The stench is the most obvious and recognisable sign of MSUD. People with MSUD frequently experience a sweet fragrance resembling maple syrup or burnt sugar in their sweat, earwax, and urine.
For each case of the illness and each form of MSUD, the quantity and intensity of symptoms vary.
Babies with untreated classic MSUD may become more agitated, sleep for longer periods of time or in different positions, and have trouble breathing and feeding. Additionally, they might experience muscle spasms, lose consciousness, or cease breathing altogether. They are susceptible to physical, mental, and developmental delays as they age.
Any age can experience intermediate, sporadic, and thiamine-responsive MSUD symptoms. When you are ill or under stress, MSUD symptoms might occasionally appear or get worse. Symptoms in adults or older children include:
- abdominal pain
- Anorexia and losing weight
- weakness or loss of control of the muscles
- uncontrollable motions
- Unsteady speech
- alterations in consciousness or difficulty staying vigilant
The Culprit: Diabetic Ketoacidosis
Sweat (or breath) with a fruity aroma may indicate diabetic ketoacidosis, a dangerous consequence of diabetes.
According to Dr. Adimoolam-Gupta, if blood glucose levels are not controlled, this happens to people with diabetes, typically type 1 diabetes.
Why? According to her, ketones, a type of chemical your liver produces when it breaks down lipids, are formed when blood glucose levels are too high and there is not enough insulin to lower them. Ketones have a delicious fragrance.
According to the Mayo Clinic, further signs of diabetic ketoacidosis can include:
- extreme thirst
- often urinating
- nausea and diarrhoea
- weakness or exhaustion
- breathing difficulty
Fix it: If you experience any of the following diabetic ketoacidosis symptoms, contact an emergency room right away. Dr. Adimoolam-Gupta advises using insulin and intravenous fluids to treat this illness quickly. Diabetes-related ketoacidosis, if left untreated, can cause a coma or possibly death.
Fortunately, managing your diabetes (via diet, exercise, medication, and regular blood sugar testing) to make sure your glucose values are in a suitable target range will lower your risk of developing ketoacidosis, according to Dr. Adimoolam-Gupta.
What vitamin gives off a maple syrup-like odor when you perspire?
The gym was where it all began. One mile into my jogging on the treadmill, I was overcome by a plume of sweet-smelling perfume. I turned to my left and saw a young coed moving forward in a pink tank top with mascara-coated eyelashes. Who uses body spray before exercising? I scoffed in silence and went on with my run, picturing sugary waffles and syrup-drenched snaps. She hopped off of her machine after twenty minutes, but the odor persisted. I wheezed. I did it. The fragrance came from me. I smelled waffle-y.
Confusion growing, I covertly lowered my nose near my shoulder and sniffed while riding the subway home. I continued uncomfortably sniffing myself while attempting to understand, just like Molly Shannon’s SNL Superstar character did. This stench reminded people of burning sugar and fat, a bottle of Aunt Jemima syrup that had burst, or the interior of an IHOP. I pondered the last time I had consumed maple syrup. I had brought jars of dark amber syrup home from Vermont months earlier for some early-season sugaring, but I hadn’t yet broken the seal.
A little online investigation at home increased my anxiousness.
Evidently, the scent of sugar is a warning sign for a serious metabolic problem, although that was largely for children under the age of three. My sugar intake was typical, if maybe a touch shallow, and neither French toast nor a sticky stack of pancakes were suspicions. I hadn’t had breakfast in a long time.
The odor persisted the following day. I was grabbing a tray from a high shelf at work when a coworker walked by. He shifted. “You! You actually do smell like maple syrup, wow. Weird. My sweet stench was audible. It was extremely overwhelming to me and those nearby.” Three days later, my lover confessed, “I kind of enjoyed it at first, but it’s starting to make me sick, like a sick child who ate too much toffee ice cream.” I had to discover the solution.
Most physiological odors are produced by bacteria that grow after sweating; sweat, which contains a variety of trace elements, minerals, and pheromones, is virtually completely odorless to humans. Nevertheless, a wide range of characteristics, such as gender, health, and heredity, have an impact on how we smell. Additionally, our individual odors can vary from day to day depending on what we eat. Body odors have as much individuality as fingerprints.
Rarely does the mole enchiladas you had for lunch transfer into a smell, but physiological odors can be reminiscent of certain foods.
