (Note 1.7).  
The following table presents net revenue for the years ended December 31:  
DKK 000  
Revenue by type  
2023  
2022  
17,867  
Revenue from sale of goods  
Revenue by partner  
Clinigen Health Limited  
Geographical areas  
France  
—
—
—
17,867  
17,867  
2.2  
RESEARCH AND DEVELOPMENT EXPENSES  
§ ACCOUNTING POLICIES  
Research expenses comprise of costs incurred during the very early stages of the drug development cycle from  
initial drug discovery until the drug is ready for administration to humans.  
Research and development expenses include costs arising from research and clinical development activities  
including employee costs for research and development personnel (i.e. salaries, bonuses, employer  
contributions to pension schemes, share-based compensation), legal expenses related to the protection,  
defense and enforcement of the Company’s intellectual property, depreciation of right-of-use assets associated  
with facilities and equipment used for research and development purposes, as well as close down and  
restructuring costs for clinical trial close-out costs and employee redundancies. The following table presents  
research and development expenses recognized for the years ended December 31:  
DKK 000  
External costs  
Employee costs (Note 2.4)  
2023  
2022  
18,385  
—
—
17,926  
Depreciation, amortization and impairment (Notes 3.1, 3.2,  
3.3)  
Total research and development expenses  
349  
36,660  
—
—
23  
 
External costs comprise mainly expenses related to third party vendors providing services related to our  
research and development activities and facility costs. Research and development expenses include costs  
relating to products sold under the French early access compassionate use program.  
As of June 2022, all research and development activity were transferred to Zevra as part of the sale of  
substantially all of the Company’s assets and business activities. All research and development expenses are  
therefore presented as discontinued operations (Note 1.7) in 2022.  
As a result of the Sale of Assets in 2022, there are no research and development expenses in 2023.  
2.3  
GENERAL AND ADMINISTRATIVE EXPENSES  
§ ACCOUNTING POLICIES  
General and administrative expenses include salaries for our employees working on pre-launch preparation  
activities as well as administrative employees and Executive Management; remuneration to the Board of  
Directors; share-based compensation costs related to such employees and the Board; depreciation of right-of-  
use assets associated with facilities not used for research and development purposes, investor relations, and  
accounting and legal fees. In addition, we include costs incurred in pre-launch preparation activities such as  
market access, marketing, and medical affairs in general and administrative expenses, including the costs  
associated with the Early Access Program for NPC in the U.S., tradename costs, market and pricing studies and  
related costs.  
The following table presents general and administrative expenses for the years ended December 31:  
DKK 000  
Intercompany expense  
2023  
2022  
8,944  
—
External costs  
5,057  
7,827  
14,157  
—
59,650  
20,699  
—
433  
89,726  
Employee costs (Note 2.5)  
Settlement of U.S. lawsuit  
Depreciation (Notes 3.2 and 3.3)  
Total general and administrative expenses  
27,041  
As of June 2022, general and administrative activity was partly transferred to Zevra as part of the sale of  
substantially all of the Company’s assets and business activities. General and administrative expenses were  
therefore in 2022 presented as both continued operations, DKK 41.2 million and discontinued operations (Note  
1.7), DKK 59.5 million.  
2.4  
EMPLOYEE COSTS  
§ ACCOUNTING POLICIES  
Employee costs primarily comprise salaries, bonuses, social security contributions, share-based compensation,  
vacation and sick leave as well as the employer portion of pension contributions. In addition, severance  
payments or termination benefits are also included under Employee Costs. The cost of these benefits is  
recognized as an expense as services are received. All employee pension plans are defined contribution plans  
and not defined benefit plans.  
Employees are eligible to receive a discretionary bonus subject to certain predefined and individual goals as  
determined by the Board of Directors. Employees are also eligible to receive an extraordinary bonus at the  
discretion of the Board of Directors.  
The following table presents Employee Costs, including remuneration to the Board of Directors and Executive  
Management, for the years ended December 31, 2023. Refer to note 4.5 for more discussion on remuneration  
of Board of Directors and Executive Management.  
24