3. Mononeuropathy means focal involvement of a single nerve
and implies a local process. Direct trauma, compression or
entrapment, vascular lesions, and neoplastic infiltration
diabetes mellitus, hypothyroidism,acromegaly, alcoholism,
and HNPP
4. mononeuropathy multiplex
signifies simultaneous or sequential damage to multiple noncontiguous nerves
vasculitis or microangiopathy in diabetes mellitus, infectious, granulomatous,
leukemic, or neoplastic infiltration, sarcoidosis and -Multifocal motor neuropathy
.
Polyneuropathy is characterized by symmetrical, distal motor, and sensory
deficits that have a graded increase in severity distally and by distal attenuation
of reflexes. The sensory deficits produce a stocking-glove pattern
5. Classification according to nerve
type involvement
predominantly motor manifestations
1. Multifocal motor neuropathy
2. Guillain-Barre syndrome
3. Acute motor axonal neuropathy
4. Porphyric neuropathy
5. Chronic inflammatory polyradiculoneuropathy
6. Neuropathy with osteosclerotic myeloma
7. Hereditary motor sensory neuropathies[ Charcot-Marie-Tooth disease)
8. Lead intoxication
Predominant sensory involvement diabetes; carcinoma; Sjogren's syndrome;
dysproteinemia; acquired immunodeficiency syndrome (AIDS); vitamin B12 deficiency;
celiac disease; intoxications with cisplatin, thalidomide, or pyridoxine; and inherited
idiopathic sensory neuropathies.
Autonomic dysfunction is seen in GBS amyloid and diabetic, idiopathic panautonomic
neuropathy, Porphyria, Hereditary sensory and autonomic neuropathy
6.
7. Electro diagnostic Studies prove the diagnosis , determine the extent of the disease
Determine the type of neuropathy ether demyelination or axonal
Nerve biopsy
Vasculitis,Amyloidosis, Sarcoidosis, Hansen's disease (leprosy]
molecular genetic tests
complete blood count, sedimentation rate, chemistry profile, fasting blood sugar, thyroid
studies, vitamin B12 level, and serum protein electrophoresis with immunofixation
electrophoresis , nasal swab for leprosy
auto antibodies gangliosidcs
General investigation used to identify the etiology of the PNPs
diagnostic Studies
9. two thirds of patients report a preceding event, most frequently an
upper respiratory or gastrointestinal infection Campylobacter
jejuni, surgery, or immunization 1—4 weeks before the onset of
neurological symptoms
12. Acute Inflammatory Demyelinating Polyradiculoneuropathy (Guillain-Barre Syndrome)
motor weakness, areflexia, paresthesias with slight sensory loss, and increased protein
in CSF without pleoeytosis (albuminocytological dissociation). The frequent finding
of motor conduction block and reduced NCVs provided electrophysiological
confirmation of widespread demyelination
Features required for diagnosis
Progressive weakness of both legs and arms
A reflexia
Clinical features supportive of diagnosis
Progression 1 day -4 weeks
Relative symmetry of signs
Mild sensory symptoms or signs
Cranial nerve involvement (bifacial palsies)
Recovery beginning 2—4 wks after progression ceases
Autonomic dysfunction
Absence of fever at onset
Laboratory features supportive of diagnosis
Elevated cerebrospinal fluid protein with normal cells count
Elcctrodiagnostic features of nerve conduction slowing or block
14. Treatment
Supportive care in intensive care
Respiratory and bulbar function, the ability to handle secretions, heart rate,
and blood pressure should be closely monitored during the progressive phase
mechanical ventilation
deterioration in forced vital capacity (FVC),
declining maximal respiratory pressures, and
hypoxemia caused by atelectasis
rapid disease progression (onset to admission in less
than 7 days), bulbar dysfunction, bilateral facial
palsies, and autonomic instability
15. lower the risks of venous thrombosis and pulmonary embolism
Prevention and prompt treatment of infections
Chest physiotherapy and frequent oral suctioning
regular turning and attention to skin, eyes, mouth, bowel, and bladder
Physical therapy
Psychological support and constant reassurance
Plasma exchange and
high-dose intravenous immune globulin
(IVIG) infusions have been shown to be equally effective in moderate to severe
weakness
Up to 30% of patients develop respiratory insufficiency requiring assisted
ventilation, and between 2% and 5% die of complications.
