Monday, August 18, 2014
September 2014 - Volume 35 - Issue 8 - p 1327-1329 doi: 10.1097/MAO.0000000000000485 Sensorineural Hearing Loss & Tinnitus
Bilateral Hearing Loss Heralding Sporadic Creutzfeldt-Jakob Disease: A Case Report and Literature Review
Salazar, Richard*; Cerghet, Mirela†; Ramachandran, Virginia‡
Supplemental Author Material Collapse Box Abstract Objective
To report a case of sporadic Creutzfeldt-Jakob disease (CJD) with bilateral hearing loss at onset and literature review of the scarce cases of CJD with similar audiologic manifestations at presentation.
A 67-yr-old man presented to the hospital for evaluation of rapid progression of behavioral decline, unsteady gait, and bilateral hearing loss. Three months before admission, he abruptly developed bilateral hypoacusis without associated tinnitus or vertigo. Shortly after, his family noted an ataxic gait and behavioral changes, for example, paranoid delusions. Initial workup, including a complete autoimmune panel and heavy metals, infectious, toxicology, and paraneoplastic panel (e.g., anti-Hu, anti-VGKC), was conducted. Electroencephalography revealed diffuse generalized slowing without periodic complexes. The presence of distortion product otoacoustic emissions bilaterally was consistent with normal cochlear function, suggesting a retrocochlear origin for symptoms of hearing loss. In the meantime, the patient developed startle myoclonus. The brain magnetic resonance imaging demonstrated asymmetric cortical ribbon along with T2 FLAIR signal hyperintensities of bilateral basal ganglia. Later on, the protein 14-3-3 in the cerebrospinal fluid came back positive, which supported the diagnosis of CJD. Only three cases of CJD with deafness at onset have been published: one sporadic, associated with symptoms suggestive of polyneuropathy; and the other two familial, with the E200K mutation. One presented with symptoms of polyneuropathy and the other with typical features.
This case illustrates the phenotypic variability of presentation of CJD in a patient with hearing loss as the initial manifestation. In patients with subacute bilateral hypoacusis and signs of dementia, the differential diagnosis of CJD must be taken into consideration.
Copyright © 2014, Otology & Neurotology, Inc.
SepOct2012 | Audiology Today 53
The Case of Encephalopathy Presenting as Hearing Loss
By Emily Na irn, Virginia Ramacha ndran, and Brad A. Stach
Case History A 67-year-old male was seen in the emergency department, accompanied by his family. The patient was unable to communicate and could not give his own case history information. It was reported that both the patient and his wife developed flulike symptoms about two months earlier. His wife recovered, but the patient reportedly woke up one morning shortly after contracting the illness and stated that he was having “difficulty hearing.” At this time he also complained of a headache at the back of his head and base of his neck. Over the course of the next two months he began to experience confusion, gait problems, slurred speech, weight loss, emotional lability, and eventually, hallucinations. The patient’s family noticed a distinct progressive decline in hearing ability during this time, with the patient talking loudly. Due to the severity and progression of symptoms, the patient’s daughter, who lives in the United States, flew to his home in Central America, escorted her father back to the United States, and brought him immediately to the emergency department. The treating physician reported that the patient was “deaf” and was speaking loudly. At this time, the patient was admitted with a working diagnosis of “encephalitis” (inflammation of the brain).
And the Diagnosis Is… Creutzfeldt-Jakob disease
worsening hearing loss were instrumental in shaping the initial diagnostic picture for the treating physicians. Hearing loss is characteristic of Susac’s syndrome but rare in CJD, and this knowledge initially led the team to lean toward the former diagnosis, which has a very different prognosis and treatment strategy. In the end, audiologic evaluation results were only able to rule out extensive peripheral hearing loss, and we were 56 Audiology Today | SepOct2012 CSI: Audi ology unable to confirm or rule out the existence of retrocochlear or cortical hearing loss. As we are well aware, the existence of peripheral sensorineural hearing loss in the older population is pervasive, so the presence of such in this patient would not be diagnostically useful. However, the absence of peripheral hearing loss, while not ruling out Susac’s syndrome, can be helpful in the diagnostic equation, because it has been shown that the hearing loss characteristic of this disorder is peripheral and not central (Rennebohm et al, 2010). This case is illustrative of the challenges inherent in diagnosing rare disorders in the face of abnormal presenting symptoms. The due diligence that ultimately resulted in the determination of CJD demonstrates the value that can be found in a multidisciplinary approach to diagnosis.