I’ve encountered friends who smell like musty cumin-seasoned pork or vinegary sauteed onions. Certain meals can release distinctive scents as they are digested. Some people who consume a lot of garlic after eating experience a strong perspiration reaction as their systems break down sulfur molecules. Allyl methyl sulfide, which is abundant in garlic, can seep via pores the next day. The majority of us are also familiar with the sharp, greenish odor that follows eating asparagus because of the urine-borne methyl mercaptan that is produced.
I was thinking about another odorous mystery when I smelled my own maple aroma. The past five years have seen a delicious aroma occasionally drift around Manhattan, as New Yorkers may remember. The Bloomberg administration launched an investigation after the Office of Emergency Management received a barrage of complaints about a smell resembling caramel at the beginning of last year. The North Bergen Frutaron flavor plant in New Jersey, where the aroma was discovered, was located across the river. Fenugreek, a spice most frequently used in Indian cuisine and the main flavoring element in fake maple syrup, was being processed at the company’s plant, among a few others.
Solotone is a highly powerful fragrant chemical found in fenugreek. Solotone travels through the body and, when ingested in large quantities, can provide a sweet, maple-like odor in perspiration and urine. It is also found in lovage, some aged rums, and molasses. The Journal of Pediatrics and Child Health has reported instances of newborn newborns with sweet scents from mothers who drank fenugreek before giving birth. Fenugreek is commonly used as a milk stimulant for nursing mothers.
Fenugreek! Fenugreek. Fenugreek? Hmm. I was still in a state of shock. The only fenugreek in my flat was in a tube that was maturing in the back of a kitchen cabinet and was covered in dust. I hadn’t eaten Indian food in weeks. I discovered the answer when I searched for pictures of fenugreek. Fenugreek is a little, spindly plant with green, teardrop-shaped leaves and short roots. Its beige pellet seeds are most frequently used as a spice because they give curries and rice dishes a savory flavor. However, the leaves, stems, and other parts of the leguminous plant are a staple in Ethiopian, Yemeni, and Indian cuisines. Methi, as it is known in Hindi, is frequently sautéed like spinach and is high in protein.
I mistook a bouquet of grass-colored, crisp greens that a friend had given me for an Indian watercress type the week before. I turned them into a peppery salad with radish and scallion, thereby transforming my body into a factory that emits lovely scents. The effect of raw fenugreek on my pores persisted for days because digestion takes many hours and the release of solotone takes several hours longer.
I took the train to Jackson Heights last week and bought a handful of fresh fenugreek leaves from an Indian grocer to confirm my suspicions. I consumed them raw in a different salad, and the eerie scent is already starting to reappear. I’m now getting the want for a little stack of fluffy buttermilk pancakes with some pooled fake syrup.
What causes body odor that has a nice scent?
A change in body odor could indicate diabetic ketoacidosis if you have diabetes. Your blood becomes more acidic and you start to smell fruity when you have high ketone levels. Your breath may smell like bleach if you have liver or kidney illness because of the buildup of toxins in your body.
Do hormonal changes cause body odor to smell?
Yes, hormonal changes can make your body odor smell. During menopause, hot flashes, nocturnal sweats, and hormonal changes result in increased perspiration, which affects how one smells. Some people think that while they are pregnant or menstruation, their body odor changes. According to research, a person’s body odor changes during ovulation—the phase of the menstrual cycle when they have a chance of becoming pregnant—to entice a mate.
Can certain foods cause body odor?
In terms of body odor, the adage “you are what you eat” may be true. You may develop body odor if you consume foods high in sulfur. Sulfur has a rotten egg odor. It can emit a foul odor when it is produced from your body through sweat. Examples of foods high in sulfur include:
- beef – red.
Other typical food sources of offensive body odor include:
- sodium monoglutamate (MSG).
- curry and cumin, for example.
- spicy meal or other hot sauce.
What odor does diabetic sweat have?
The aroma of someone’s perspiration is influenced by a variety of factors. Body odor can be affected by diet, physical activity, and bacterial illnesses.
Sweating that smells like ammonia can also be a sign of a health issue like diabetes or renal disease.
Deodorants can be used to mask scents, and antiperspirants can be used to lessen sweating. To help lessen the ammonia smell in perspiration, a doctor can treat any underlying medical issues.
Does maple syrup urine illness affect adults?
