SUB-ACUTE COMBINED DEGENERATION OF THE CORD SECONDARY TO PERNICIOUS ANAEMIA INTRODUCTION There are two major problems in diagnosing Pernicious Anaemia. Firstly the symptoms are insidious- they dont suddenly appear but gradually emerge sometimes over a very long time which means it can, and often does,take many years before the symptoms are experienced by the patient to the extent that medical advice is sought. Secondly the symptoms of Pernicious Anaemia are many and often vague and not only do they manifest themselves in different degrees of severity but also physicians often associate them as indicators of the patient having a wide variety of other diseases and medical conditions. These two problems with the symptoms of Pernicious Anaemia can, either individually or together, lead to the patient receiving a late diagnosis- and this can and does lead to damage to the patients nervous system. A patient with Pernicious Anaemia who has yet to be diagnosed and treated will be deficient in Vitamin Bn. Vitamin B12 is not only needed to produce healthy red blood cells that will transport oxygen around the patients body, but it is also needed, along with various other substances, to maintain a healthy nervous system. Left untreated the Vitamin 812 Deficiency leads to damage to the insulation that surrounds nerves. This coating is called the Myelin Sheath and its purpose is to speed up nerve impulses. Severe B12 deficiency over a period of time leads to the Myelin Sheath wasting away Demyelination. When the Myelin sheath withers away due to lack of Vitamin B12 then the diagnosis is Sub-Acute Combined Degeneration of the Cord Secondary to Pernicious Anaemia (SACDC). The transmission of signals along nerves is compromised or entirely lost and left untreated the nerve will ultimately become useless. SYMPTOMS The Nervous System can be divided into two main divisions- Peripheral Nerves and nerves associated with the Central Nerves found in the spinal cord (central nervous system). Symptoms of damage to the Peripheral Nerves (nerves of the skin): • Pins and Needles (paresthesia) especially in fingers and toes • Heat sensitivity (symptoms worsen or reappear upon exposure to heat, such as a hot shower) • Odd sensation in legs, arms,c hest, or face, such as tingling or numbness (neuropathy) • Burning sensation in legs and feet (Grierson Gopalan Syndrome) Symptoms of damage to the CentralNervous System (damage to the spinal cord): • Awkward Gait (strange walk) • Stumbling- the need to hold on to something when walking • Difficulty coordinating movement or balance disorder • Inability to stand up straight with eyes closed • Loss of dexterity • Sudden electric shocks running down the body following a sudden forward head movement- Lhermittes sign- (also called the Barber Chair Phenomenon) Blurriness in the centralvisual field that affects only one eye,may be accompanied by pain upon eye movement • Double vision • Loss of vision/hearing • Weakness of arms or legs • Cognitive disruption,including speech impairment and memory loss • Difficulty controlling bowel movements or urination • Irritability and lack of patience Patients will experience some or all of the above symptoms but to varying degrees of intensity. The symptoms are very similar to those of Multiple Sclerosis which leads to many patients being investigated for that condition and,in some cases initially being diagnosed with MS. But it is the absence of healthy levels of Vitamin B12 combined with the above symptoms which leads medical professionals to diagnose SACDC. CONFIRMING THE DIAGNOSIS The doctor will conduct simple tests to determine the extent of the damage to the patients nervous system including the Sharp/Blunt Test where a sharp and then blunt object is placed on the skin and the patient has to differentiate between which of the two objects are being used by the doctor. The Finger to Nose Test is also used to determine the extent of the nerve damage by asking the patient to slowly touch their extended finger to their nose to evaluate coordination. Tuning Forks can be used to provide more information about the damage to the nerves as will the patients ability to walk heel to toe and will help the doctor to determine the extent and type of damage that has occurred to the patients nervous system. Nerve Conduction Tests can also be used which involve tape measures and electrical signals that will allow doctors to determine the extent of the damage to the central nervous system. A Magnetic Resonance Image scan (M.R.I.) is sometimes used to ensure no other medical conditions exist. Defining the Diagnosis So what exactly does Sub Acute Combined Degeneration of the Cord Secondary to Pernicious Anaemia mean? Sub-Acute refers to the fact that the problem is not acute (short-term) nor is it chronic (long-term) in that now any B12 deficiency has been corrected hopefully no further damage will occur. Thus the condition is not chronic in the sense that the cause of the problem has been identified and hopefully some nerves will repair themselves. (There is some evidence that the use of one particular type of artificial B12- Methylcobalamin- can and does promote repair to the damage caused to the Myelin Sheath by low B12 levels- but Methylcobalamin is not licensed for use in the UK or North America). Combined means that both the peripheral and central nerves have sustained damage. Degeneration means that the nerves have not continually renewed themselves as they are supposed to do and have withered away to some extent or other. Of the Cord refers to the spinal cord- the super-highway through which nerve impulses travel. Secondary to Pernicious Anaemia means a product of, or caused by way of, Pernicious Anaemia. Patient Experiences The patient with SACDSC will experience all the difficulties of Pernicious Anaemia as well as some, or all, of the following: • Difficulty in performing daily tasks such as washing hair in the shower or putting on underwear or hosiery- this is due to the balance problems encountered. • Walking in the dark. Because the patients internal balance mechanism may be damaged the sufferer will use fixed objects to focus on in order to compensate for the lack of internal balance. These objects are not visible in the dark and so the patient encounters problems when walking. • Shoe-staring- not a style of music but the need to look at the ground when walking as the ground provides a fixed object on which to focus. This can lead to posture and back problems. • Inability to run or jog. The nerves are simply unable to transmit the signals needed fast enough from the brain to the muscles. • Tripping, stumbling and falling- this is especially true if the peripheralnerves in the feet and lower legs are damaged. The patient is unable to feel any obstacles or potential obstacles. This is especially true when walking in the countryside where brambles, ivy and vines are particular problems. • Extreme vertigo associated with heights. Looking at a void, over a cliff or to the side of high bridges for example, means that there is no focal point on which to concentrate which leads to giddiness and a feeling of vulnerability. Treatment In the UK the British National Formulary- the reference work that doctors refer to that states how medical conditions should be treated- advises that where there is any neurological involvement with Pernicious Anaemia then the treatment should be a lmg/ml injection of Hydroxocobalamin (B12) every other day until there is no further improvement. Unfortunately there are two problems with the patient who has neurological damage getting the treatment he or she should be receiving. Firstly, not many doctors are aware that the BNF makes the difference between treating Pernicious Anaemia and treating Pernicious Anaemia where there is any neurological involvement. Secondly, some doctors are reluctant to identify some symptoms being experienced by the patient as being due to neurological issues. These include the memory loss, forgetting everyday names for things (nominal aphasia), mood swings, irritability etc. These are as much due to neurological involvement as any numbness, balance problems and pins and needles but some doctors are reluctant to consider them to be so. Copied from the Pernicious Anaemia Society pernicious-anaemia-society.org/ The best video that explains what SACD is..it shows the lesions (white spots) on the brain, well worth watching: https://youtube/watch?v=MbvQd1EDjFY all seems very very familiar :(
Posted on: Tue, 05 Aug 2014 00:19:31 +0000
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