SEE FULL TEXT ;
Familial Creutzfeldt–Jakob disease with E200K mutation presenting with neurosensorial hypoacusis
Creutzfeldt–Jakob disease (CJD) is characterised by rapidly progressive dementia, myoclonus, ataxia, visual disturbances and motor dysfunction. Neuropathological examination shows diffuse spongiosis, neuronal loss, gliosis and a variable degree of amyloid plaque deposition composed of protease-resistant prionic protein (PrPRES) in several locations, including the brain stem. The most frequent clinical presentations are dementia, ataxia or visual symptoms. Most of the cases are sporadic. Only 10–15% are familial, and the most frequent point mutation is E200K. The course of disease, investigation results and neuropathology are similar to those of the sporadic form of CJD. The typical clinical presentation of E200K is a rapidly progressive dementia with myoclonus and pyramidal, cerebellar or extrapyramidal signs.1 We report a familial case with an unusual onset, with deafness and polyneuropathy.
A 53-year-old man presented with subacute progressive bilateral hypoacusis, with tinnitus in the left ear. He was a frequent diver and the symptoms were attributed to barotrauma. During the following months, his hypoacusis worsened and he progressively developed bilateral stocking-type paresthaesia and gait instability. On examination, he was alert and cooperative, although communication was mildly affected because of the hypoacusis. He showed emotional lability; his speech was slow but fluent, and he was partially disoriented in time. Extrinsic ocular motility, cranial nerves and muscular strength were normal. Lower limbs showed mild hypertonia, right extensor plantar response, stocking-type hypoaesthaesia and hypopallaesthaesia, and moderate gait ataxia. An audiometric examination showed bilateral neurosensorial hypoacusis, and nerve conduction studies showed a mixed axonal polyneuropathy. Computed tomography and magnetic resonance imaging of the brain were normal and the electroencephalography (EEG) showed non-specific changes.
These symptoms led to an initial suspicion of a paraneoplastic disorder, and an examination for malignant disease was started. At this moment, we learnt that the patient’s mother had died of neuropathologically confirmed CJD; hence we conducted a CSF 14-3-3-protein detection test, which was positive. Serial EEGs showed repeated non-specific changes. Brain stem auditory evoked potentials (BAEPs) could not be performed, owing to lack of patient collaboration.
During the following 2 weeks, myoclonus appeared and rapidly generalised, mental status deteriorated and progressive ataxia confined the patient to bed. He died of respiratory infection 10 months after onset of symptoms.
Neuropathological examination showed neuronal loss, microspongiosis and astroglial and microglial proliferation predominantly in the isocortex, entorhinal cortex, and hippocampal CA1 region, striatum, amygdala and cerebellar cortex. Punctate, synaptic-like deposits of PrPRES in the cerebral and cerebellar cortices were found, as well as scattered large PrPRES deposits in the granular layer of the cerebellum. The mesencephalon did not show spongiosis, but gliosis in colliculum and periaqueductal grey matter were detected.
Marked neuronal loss and gliosis in the vestibular and cochlear nuclei were observed, associated with PrPRES deposition (fig 1). Western blot of PrPRES showed a three-band pattern, with an unglycated fragment migrating at 21 kDa, corresponding to PrPRES type 1. Genetic sequencing of PrP showed the presence of the E200K mutation in heterozygosis. No insertions or deletions were found in the 51–91 region. The patient was heterozygotic for the M/V polymorphism at codon 129.
