GENERAL MEDICINE CASE DISCUSSION: PARAPERESIS
PRESENTED BY:
SS21056252.jpg
https://appliedradiology.com/articles/anterior-cerebral-artery-vasculopathy-secondary-to-miliary-tb
An 18 yr old male presented with complaints of
Difficulty in walking since 1 month
Bilateral lower limb weakness since 1 month
Pain in the lower limb calf muscles since 1 month.
h/o difficulty in standing from sitting position.
h/o difficulty in climbing stairs
h/o difficulty in holding footwear
h/o slipping of footwear without knowledge
h/o muscle wasting (LL>UL)
Patient is a known alcoholic
GENERAL EXAMINATION:
pallor present. patient is conscious and coherent.
CNS EXAMINATION:
Pt is conscious and cranial nerves intact
Sensory system - normal
Cerebellar and meningeal signs - absent
Motor system:
bulk- decreased in UL and LL
Tone: normal in UL. Hypotonia in lower limbs
Plantar reflex- absent
Deep tendon reflexes- Absent
Presence of lower limb hypotonia and absence of reflexes points towards a possible LMN lesion causing flaccid papaperesis.
ON INVESTIGATION:
Hb- 10.4 gm/dl
Creatine kinase level was normal - rules out any neuromuscular cause of paraparesis ( CK is normally raised in neuromuscular disorders)
NORMALLY WHEN WE THINK OF FLACCID PARAPARESIS THE SITE OF LESION MIGHT BE ONE OF THE FOLLOWING
"ANTERIOR HORN CELL--NERVE ROOT--PLEXUS--PERIPHERAL NERVE--NMJ--MUSCLE
TO RULE OUT FEW CAUSES:
ON FURTHER INVESTIGATION:
Nerve conduction study- was suggestive of bilateral common peroneal and rural nerve involvement
DIAGNOSIS: Flaccid paraparesis secondary to PERIPHERAL NEUROPATHY ( bilateral common peroneal and rural nerve involvement )
PERIPHERAL NEUROPATHY:
Peripheral neuropathy is a term describing disease affecting the peripheral nerves, meaning nerves beyond the brain and spinal cord.
Symptoms depend on the type of nerves damaged- motor or sensory or autonomous
* in our patient pain in legs might have been due to sural nerve involvement which is a sensory nerve supplying the calf region of the leg whereas peroneal nerve is a mixed nerve and due to its involvement there is lower limb weakness.*
General Causes of Peripheral Neuropathy are:
Peripheral Neuropathy Fact Sheet | National Institute of ...www.ninds.nih.gov › Patient-Caregiver-Education › Pe...
SURAL NERVE BIOPSY was ordered to know the further pathology
H/O bilateral lower limb edema- non pitting type.
H/O difficulty in squatting position and diificulty in getting up from squatting position.
H/O difficulty in wearing and holding footwear
ON EXAMINATION
GENERAL EXAMINATION- NORMAL
CNS EXAMINATION
Pt is conscious, coherent, co-operative
Higher mental functions- normal
cranial nerves- intact
sensory system- normal
No cerebellar and meningeal signs
Motor system:
Tone- normal
Power- LL- 4/5 in both lower limbs
reflexes absent in both lower limbs
DIFFERENTIAL DIAGNOSIS
Progressive Proximal Muscle weakness is a consistent feature of
A neuro-muscular disease can be considered but the lack of ocular symptoms and fluctuating symptoms rules out disorders like myaesthenia gravis and lack of autonomic symptoms are atypical of Lambert Eaton Myasthenic syndrome.
At this stage we have a probable diagnosis of a MYOPATHY which has to be further investigated
ON INVESTIGATION
TREATMENT: Presently there is no cure for muscular dystrophy.
