HSP Types

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Hereditary spastic paraplegia (HSP) is the name for a group of inherited diseases, which have lower limb weakness at their core. New types of HSP are being regularly discovered, thanks to advances in genetics.

The HSP Support Group have collated this simplified table using information from the following sources:

NCBI - National Centre for Biotechnology Information (USA)

OMIM® - Online Mendelian Inheritance in Man® (USA)

NORD – National Organisation for Rare Disorders (USA)

GARD - The Genetic and Rare Diseases Information Center (USA) 

As new research is conducted, findings in this table are likely to change. If you believe there should be an amendment to the information here, please contact us.

HSP type

Gene name

Age of onset

Pure / complicated

SPG1

L1CAM

Early

Complicated

SPG2

PLP1

Variable

Variable

SPG3A

ATL1

Early

Pure

SPG4

SPAST

Variable

Variable

SPG5A

CYP7B1

Variable

Variable

SPG6

NIPA1

Variable

Variable (often pure)

SPG7

SPG7

Variable

Variable

SPG8

KIAA0196

Adult

Pure

SPG9A

ALDH18A1

Variable (often teenage onwards)

Complicated

SPG9B

ALDH18A1

Early

Complicated

SPG10

KIF5A

Variable

Variable

SPG11

SPG11

Variable

Complicated

SPG12

RTN2

Variable (often before 20 years)

Pure

SPG13

HSP60

Variable

Pure

SPG14

Adult

Complicated

SPG15

ZFYVE26

Early

Complicated

SPG16

Early

Complicated

SPG17

BSCL2

Variable (often teenage)

Complicated

SPG18

ERLIN2

Early

Variable

SPG19

Adult

Pure

SPG20

SPG20

Early

Complicated

SPG21

ACP33

Early (often teens to 20’s)

Complicated

SPG22 

SLC16A2

Early

Complicated

SPG23

RIPK5

Early

Complicated

SPG24

Early

Pure

SPG25

Adult

Complicated

SPG26

B4GALNT1

Early

Complicated

SPG27

Variable

Pure

SPG28

DDHD1

Early

Variable

SPG29

Early (often teenage)

Complicated

SPG30

KIF1A

Early (often teenage)

Complicated

SPG31

REEP1

Variable

Variable

SPG32

Early

Complicated

SPG33

ZFYVE27

Adult

Pure

SPG34

Variable (often teens to 20’s)

Pure

SPG35

FA2H

Early

Complicated

SPG36

Variable

Complicated

SPG37

Variable

Pure

SPG38

Variable

Complicated

SPG39

PNPLA6

Early

Complicated

SPG41

Early

Pure

SPG42

SLC33A1

Variable

Pure

SPG43

C19orf12

Early

Variable

SPG44

GJC2

Early

Complicated

SPG45

NT5C2

Early

Complicated

SPG46

GBA2

Variable

Complicated

SPG47

AP4B1

Early

Complicated

SPG48

AP5Z1

Variable

Complicated

SPG49

TECPR2

Early

Complicated

SPG50

AP4M1

Early

Complicated

SPG51

AP4E1

Early

Complicated

SPG52

AP4S1

Early

Complicated

SPG53

VPS37A

Early

Complicated

SPG54

DDHD2

Early

Complicated

SPG55

C12orf65

Early

Complicated

SPG56

CYP2U1

Early

Variable

SPG57

TFG

Early

Variable

SPG58

KIF1C

Early

Complicated

SPG59

USP8

Early

Pure

SPG60

WDR48

Early

Complicated

SPG61

ARL6IP1

Early

Complicated

SPG62

ERLIN1

Early

Variable

SPG63

AMPD2

Early

Variable

SPG64

ENTPD1

Early

Complicated

SPG66

ARSI

Early

Complicated

SPG67

PGAP1

Early

Complicated

SPG68

KLC2

Early

Complicated

SPG69

RAB3GAP2

Early

Complicated

SPG70

MARS

Early

Variable

SPG71

ZFR

Early

Complicated

SPG72

REEP2

Early

Pure

SPG73

CPT1C

Adult

Pure

SPG74

IBA57

Early

Complicated

SPG75

MAG

Early

Complicated

SPG76

CAPN1

Adult

Complicated

SPG77

FARS2

Early

Variable

SPG78

ATP13A2

Adult

Complicated

SPG79

UCHL1

Early

Complicated

Yet to Be Confirmed/Classified

HSP type

Gene name

Age of onset

Pure / complicated

HSNSP

CCT5

Early

Complicated

SERAC1

Early

KY

Early

PLA2G6

Early

ATAD3A

Early

Complicated

KCNA2

Early

Granulin

MT-ATP6

Adult

Complicated

ADAR

Early

Pure

BICD2

Variable

Complicated

DMN23

Early

Complicated

ATP2B4 (PMCA4)

Adult

Pure

TUBB4A2

Early

Complicated

ALDH3A2

Early

Complicated

GAD1

Early

Complicated

GRID2

Early

Complicated

KLC2

Early

Complicated

KLC4

Early

Complicated

IFIH1

Early

Pure

This latest table was updated in September 2021 and provides a snapshot of HSP. Studies of some HSP types have resulted in conflicting information for age of onset and pure / complicated form. Due to this, in places the data includes the most commonly occurring findings. For some types of HSP your diagnosis may therefore not fit this table.

Using the table

The table includes the HSP type, the gene, typical age of onset and typical form (pure or complicated).

HSP Type column

HSP types are identified by the letters SPG then a number. The numbers correlate to the discovery of the faulty gene, so SPG1 was the first to be discovered, in 1994, with SPG79 being the most recent. As you can see from the table there are a number of genes yet to be classified. It is also possible to have a HSP diagnosis, without knowing your HSP type.

Gene name column

This column includes the gene which causes the HSP Type.

Age of onset column

Early onset covers symptoms occurring from birth to the second decade of life (0-19)
Variable means symptoms can occur at any age.
Adult means symptoms typically present after the second decade (20+).

Any additional wording in brackets denotes the most common age of onset.

Pure / Complicated column

Pure means a uncomplicated version of HSP
Complicated means a complicated version of HSP
Variable means that those with the HSP type can fall under either category dependent on their symptoms.

For more information on pure and complicated HSP visit our symptoms page.