Researchers - Initial Numbers

So, Thanks to a post from Rob from EURODIS on Rarediseasecommunity, I have been looking at PubMed, which has a directory of medical publications. On 20th April there were 589 different papers listed for HSP. I'm doing a bit of number crunching on this information. There are two simple sets of numbers - number of papers per year and number of papers per journal which are easy to find out:

Papers per year:

Year	Papers
2012	4
2011	35
2010	38
2009	49
2008	45
2007	45
2006	48
2005	28
2004	36
2003	36
2002	30
2001	27
2000	18
1999	25
1998	24
1997	13
1996	4
1995	9
1994	10
1993	16
1992	8
1991	13
1990	18
1989	6
1988	3
1978	1

Papers per journal - note that I've yet to decipher the abbreviations:

PapersByJournal

Journal	Papers	Most Recent
Neurology	48	2010
J Neurol Neurosurg Psychiatry	24	2010
J Neurol Sci	23	2011
J Neurol	22	2011
Arch Neurol	22	2009
Neurogenetics	21	2011
Am J Hum Genet	20	2008
Eur J Neurol	17	2012
J Med Genet	16	2009
Ann Neurol	15	2007
Mov Disord	13	2011
Clin Genet	13	2011
Hum Mutat	12	2011
Hum Mol Genet	12	2010
Brain	10	2010
Hum Genet	10	2010
No To Shinkei	9	2003
Acta Neurol Scand	9	2002
Zhonghua Yi Xue Yi Chuan Xue Za Zhi	8	2009
Am J Med Genet	8	2002
Clin Neurol Neurosurg	6	2011
Eur J Hum Genet	6	2009
Nat Genet	6	2007
Neuromuscul Disord	5	2009
Ryoikibetsu Shokogun Shirizu	5	2001
J Child Neurol	4	2010
Chin Med J (Engl)	4	2009
J Cell Biol	4	2009
Neuropediatrics	4	2006
Rinsho Shinkeigaku	4	2001
Dev Med Child Neurol	4	1993
Can J Neurol Sci	3	2012
Neurol Sci	3	2011
Pediatr Neurol	3	2011
Prenat Diagn	3	2010
Brain Dev	3	2010
Arq Neuropsiquiatr	3	2010
J Neurochem	3	2009
Funct Neurol	3	2007
Curr Opin Neurol	3	2007
Muscle Nerve	3	2007
Eur Neurol	3	2007
Neuroradiology	3	2006
Cell	3	2005
Curr Biol	3	2005
Zh Nevropatol Psikhiatr Im S S Korsakova	3	1991
Clin Neurophysiol	2	2011
Acta Neuropathol	2	2011
Genome Res	2	2011
J Clin Neuromuscul Dis	2	2011
BMC Biol	2	2010
J Clin Invest	2	2010
BMC Neurol	2	2010
Am J Med Genet B Neuropsychiatr Genet	2	2009
Arch Phys Med Rehabil	2	2009
Hong Kong Med J	2	2009
J Inherit Metab Dis	2	2008
Lancet Neurol	2	2008
Neurosci Lett	2	2008
J Biol Chem	2	2008
Expert Opin Pharmacother	2	2007
Int J Obstet Anesth	2	2007
Am J Med Genet A	2	2005
Neuropathology	2	2005
Amyotroph Lateral Scler Other Motor Neuron Disord	2	2005
Neuropathol Appl Neurobiol	2	2004
Nervenarzt	2	2003
Genomics	2	2003
Semin Neurol	2	1999
Nat Med	2	1998
Pediatr Neurosurg	2	1995
Genet Couns	2	1994
Ital J Neurol Sci	2	1993
Eur Arch Psychiatry Neurol Sci	2	1990
Psychosomatics	1	2012
Spinal Cord	1	2011
Eur J Paediatr Neurol	1	2011
Curr Mol Med	1	2011
Eur J Med Genet	1	2011
Orphanet J Rare Dis	1	2011
Nat Rev Neurosci	1	2011
PLoS Genet	1	2011
Genet Med	1	2011
Gait Posture	1	2011
Amyotroph Lateral Scler	1	2011
Rev Esp Anestesiol Reanim	1	2011
Mol Cell Neurosci	1	2011
Neurologist	1	2011
Mitochondrion	1	2011
Zh Nevrol Psikhiatr Im S S Korsakova	1	2010
No To Hattatsu	1	2010
PLoS Biol	1	2010
Toxicol Lett	1	2010
Dis Model Mech	1	2010
J Lipid Res	1	2010
Ann Clin Lab Sci	1	2010
Hum Brain Mapp	1	2010
Neuroepidemiology	1	2009
Rom J Morphol Embryol	1	2009
Clin Rehabil	1	2009
AJNR Am J Neuroradiol	1	2009
Ann Anat	1	2009
Adv Exp Med Biol	1	2009
Synapse	1	2009
Nature	1	2008
Acta Neurol Scand Suppl	1	2008
SEB Exp Biol Ser	1	2008
BMC Med Genet	1	2008
J Neurosci	1	2008
J Neurogenet	1	2008
J Intellect Disabil	1	2008
Neuroscience	1	2008
Med Arh	1	2007
Mol Cell Biol	1	2007
Mol Neurobiol	1	2007
Brain Res Bull	1	2007
Pediatr Radiol	1	2007
Tidsskr Nor Laegeforen	1	2007
Curr Neurol Neurosci Rep	1	2006
Anesth Analg	1	2006
J Hum Genet	1	2006
Trends Mol Med	1	2006
Ren Fail	1	2006
Panminerva Med	1	2006
Proc Natl Acad Sci U S A	1	2006
Pharmacol Ther	1	2006
Cell Mol Neurobiol	1	2005
Ceylon Med J	1	2005
Biochem Biophys Res Commun	1	2005
J Clin Neurosci	1	2004
Ned Tijdschr Geneeskd	1	2004
Parkinsonism Relat Disord	1	2004
Neuroreport	1	2003
IUBMB Life	1	2003
Exp Neurol	1	2003
Nihon Rinsho	1	2002
Masui	1	2002
Int Rev Neurobiol	1	2002
Neurol Clin	1	2002
Curr Opin Genet Dev	1	2001
Rev Prat	1	2001
Pharmacogenomics	1	2001
Retina	1	2000
Rev Neurol (Paris)	1	2000
Neurophysiol Clin	1	2000
Can J Anaesth	1	1999
Biochim Biophys Acta	1	1999
Electromyogr Clin Neurophysiol	1	1999
Physiother Res Int	1	1999
Neurochirurgie	1	1998
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi	1	1998
Schweiz Med Wochenschr	1	1998
Hum Hered	1	1998
Am J Phys Med Rehabil	1	1998
J Clin Epidemiol	1	1997
Epilepsia	1	1997
Acta Paediatr	1	1996
Acta Neurochir Suppl	1	1995
Brain Pathol	1	1995
Aust N Z J Psychiatry	1	1995
Vojnosanit Pregl	1	1993
Electroencephalogr Clin Neurophysiol	1	1993
Paraplegia	1	1993
J Neurosurg Sci	1	1993
Neurourol Urodyn	1	1993
Aust Vet J	1	1993
Orthop Rev	1	1992
Minerva Med	1	1992
Clin Neuropharmacol	1	1991
Clin Exp Neurol	1	1991
Fortschr Neurol Psychiatr	1	1991
J Assoc Physicians India	1	1990
Am J Hematol	1	1990
N Engl J Med	1	1990
Prog Clin Biol Res	1	1989
J Med Assoc Thai	1	1989
Medicina (B Aires)	1	1988
Ann Pediatr (Paris)	1	1978