Muscular dystrophy: Symptoms, treatment, types, and causeswww.medicalnewstoday.com ›
articles
DUCHENNE MUSCULAR DYSTROPHY
"Duchenne muscular dystrophy (DMD) is a genetic condition that affects the muscles, leading to muscle wasting that gets worse over time. DMD occurs primarily in males, though in rare cases may affect females. The symptoms of DMD include progressive weakness and loss (atrophy) of skeletal and heart muscles. Early signs of DMD may include delayed ability to sit, stand, or walk and difficulties learning to speak. Muscle weakness is usually noticeable in early childhood. Most children with DMD use a wheelchair by their early teens. Heart and breathing problems also begin in the teen years and lead to serious, life threatening complications. DMD is caused by genetic changes in the DMD gene. DMD is inherited in an X-linked recessive pattern and may occur in people who do not have a family history of DMD. While there is no known cure for DMD, there are treatments that can help control symptoms. Becker muscular dystrophy (BMD), a milder form of muscular dystrophy, is also caused by DNA variants in the DMD gene"
Duchenne muscular dystrophy | Genetic and Rare Diseases ...rarediseases.info.nih.gov › diseases › duchenne-muscul...
BECKER MUSCULAR DYSTROPHY
"Becker muscular dystrophy (BMD) is an inherited condition that causes progressive weakness and wasting of the skeletal and cardiac (heart) muscles. It primarily affects males.The age of onset and rate of progression can vary. Muscle weakness usually becomes apparent between the ages of 5 and 15. In some cases, heart involvement (cardiomyopathy) is the first sign.BMD is caused by a mutation in the DMD gene and is inherited in an X-linked recessive manner. BMD is very similar to DMD , except that in BMD, symptoms begin later and progress at a slower rate.There is no cure for this condition"
Becker muscular dystrophy - Genetic and Rare Diseases - NIHrarediseases.info.nih.gov › diseases › becker-muscular-...
P. TRISHAALA REDDY
ROLL NO 121 8TH SEMESTER
This week we were given 3 cases of patients presenting with lower limb weakness also known as
PARAPERESIS as a part of GENERAL MEDICINE case discussion.
THE 3 cases an be found in the given links:
CASE 3: https://hitesh116.blogspot.com/2020/05/12may-2020-elog-medicine-intern.html?m=1
PARAPERESIS
PARAPERESIS is the partial paralysis of both the legs. It is characterised by progressive weakness and spasms in the legs.
True weakness occurs when muscles loose their strength.
Some common Causes of Paraperesis:
A 23 yr old male patient presents with:
1. Bilateral lower limb weakness associated with tingling and numbness since 5 days.
Had a sudden fall when got up for urination.
2. H/o 3-4 episodes of vomitings 5 days ago- non projectile, non bilious, food particles present.
3.Sudden fall when he got up for urination.
Past History:
Gluteal abscess since 5 months ( operated 5 months back)
Scrotal abscess since 20 days ( incision and drainage 10 days back)
NO Similar complaints in the past.
NO other relevant past history.
ON EXAMINATION:
Vitals and General examination - normal
CNS examination:
conscious
speech - normal
cranial nerves- intact
Motor system:
bulk- normal
tone- hypotonia in both lower limbs
power- decreased power in both lower limbs
Reflexes - present
plantar reflex- extensor in both right and left
Ankle clonus- present in rt, absent in left
Involuntary movements- absent
Sensory system- normal
Cerebellar signs- absent
Meningeal signs- absent
AT this point we can rule out:
LMN lesions- due to presence of reflexes and extensor plantar reflex
Meningitis- due absence of meningeal signs
ON FURTHER INVESTIGATION:
PARAPERESIS
PARAPERESIS is the partial paralysis of both the legs. It is characterised by progressive weakness and spasms in the legs.
True weakness occurs when muscles loose their strength.
Some common Causes of Paraperesis:
- Arthritis of the spine
- Herniated disc
- Spinal cord injury, abscess or tumor
- Spinal degeneration
- Spinal stenosis
- Infections of spinal cord
- Gullian barre syndrome
- Multiple sclerosis
- Muscular dystrophy
- Myasthenia Gravis
- Polymyositis
- Botulinum toxin
- Organo phosphorus poisoning
- Stoke
- Cerebral palsy
- Transient ischaemic attacks
- Vit B12 deficiency
- Injury to joints, bones and muscles
CASE 1:
A 23 yr old male patient presents with:
1. Bilateral lower limb weakness associated with tingling and numbness since 5 days.
Had a sudden fall when got up for urination.
2. H/o 3-4 episodes of vomitings 5 days ago- non projectile, non bilious, food particles present.
3.Sudden fall when he got up for urination.
Past History:
Gluteal abscess since 5 months ( operated 5 months back)
Scrotal abscess since 20 days ( incision and drainage 10 days back)
NO Similar complaints in the past.