More than two thirds of the 17 patients in our sample showed signs of a movement problem during a clinical examination, mostly tremor and dystonia or a combination of the two. These were evident in all cases in the upper limbs, with additional involvement in the neck and lower limbs in two individuals each. In addition to movement abnormalities, learning difficulties and pyramidal symptoms in the lower limbs were the most frequent neurological findings, appearing in 64.7% and 58.8% of cases, respectively. The most debilitating clinical finding was the coexistence of dystonia and spasticity in the lower limbs, which greatly decreased patients’ capacity to walk on their own and adversely affected their quality of life.
Movement problems in MSUD have only previously been characterized as recurring paroxysmal ataxia or paroxysmal dystonia in intermittent variant MSUD, and during bouts of decompensation. These descriptions all took place in the setting of a toxic encephalopathy.
1,8,9 After receiving the proper dietary treatment in these instances, movement problems tended to get better.
Many of the adult patients in our sample continued to experience mobility disorders despite rigorous food restrictions and the absence of recurrent metabolic decompensation. We noticed no significant cerebellar symptoms, such as ataxia, as had been previously reported3, either at the time of presentation or during episodes of decompensation, despite the typical severe cerebellar edema evident on neuroimaging10 and the presence of clear neuropathological alterations in the cerebellum. 1
As recently demonstrated by Gaucher’s disease-related parkinsonism, movement disorders caused by metabolic diseases are of great interest since they may offer some insights into pathogenesis. Our clinical findings call into question the type of pathology that underlies the mobility problems experienced by adult MSUD patients. Regarding neuropathology, it is well-known that MSUD causes significant neuronal loss in the pontine nuclei and substantia nigra11, and striatal damage has been shown in both the animal model of MSUD12 and in affected children. 4
Furthermore, leucine has a strong affinity for the L1-neutral amino acid transporter, which is involved in the transport of other amino acids into the central nervous system. As a result, high plasma concentrations of leucine inhibit the uptake of some amino acids, such as phenylalanine, tyrosine, and tryptophan, into the brain. According to Zinnanti et alanimal .’s model of MSUD13, which showed a drop in dopamine levels in the brain of MSUD mice by more than 60% coupled with lower GABA and glutamate levels, this results in the depletion of some neurotransmitters in the brain such dopamine and serotonin. In MSUD mice, the onset of limb dystonia and aberrant gait patterns coincided with the neurotransmitter depletion, particularly dopamine.
Although these findings were reported during acute encephalopathy, it is possible that altered neurotransmitter levels, particularly dopamine, are present in adult MSUD patients’ brains as a long-term consequence and are to blame for the emergence of dystonia, as seen in other diseases marked by decreased dopamine availability (juvenile Parkinson’s disease, dopa-responsive dystonia).
14,15 A series of 36 infants revealed an intriguing finding: 42.2% of patients had dystonia at the time of diagnosis. This finding was linked to high serum leucine-to-tyrosine ratios, further indicating that acute leucinosis with secondary tyrosine deficiency may be a factor in the development of dystonia. 4
Last but not least, a disruption of cerebral energy metabolism caused by the toxic effects of BCKA on the central nervous system with lower levels of ATP and phosphocreatine has been further explained as the cause of movement disorders in MSUD mice.13 This disruption may affect how normally the basal ganglia and other regions of the brain function, which depend heavily on the mitochondrial respiratory chain.
The irreversible damage brought on by early encephalopathy at onset is likely to have a role in the development of movement disorders, in addition to these neurochemical changes that may, at least in part, be to blame for the movement disorders seen in our patients. In this context, a brain MRI scan of three of our patients with movement disorders (parkinsonism, rest tremor, dystonia, and spastic-dystonic gait) revealed no abnormalities of the cerebellum or basal ganglia. We cannot rule out the possibility that further patients in our series may have structural damage found on neuroimaging, though.
Given the aforementioned biochemical changes, it’s possible that both the frequency and the severity of decompensation events are related to the development of movement problems in MSUD. However, because not all decompensation occurrences in our series led to hospital admission or were well-documented, it was impossible to determine the total number of decompensation episodes over the course of a person’s life.
Symptomatic medications, such as local botulinum toxin injections in patients with dystonia or dopaminergic drugs in patients with parkinsonism, should be taken into consideration as prospective treatments in the future for movement disorders. Deep brain stimulation (DBS) has been utilized successfully in a small number of instances of other inborn metabolic abnormalities that also present with movement problems, such as Lesh-Nyhan syndrome, but no examples of patients with MSUD and movement disorder have been documented to date. 16,17