Only two cases of CJD with deafness at onset have been published: one sporadic, associated with symptoms suggestive of polyneuropathy,2 and the other familial, with the E200K mutation and typical features.3 Other cases have been reported as presenting with auditory agnosia or with cortical deafness, and early involvement of the acoustic pathway was already detected through demonstration of progressive BAEP deterioration in patients with CJD who did not present deafness in the course of the disease.
The first case was that of a 71-year-old woman who presented with a sudden change in hearing and aural fullness, and a vague feeling of imbalance.2 Hearing loss and gait instability worsened rapidly Audiometry showed bilateral neurosensorial hearing loss, and BAEPs were initially normal. She later developed signs of polyneuropathy and mental deterioration, left homonynous hemianopsia and decreased vibratory and pinprick sensation. The second case was that of a 46-year-old Italian woman with the E200K mutation, who had rapidly worsening hearing loss.3 Three weeks later she developed an unstable gait, and her condition rapidly progressed to bilateral deafness, ataxia, myoclonus, pyramidal and extrapyramidal dysfunction, and mental deterioration. She died 6 months after the onset of the disease. Magnetic resonance imaging scans showed high signal areas, mostly in the caudate and putamen, EEGs showed periodic sharp-wave complexes, and protein 14-3-3 was present in the cerebrospinal fluid. Audiometric investigation showed bilateral sensorineural hearing loss, and BAEP abnormalities from the beginning seemed to confirm early brain stem involvement. The course of the illness, clinical features and EEG recordings were similar to those of the sporadic form of CJD.
Accumulation of PrP in the brain stem has been found to be an early pathological event in sporadic CJD, but these deposits are not necessarily associated with clinical symptoms or neuronal loss, and the brain stem seems to remain relatively resistant to the pathological process of sporadic CJD.4 Neuropathological changes in brain stem structures have been described in sporadic and familial CJD, associated with atypical onset, with gaze disorders and with fatal familial insomnia. Unfortunately, necropsy was not possible in the two patients with early deafness, and to our knowledge specific involvement of cochlear and vestibular nuclei has not been reported previously
Western blot of PrP showed a type 1 pattern in our case. This is the pattern usually observed in sporadic CJD M/M homozygotic at codon 129, and it has also been described in patients with the E200K mutation associated with the allele 129M in the mutated chromosome.5 It is not known whether the glycation pattern of abnormal PrP has an influence on phenotype. In our patient also, who was M/V homozygotic, the codon 129 status of the mutated allele was not investigated. This case illustrates the phenotypic variability of presentation of CJD, and describes the specific involvement of brain stem auditive nuclei in a patient with hypoacusis as the initial manifestation, thereby reflecting early brain stem involvement.
Prions 2005, pp 31-40 Dura mater related Creutzfeldt-Jakob disease in Japan: Relationship between sites of grafts and clinical features
T. Sato, M. Masuda, Y. Utsumi, Y. Enomoto, M. Yamada, H. Mizusawa, T. Kitamoto
A nationwide survey documented 117 cases of Creutzfeldt-Jakob disease (CJD) transmitted from cadaveric dura mater grafts in Japan to September 2004. Of these, 110 patients were identified to have received the same type of lyophilized cadaveric dura mater graft during the period between 1978 and 1991. Incubation period from grafting to onset of symptoms varied from 16 months to 23 years, with most patients developing neurological symptoms after 2 to 15 years. We conducted q retrospective review of the full medical records of 107 of dura-related CJD (dCJD) patients. Patients were divided into two groups by site of neurosurgical or orthopedic procedure (supratentorial vs. infratentorial). Hemiparesis or hemianopsia developed as an initial manifestation in 31.9% of 47 patients with supratentorial grafts but did not develop among any of the infratentorial group (p<0 .0001="" 25.0="" brainstem="" but="" conversely="" diplopia="" div="" facial="" group="" hearing="" in="" infratentorial="" ipsilateral="" loss="" not="" noted="" nystagmus="" of="" or="" p="" paresis="" paresthesia="" seen="" supratentorium="" symptoms="" the="" were="">
Hearing loss as the initial presentation of Creutzfeldt-Jakob disease
July 31, 2004 by Priya Krishna, MD; Carol Bauer, MD, FACS
Creutzfeldt-Jakob disease is a rare type of spongiform encephalopathy. Affected patients present with constitutional symptoms, which progress to severe mental deterioration and movement disorders. Dizziness is the most common early otologic symptom. Few reports in the literature describe patients with Creutzfeldt-Jakob disease who present with sudden-onset hearing loss as their primary symptom for seeking treatment. This paper discusses one such patient and reviews the clinical presentation, treatment options, and relevant literature.