NO other relevant past history.
ON EXAMINATION:
Vitals and General examination - normal
CNS examination:
conscious
speech - normal
cranial nerves- intact
Motor system:
bulk- normal
tone- hypotonia in both lower limbs
power- decreased power in both lower limbs
Reflexes - present
plantar reflex- extensor in both right and left
Ankle clonus- present in rt, absent in left
Involuntary movements- absent
Sensory system- normal
Cerebellar signs- absent
Meningeal signs- absent
AT this point we can rule out:
LMN lesions- due to presence of reflexes and extensor plantar reflex
Meningitis- due absence of meningeal signs
ON FURTHER INVESTIGATION:
- As the patient shows high risk behaviour it is important to test for viral serology, mainly HIV. However on investigation viral serology was negative.
- x-ray chest showed multiple nodules in pulmonary apices suggestive of Pulmonary Tuberculosis and a possible Miliary tuberculosis ( disseminated TB)
- On x-ray of abdomen-L4 L5 spondylodiscitis with left psoas abscess ( which are seen in pott’s disease) which might have been a reason for cord compression and paraparesis but Is ruled out as clinical examination is not consistent with a LMN lesion
- MRI brain showed diffuse enhancement in right and left cerebral hemispheres-diffuse enhancement rules out the possibility of a Tumor such as a Tuberculoma in the cortex.
Diffuse enhancement in the parasaggital cortex of the frontal cortex (motor cortex) is suggestive of a Vasculitis in the ACA territory of the cortex causing brain infraction which is causing lower limb weakness.
This is due to blood borne spread of TB.
This is due to blood borne spread of TB.
SS21056252.jpg
https://appliedradiology.com/articles/anterior-cerebral-artery-vasculopathy-secondary-to-miliary-tb
TREATMENT :
T.ATT 3 tabs/day fdc
T.Benadon 40mg/od
T.pregabalin 75mg/po/h/s
OINT.MEGAHEAL FOR LOCAL APPLICATION
SITZ BATH WITH BETADINE TID
FREQUENT CHANGE OF POSITION
CASE 2:
Difficulty in walking since 1 month
Bilateral lower limb weakness since 1 month
Pain in the lower limb calf muscles since 1 month.
h/o difficulty in standing from sitting position.
h/o difficulty in climbing stairs
h/o difficulty in holding footwear
h/o slipping of footwear without knowledge
h/o muscle wasting (LL>UL)
Patient is a known alcoholic
GENERAL EXAMINATION:
pallor present. patient is conscious and coherent.
CNS EXAMINATION:
Pt is conscious and cranial nerves intact
Sensory system - normal
Cerebellar and meningeal signs - absent
Motor system:
bulk- decreased in UL and LL
Tone: normal in UL. Hypotonia in lower limbs
Plantar reflex- absent
Deep tendon reflexes- Absent
Presence of lower limb hypotonia and absence of reflexes points towards a possible LMN lesion causing flaccid papaperesis.
ON INVESTIGATION:
Hb- 10.4 gm/dl
Creatine kinase level was normal - rules out any neuromuscular cause of paraparesis ( CK is normally raised in neuromuscular disorders)
NORMALLY WHEN WE THINK OF FLACCID PARAPARESIS THE SITE OF LESION MIGHT BE ONE OF THE FOLLOWING
"ANTERIOR HORN CELL--NERVE ROOT--PLEXUS--PERIPHERAL NERVE--NMJ--MUSCLE
- Anterior horn cell disease e.g. motor neuron disease (NB: mixed upper and lower motor neuron signs), poliomyelitis
- Cauda Equina Syndrome secondary to disc herniation/epidural abscess/mets/haematoma etc.
- Lumbosacral plexopathy secondary to trauma/tumour/abscess (usually unilateral)
- Motor neuropathies:
- Inflammatory: guillain-barre syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, sarcoid, vasculitis, paraprotein, amyloidosis
- Infectious: HIV, diphtheria, lyme disease, HTLV-1, west nile virus, rabies, enteroviruses
- Toxins: lead, arsenic, thallium, mercury, shellfish, pufferfish poisoning
- Metabolic: diabetic amyotrophy, porphyria
- Drugs: ciclosporin, gold, penicillamine
- Congenital: charcot marie tooth disease (HSMN)
- Neuromuscular junction disorders:
- Myaesthenia gravis
- Lambert-Eaton myaesthenic syndrome
- Botulism
- Organophosphate poisoning
- Tick paralysis
- Snake venom
- Myopathies:
- Inflammatory: polymyositis/dermatomyositis. NB: can overlap with other connective tissue disease eg mixed connective tissue disease
- Other connective tissue disease eg. SLE, vasculitis, RA, systemic sclerosis.