20% still have residual motor weakness 1 year later. Approximately 70% of
patients complete their recovery in 12 months and 82% in 24 months.
16. Chronic Inflammatory Demyelinating
similar clinical features and CSF change and pathological abnormalities of multifocal
Inflammatory demyelination, with nerve conduction features reflecting
demyelination. to GBS
The major differences between the two conditions are
in the time course and their response to corticosteroids
Prednisone, plasmapheresis, and IVIg
Multifocal Motor Neuropathy with Conduction Block
more common in men
Progressive, asymmetrical, predominantly distal limb weakness. Profound
weakness in muscles with normal bulk
Treatment with IVIG is currently the preferred treatment
17. Porphyric Neuropathy
inactivation of one of allelic genes that encodes for an enzyme of the heme biosynthetic
pathway this provokes a compensatory overproduction of porphyrins and their precursors
Dominantly inherited disorders
Fits + abdominal +autonomic manifestations
Only a few patients progress to develop the more ominous motor neuropathy with
generalized, proximal, or asymmetrical muscle weakness developing over days or
weeks
18. Ataxia imbalance and incoordination involving either gait and limbs it is
either due to
a disorder involving the cerebellum or its connections or due to
proprioceptive disorder Types
1. truncal ataxia
2. gait ataxia
3. appendicular ataxia
sensory ataxia
Cerebeller ataxia
Neurological Signs
1. Stance and gait :
tandem gait
Romberg test
Ataxic gait is characterized by a widened base and an irregular staggering
appearance
19. 2. Limb incoordination
finger-to-nose test
heel-to-shin maneuver
Dysmetria under-reached (hypometria) or over-reached (hypermetria
Dysdiadochokinesis: This term refers to irregularity of the rhythm
and amplitude of rapid alternating Movements
Decomposition of movements;
Rebound phenomena
Romberg test + Impaired position and vibration sense and the deep tendon
reflexes
Dys synergia
20. 1. Stroke intracerebral he
2. Genetic
3. Ataxic cerebral palsy,
4. Infections like acute cerebellitis, abscess;
5. posterior fossa tumors like cerebellar gliomas, ependymomas, pontine glioma;
meningiomas
6. vascular malformations;
7. congenital anomalies like Arnold-Chiari malformation;
8. toxic such as anticonvulsants;
9. immune related to neoplasm's, especially opsoclonus-myoclonus anti-GAD and
anti-gliadin antibodies
10. MS
11. alcohol;
12. Hypothyroidism
13. Toxic: mercury;
21. Friedreich '$ Ataxia
spinocerebeller disease
age at onset of younger than 25 years, typically early in adolescence
1--Onset is with increasing gait difficulties and neurological examination reveals
gait ataxia, loss of proprioceptive sense in the lower limbs, and absence of deep
tendon reflexes, cither generalized or in the lower limbs
2- Skeletal deformity
3--upper motor neuron findings such as extensor plantar responses and
4- Optic atrophy
5-Rarely, patients may present with cardiac disease or .: spinal deformity
about 9-15 years after onset lose ambulation increasing ataxia of both upper
and lower limbs, profound proprioceptive loss, areflexia, weakness of lower
limb muscles, dystonia, flexor spasms, and increasing dysarthria and dysphagia
Optic atrophy and hearing loss may occur in many patients
Systemic abnormalities abnormal electrocardiogram recordings, hypertrophic
cardiomyopathy in about 50% of the patients, and diabetes in 10%. Skeletal
abnormalities such as spinal deformities and foot deformities are common
22. The mean age at death --late in the fourth decade---Cause of death is usually cardiac
MRI scans of the brain reveal no abnormalities in the cerebellum; rather, the upper
cervical cord shows atrophy
Nerve conduction studies show early absence or reduction of sensory nerve potentials
in a diffuse fashion
the protein frataxin
coenzyme Q and vitamin F.
A tax ia-Telangiectasia the disease has its onset in the first decade. Children
develop progressive ataxia associated with hypotonia, areflexia, peripheral
neuropathy, and choreoarhetosis
Telangiectasias develop over the conjunctivae ,
ear lobes, and other areas during the second half of the first
decade.
Many patients have decreased immunoglobulin A (IgA) levels; decreased munoglobulin
E and immunoglobulin M levels, lymphocytopenia
The Fried Reich's Ataxia Mutation===the disease is recessively inherited on
chromosome 9