J Neurol Neurosurg Psychiatry 1994;57:872-873 doi:10.1136/jnnp.57.7.872 Research Article
A case of Creutzfeldt-Jakob disease presenting with cortical deafness. E Tobias, C Mann, I Bone, R de Silva, J Ironside J Neurol Neurosurg Psychiatry 1994;57:7 872-873doi:10.1136/jnnp.57.7.872
In conclusion, this pathologically verified case of Creutzfeldt-Jakob disease presented with cortical deafness. This is, to our knowledge, the first description of such a presentation, and emphasises the variety of cortical disturbances with which this disease can present...
Sunday, December 15, 2013
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007
Date: March 21, 2007 at 2:27 pm PST
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II
Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007
Cattle feed delivered between 01/12/2007 and 01/26/2007
Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
Firm initiated recall is ongoing.
Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE
Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007
The firm does not utilize a code - only shipping documentation with commodity and weights identified.
Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.
Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE
ID and NV
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
Friday, December 14, 2012
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012
In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. ***However, this recommendation is guidance and not a requirement by law.
Animals considered at high risk for CWD include:
1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and
2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.
Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.
The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.
Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.
There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.
36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).
The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE).
Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.
The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).
In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.
In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.
Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.
The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month.
nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment.
sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?
Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end
Sunday, June 29, 2014
Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
Tuesday, August 12, 2014
*** MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014 ***
see history of record of either the biggest cover up of mad cow disease, or one of the biggest blunders of the mad cow debacle, just my opinion of the facts...tss
Monday, June 02, 2014
Confirmed Human BSE aka mad cow Variant CJD vCJD or nvCJD Case in Texas
Tuesday, April 01, 2014
*** Questions linger in U.S. CJD cases 2005, and still do in 2014 ***
Monday, August 18, 2014
*** Barriers to Creutzfeldt-Jakob Disease Autopsies, California and the USA
PLEASE REMEMBER ;
The Akron, Ohio-based CJD Foundation said the Center for Disease Control revised that number in October of 2004 to about one in 9,000 CJD cases per year in the population group age 55 and older.
HAVE YOU GOT YOUR CJD QUESTIONNAIRE ASKING REAL QUESTIONS PERTAINING TO ROUTE AND SOURCE OF THE TSE AGENT THAT KILLED YOUR LOVED ONE ???
if not, why not...
Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
2000 Terry S. Singeltary Sr.
just speaking of human TSE's; "different strains (of same disease), different routes of infection (of same disease), different infectivity levels (dose rate) of the (same disease) = different symptoms, different lengths of illness from 1st onset of illness to death, (of the same disease) + different cultures = different geographical locations = different strains (of same disease)...TSS"
see full text ;
p.s. for anyone wanting to read the old links, you will have to change the links with lyman in it, change lyman to madcow, leave everything else the same, and then copy and paste that link in the link window and it should work. the vegsource site was the first site I started documented the history of the usda inc. mad cow follies. it was before the blogs, before social media, and all that stuff. thank you to Jeff Nelson for allowing me to document this history, and keeping it on files for everyone to witness. this was the second message board. there was a first with Howard Lyman and Oprah Winfrey, but after the first trial, and before the second trial, they took the first website down, no history of that board is available, but when it came back after the second trial was victorious as well, then it just became a board for me to post science and history of the TSE prion aka mad cow type disease too. ...
'v' CJD could it be vaccineCJD???
change to ;
YOU can see the history of the USDAinc MAD COW FOLLIES HERE. remember, I was young and dumb, my manors were not to write home to mom about, but I was persistent in wanting to find the truth. ...