- Cancer (paraneoplastic: carcinomatous neuromyopathy)
- Drugs (statins, steroids)
- Infections (bacterial infections, HIV, CMV, EBV, Hepatitis)
- Endocrine (thyroid, addisons, osteomalacia, cushings, acromegaly, diabetic amyotrophy)
- Toxins (alcohol)
- Metabolic (renal/liver failure, electrolyte disturbance e.g. periodic paralysis)
- Miscellaneous (inclusion body myositis, rhabdomyolysis, sarcoidosis, mitochondrial myopathy, muscular dystrophy"
TO RULE OUT FEW CAUSES:
- Charcot marie tooth- absent high stepping gait pes caves, tooth clawing and scoliosis
- Gullain barre syndrome- ptosis, ophthalmoplegia, facial nerve involvement and bulbar signs are absent
- Cauda equina- bowel, bladder and sexual dysfunction and lower back pain absent
- Spina bifida- bowel and bladder involvement absent
- Polio- limb shortening absent
- Normal CREATINE KINASE levels- rules out any muscular origin to paraparesis
- Viral cause: serology negative
- Diabetes was ruled out on investigation
ON FURTHER INVESTIGATION:
Nerve conduction study- was suggestive of bilateral common peroneal and rural nerve involvement
DIAGNOSIS: Flaccid paraparesis secondary to PERIPHERAL NEUROPATHY ( bilateral common peroneal and rural nerve involvement )
PERIPHERAL NEUROPATHY:
Peripheral neuropathy is a term describing disease affecting the peripheral nerves, meaning nerves beyond the brain and spinal cord.
Symptoms depend on the type of nerves damaged- motor or sensory or autonomous
* in our patient pain in legs might have been due to sural nerve involvement which is a sensory nerve supplying the calf region of the leg whereas peroneal nerve is a mixed nerve and due to its involvement there is lower limb weakness.*
General Causes of Peripheral Neuropathy are:
- physical injury/ trauma
- diabetes leading to polyneuropathy
- vascular and blood problems leading to decreased oxygen supply to nerves. e.g: smoking, vasculitis
- systemic or local autoimmune disorders such as sjogren's, rheumatoid arthritis etc
- hormonal imbalances
- kidney and liver diseases leading to accumulation of toxic substances which may damage the nerves
- nutritional and vitamin imbalances , alcoholism and ingestion of toxic substances. Vit B12 def and excess Vit B6 are most frequent vitamin related causes
- malignant and benign tumors
- chemotherapy drugs
- infection like varicella zoster , CMV etc
Peripheral Neuropathy Fact Sheet | National Institute of ...www.ninds.nih.gov › Patient-Caregiver-Education › Pe...
SURAL NERVE BIOPSY was ordered to know the further pathology
- Usual causes of sural nerve neuropathy include : compression by a mass lesion such a ganglion, trauma, inflammatory and vasculitic diseases
- Peroneal neuropathy is commonly caused due to external compression at the level fibular head
- The patient is a known to take alcohol, it might lead to vit B12 ,B6, thiamin, niacin and folate deficiency which cause peripheral neuropathy.
CASE 3:
An 18 yr old male patient came to the OPD with chief complaints of
Bilateral lower limb weakness since 20 days
HOPI: Weakness in bilateral lower limbs started 2 yrs ago. It started as a proximal muscle weakness and over the years has now progressed to a distal weakness.
Bilateral lower limb weakness since 20 days
HOPI: Weakness in bilateral lower limbs started 2 yrs ago. It started as a proximal muscle weakness and over the years has now progressed to a distal weakness.
H/O bilateral lower limb edema- non pitting type.
H/O difficulty in squatting position and diificulty in getting up from squatting position.