More details in a later post - the link with all this lovely info is here: http://www.ncbi.nlm.nih.gov/pubmed?term=%22Spastic%20Paraplegia,%20Hereditary%22[Majr]

Spastic Gait

I realised that I've been blogging for a while and haven't really touched upon the primary outcome of FSP/HSP - the spastic gait. This is also identified on some FSP sites as a scissor gait.


There are a number of references which give descriptions of a spastic gait - here's part of one I like:


When a person has a spastic gait, his legs are typically weak and abnormally stiff. As he walks, he holds his legs closer together than normal, drags his feet or toes, and lacks the typical flexibility in his ankles and knees. When a person has a spastic gait, his legs, toes, and feet are stiffer than normal. He typically does not flex his muscles and bend his legs as he walks. Instead, long-term muscle contractions usually  cause him to drag one foot or his toes when he walks. Sometimes medications may also be used to treat this gait problem. For example, medicines may be used to reduce muscle contraction, but the effects of many medications are still being studied. (http://www.wisegeek.com/what-is-a-spastic-gait.htm)


Here is a description of scissor gait http://www.gpnotebook.co.uk/simplepage.cfm?ID=818937856: Scissor gait is the term used to describe the manner of walking that is seen in patients with spastic paraplegia, the features are:

• rigidity and excessive adduction of the leg in swing

• plantar flexion of the ankle

• flexion at the knee

• adduction and internal rotation at the hip

• contractures of all spastic muscles

The individual is thus forced to walk up on tip-toe, the other contractures resulting in the knees rubbing together and crossing in a manner analogous to scissors.

Of course, the shortest definitions are often the best: http://medical-dictionary.thefreedictionary.com/Scissors+gait:
scissors gait a crossing of the legs while advancing with slow, small steps.
spastic gait a walk in which the legs are held together and move in a stiff manner, the toes seeming to drag and catch. A slow, shuffling gait in which the patient appears to be wading in water. Knee and hip movements are restricted.


What I'm looking for is the sequence. It would appear to start like this: Initial symptoms are typically difficulty with balance, stubbing the toe or stumbling. (symptoms: http://en.wikipedia.org/wiki/Hereditary_spastic_paraplegia) but there doesnt seem to be much around which describe the next steps. Perhaps this is something I'll have to note myself in my symptoms updates.


I also found these exercises which claim to improve gait:
http://www.ncpad.org/exercise/fact_sheet.php?sheet=566&section=2632