H/O difficulty in wearing and holding footwear
ON EXAMINATION
GENERAL EXAMINATION- NORMAL
CNS EXAMINATION
Pt is conscious, coherent, co-operative
Higher mental functions- normal
cranial nerves- intact
sensory system- normal
No cerebellar and meningeal signs
Motor system:
Tone- normal
Power- LL- 4/5 in both lower limbs
reflexes absent in both lower limbs
DIFFERENTIAL DIAGNOSIS
Progressive Proximal Muscle weakness is a consistent feature of
- Myopathies
ACQUIRED MYOPATHY ( inflammatory OR toxic)
GENETIC MYPPATHY ( muscular distrophy, late onset congenital myopathy or metabolic myopathy)
A neuro-muscular disease can be considered but the lack of ocular symptoms and fluctuating symptoms rules out disorders like myaesthenia gravis and lack of autonomic symptoms are atypical of Lambert Eaton Myasthenic syndrome.
Nerve conduction studies further help to rule out neuro muscular disorders.
ON INVESTIGATION
- CBP - normocytic normochromic with leucocytosis
- RFT - increases creatinine and uric acid levels
- CUE- albumin + and pus cells present
- Muscle biopsy- suggestive of polymyositis and Muscalar dystrophy
Acquired myopathies are ruled out due to absence of antibodies and no H/O of exposure to any toxic substances
Metabolic Myopathies are ruled out due to absence of any clinical signs or lab investigations consistent with metabolic disorders
Increased levels of creatine kinase and muscle biopsy point towards a possibility of a MUSCULAR DYSTROPHY.
MUSCULAR DYSTROPHY
Muscular distrophy refers to a group of disorders that cause progressive loss of muscle mass and loss of muscle strength.
They occur due to genetic mutations in muscle proteins ( majorly Distrophin) which are needed for building up and normal functioning of the muscles.
DIFFERENT TYPES OF MUSCULAR DYSTROPHIES
- Duchenne muscular dystrophy
- Becker muscular dystrophy
- Myotonic (Steinert’s disease)
- Congenital
- Facioscapulohumeral (FSHD)
- Limb-girdle
- Oculopharyngeal muscular dystrophy
FURTHER INVESTIGATIONS REQUIRED:
TREATMENT: Presently there is no cure for muscular dystrophy.
- corticosteroids- increase muscle strength and slow progression
- Heart medications if associated with any heart conditions
- Physiotherapy.
Muscular dystrophy: Symptoms, treatment, types, and causeswww.medicalnewstoday.com ›
articles
DUCHENNE MUSCULAR DYSTROPHY
"Duchenne muscular dystrophy (DMD) is a genetic condition that affects the muscles, leading to muscle wasting that gets worse over time. DMD occurs primarily in males, though in rare cases may affect females. The symptoms of DMD include progressive weakness and loss (atrophy) of skeletal and heart muscles. Early signs of DMD may include delayed ability to sit, stand, or walk and difficulties learning to speak. Muscle weakness is usually noticeable in early childhood. Most children with DMD use a wheelchair by their early teens. Heart and breathing problems also begin in the teen years and lead to serious, life threatening complications. DMD is caused by genetic changes in the DMD gene. DMD is inherited in an X-linked recessive pattern and may occur in people who do not have a family history of DMD. While there is no known cure for DMD, there are treatments that can help control symptoms. Becker muscular dystrophy (BMD), a milder form of muscular dystrophy, is also caused by DNA variants in the DMD gene"
Duchenne muscular dystrophy | Genetic and Rare Diseases ...rarediseases.info.nih.gov › diseases › duchenne-muscul...
BECKER MUSCULAR DYSTROPHY
"Becker muscular dystrophy (BMD) is an inherited condition that causes progressive weakness and wasting of the skeletal and cardiac (heart) muscles. It primarily affects males.The age of onset and rate of progression can vary. Muscle weakness usually becomes apparent between the ages of 5 and 15. In some cases, heart involvement (cardiomyopathy) is the first sign.BMD is caused by a mutation in the DMD gene and is inherited in an X-linked recessive manner. BMD is very similar to DMD , except that in BMD, symptoms begin later and progress at a slower rate.There is no cure for this condition"
Becker muscular dystrophy - Genetic and Rare Diseases - NIHrarediseases.info.nih.gov › diseases › becker-muscular-...
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